BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized ...BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized rehabilitation are also crucial.Here,we present a surgical strategy to prevent the recurrence of MO.CASE SUMMARY A 28-year-old female patient was admitted for the first time for a comminuted fracture of the left olecranon.However,incorrect postoperative rehabilitation resulted in the development of elbow joint stiffness with ectopic ossification,causing a loss of normal range of motion.The patient was diagnosed with MO based on physical examination,X-ray findings,and clinical presentation.We devised a surgical strategy to remove MO,followed by fixation with an Ilizarov frame,and implemented a scientifically reasonable rehabilitation plan.The surgery lasted for 3 h with an estimated blood loss of 45 mL.A drainage tube was placed after surgery,and fluid was aspirated through ultrasound-guided puncture.The patient experienced a significant reduction in joint stiffness after surgery.In the final follow-up at 9 mouths,there was evident improvement in the range of motion of the elbow joint,and no other symptoms were reported.CONCLUSION The Ilizarov frame is an advantageous surgical technique for facilitating rehabilitation after MO removal.It offers benefits such as passive recovery,individualized treatment,and prompt recovery.展开更多
Rationale:Fever with myositis and rhabdomyolysis is a medical emergency requiring prompt diagnosis and management.Scrub typhus associated myositis with rhabdomyolysis is rare.Patient concerns:A 36-year-old female pres...Rationale:Fever with myositis and rhabdomyolysis is a medical emergency requiring prompt diagnosis and management.Scrub typhus associated myositis with rhabdomyolysis is rare.Patient concerns:A 36-year-old female presented with intermittent fever up to 38.6℃,jaundice and progressive weakness of all four limbs.Diagnosis:Scrub typhus associated myositis and rhabdomyolysis.Intervention:Doxycycline 100 mg twice daily and injection of ceftriaxone 1 gm twice daily along with continuous intravenous fluids.Outcome:Fever resolved with normalization of liver function and recovery of muscle power.Lessons:Presence of myositis and rhabdomyolysis is uncommon in scrub typhus;high clinical suspicion should be kept in patients with atypical manifestations of scrub typhus.展开更多
BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could ...BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could be difficult to understand and a wrong diagnosis could lead to an improper cure.CASE SUMMARY We herein report the case of IMO in a healthy 69-year-old man.The patient presented with an abdominal mass in the left lower quadrant.A computed tomography scan showed an inhomogeneous mass with multiple calcifications.The patient underwent radical excision of the mass.Histopathological findings were compatible with MO.Five months later the patient showed a recurrence causing hemorrhagic shock due to intractable intralesional bleeding.The patients eventually died within three months since recurrence.CONCLUSION The case described could be classified as post-traumatic MO that developed close to the previously fractured iliac bone.The subsequent surgical procedure was ineffective and the disease rapidly recurred.The misleading intraoperative diagnosis led to improper surgical treatment with a dramatic evolution.展开更多
Objective: With scant studies in the literature, little is known about the risk factors for tuberculosis in patients with dermatomyositis/polymyositis. Therefore, the aim of the present study was to analyze the predic...Objective: With scant studies in the literature, little is known about the risk factors for tuberculosis in patients with dermatomyositis/polymyositis. Therefore, the aim of the present study was to analyze the predictive factors for tuberculosis development in dermatomyositis/polymyositis. Methods: This single-center, retrospective, cohort study initially included 290 patients with dermatomyositis/polymyositis, from 2002 to 2016. Tuberculosis (pulmonary and/or extra-pulmonary) was confirmed after dermatomyositis/polymyositis diagnosis in 12 patients (4.1%) (Tuberculosis+ group). For the control group (Tuberculosis−), 24 patients without tuberculosis were arbitrarily selected in the same period and matched for age, ethnicity, gender, age at disease diagnosis, disease duration and type (dermatomyositis or polymyositis). Results: Tuberculosis occurred for a median of 16 months after dermatomyositis/polymyositis diagnosis. Clinical, laboratory and treatment features were similar in Tuberculosis+ and Tuberculosis−groups (P > 0.05). However, previous pulmonary fibrosis in dermatomyositis/polymyositis was more prevalent in the Tuberculosis+ group (41.7 vs. 8.3%;P = 0.029). Moreover, on a multivariate logistic regression model, pulmonary fibrosis was significantly associated with Tuberculosis (Odds ratio: 9.59, 95% confidence interval: 1.17 - 78.82). Tuberculosis affected 3 dermatomyositis cases for every 1 polymyositis case, with predominantly pulmonary followed by extra-pulmonary involvement (pleura, cutaneous, muscular, joint, soft tissue and hematologic). Two or more sites were affected in 41.7% of cases. Conclusions: Previous pulmonary fibrosis in dermatomyositis/polymyositis was a predictive factor associated with tuberculosis development. Further studies are needed to confirm these results.展开更多
BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history...BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history and nonspecific early clinical manifestations,which may lead to the failure of timely and effective diagnosis and treatment in some patients.We report the diagnosis and treatment of a child with nontraumatic myositis ossificans and summarize the clinical characteristics and diagnosis and treatment of the disease.CASE SUMMARY An 8-year-old girl first came to our hospital for more than a week with pain in the right lower limb.There was no history of trauma or strenuous activities.On physical examination,no mass on the right thigh was found,and the movement of the right lower extremity was limited.Ultrasonography showed synovitis of the hip,and bed rest was recommended.Three days later,the child’s pain persisted and worsened,accompanied by fever and other discomforts.She came to our hospital again and a mass was found on the right thigh with redness and swelling on the surface.The images showed a soft tissue tumor on the right thigh with calcification.Routine blood tests revealed that the inflammation index was significantly increased.In case of infection,the patient was given antibiotics,and the pain was relieved soon after,without fever.However,the right thigh mass persisted and hardened.The patient underwent incision biopsy more than 1 mo later,and the postoperative pathology showed nontraumatic myositis ossificans.After approximately 9 mo of observation,the tumor still persisted,which affected the life of the child,and then resection was performed.Since follow-up,there has been no recurrence.CONCLUSION Due to the difficulty in discerning a child’s medical history and the diverse early manifestations,it is difficult to diagnose nonossifying muscle disease in children in its early stage.Measures such as timely follow-up and periodic image monitoring are conducive to early diagnosis of the disease.The disease has a certain degree of self-limitation,and it can be observed and treated first.If the tumor persists in the later stage or affects functioning,then surgery is considered.展开更多
In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with to...In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with tofacitinib. Case Report: An adult female patient presented with definite dermatomyositis and with refractoriness to high doses of intravenous and oral glucocorticoids, intravenous human immunoglobulin, several immunosuppressive drugs (methotrexate, azathioprine, and leflunomide) and two previous immunobiological drugs (rituximab and abatacept). However, the patient had a good outcome with tofacitinib. Conclusions: Tofacitinib appears to be a promising alternative therapy for refractory dermatomyositis.展开更多
AIM To define clinical criteria to differentiate eosinophilic gastrointestinal disorder(Eo GD) in the esophagus. METHODS Our criteria were defined based on the analyses of the clinical presentation of eosinophilic eso...AIM To define clinical criteria to differentiate eosinophilic gastrointestinal disorder(Eo GD) in the esophagus. METHODS Our criteria were defined based on the analyses of the clinical presentation of eosinophilic esophagitis(Eo E), subepithelial eosinophilic esophagitis(s Eo E) and eosinophilic esophageal myositis(Eo EM), identified by endoscopy, manometry and serum immunoglobulin E levels(s-Ig E), in combination with histological and polymerase chain reaction analyses on esophageal tissue samples.RESULTS In five patients with Eo E, endoscopy revealed longitudinal furrows and white plaques in all, and fixed rings in two. In one patient with s Eo E and four with Eo EM, endoscopy showed luminal compression only. Using manometry, failed peristalsis was observed in patients with Eo E and s Eo E with some variation, while Eo EM was associated with hypercontractile or hypertensive peristalsis, with elevated s-Ig E. Histology revealed the following eosinophils per high-power field values. Eo E = 41.4 ± 7.9 in the epithelium and 2.3 ± 1.5 in the subepithelium; s Eo E = 3 in the epithelium and 35 in the subepithelium(conventional biopsy); Eo EM = none in the epithelium, 10.7 ± 11.7 in the subepithelium(conventional biopsy or endoscopic mucosal resection) and 46.8 ± 16.5 in the muscularis propria(peroral esophageal muscle biopsy). Presence of dilated epithelial intercellular space and downward papillae elongation were specific to Eo E. Eotaxin-3, IL-5 and IL-13 were overexpressed in Eo E.CONCLUSION Based on clinical and histological data, we identified criteria, which differentiated between Eo E, s Eo E and Eo EM, and reflected a different pathogenesis between these esophageal Eo GDs.展开更多
Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis....Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis. A 33-year-old Moroccan woman presented with a 2-month history of left inguinal inflammatory pain with limping gait, MRI examination suggested a malignant neoplasm such as soft tissue osteosarcoma. A diagnosis of myositis ossificans was made by incisional biopsy. Conservative management with clinical and radiological follow up of 19 months confirm the diagnosis. The symptoms resolved within seven months. Myositis ossificans should be considered by clinicians as a possible diagnosis for a soft tissue lesion.展开更多
BACKGROUND Proliferative myositis is a rare benign tumor that is typically self-limiting and does not become malignant.It can be cured by simple resection without reported recurrence.Due to its rapid growth,hard struc...BACKGROUND Proliferative myositis is a rare benign tumor that is typically self-limiting and does not become malignant.It can be cured by simple resection without reported recurrence.Due to its rapid growth,hard structure and ill-defined borders,it can however be mistaken for malignant tumors such as sarcomas.CASE SUMMARY We investigate the case of a 64-year-old male with proliferative myositis of the abdominal wall,who was preoperatively administered a needle aspiration biopsy and given a simple excision and patch repair.We then compared it with other similar cases to determine the effectiveness of this treatment method.CONCLUSION Resection with follow-up observation has shown to be an effective treatment method for proliferative myositis.To avoid unnecessarily extended or destructive resection,a thorough and conclusive diagnosis is crucial,which requires adequate imaging and pathological knowledge.展开更多
BACKGROUND: Infective myositis is rare. The retrospective report of clinical data and symptoms from one patient with infective myositis will hopefully provide more information for clinicians in the diagnosis of this ...BACKGROUND: Infective myositis is rare. The retrospective report of clinical data and symptoms from one patient with infective myositis will hopefully provide more information for clinicians in the diagnosis of this disease. METHODS: A male patient, 65 years old, was admitted with "fever and muscle pain since four days ago, accompanied by inertia of all limbs for one day", to the First Department of Neurology, Guangdong Provincial Hospital of Traditional Chinese Medicine on April 25th, 2005. Following admission, a history of diseases was record, and detailed physical and neurological examinations were performed. During the examination, symmetrical myasthenia appeared, tendon reflex disappeared, and creatine kinase levels were increased 500 times higher than normal. The patient was primarily diagnosed with hypokalemic periodic paralysis, myositis, Guillain-Barre syndrome, and upper respiratory infection. Subsequently, the patient was treated with the following: cefuroxime for infection, potassium supplements, breviscapin for promoting blood circulation through the removal of stasis, and ATP/CO-A for myocardial nutrition. Antiviral drugs were not administered. However, laboratory samples were continuously monitored. Creatine kinase levels decreased to normal, and muscle pain was obviously relieved following antibiotics treatment. The results led to a final diagnosis of infective myositis. RESULTS: Four days after treatment (April 29th), muscular tenderness and throat congestion were obviously improved (+/-). The neurological examination showed the patient was conscious, cooperated with treatment, and had normal intellect. No abnormalities of the cranial nerve were observed upon examination. Proximal and distal muscle strength and muscular tensions of the four limbs were all normal. Reflexes of the right biceps brachii muscle and its tendon were decreased, and knee tendon and Achilles tendon reflex were not induced. A Babinski reflex was not detected. The neurological examination presented no abnormalities, and the related creatine kinases were within normal range by re-examination at 2 weeks, and l and 3 months after discharge. CONCLUSION: A diagnosis of infective myositis should be considered for patients with clear prodromes of infection, that include symptoms of periodic paralysis and elevated creatine kinase levels. Treatment with sufficient antibiotics can obtain good results.展开更多
BACKGROUND Trismus is a common problem with various causes.Any abnormal conditions of relevant anatomic structures that disturb the free movement of the jaw might provoke trismus.Trismus has a detrimental effect on th...BACKGROUND Trismus is a common problem with various causes.Any abnormal conditions of relevant anatomic structures that disturb the free movement of the jaw might provoke trismus.Trismus has a detrimental effect on the quality of life.The outcome of this abnormality is critically dependent on timely diagnosis and treatment,and it is difficult to identify the true origin in some cases.We present a rare case of trismus due to fungal myositis in the pterygoid muscle,excluding any other possible pathogenesis.CASE SUMMARY The patient presented with a 2-mo history of restricted mouth opening.Computed tomography showed obvious enlargement of the left pterygoid muscles.Furthermore,the patient had trismus without obvious predisposing causes.The primary diagnosis was pterygoid myosarcoma.Consequently,lesionectomy of the left pterygoid muscle was performed.Intraoperative frozen biopsy implied the possibility of an uncommon infection.Postoperative pathologic examination confirmed myositis and necrosis in the pterygoid muscle.Fungi were detected in both muscle tissue and surrounding necrotic tissue.The patient recovered well with antifungal therapy and mouth opening exercises.The rarity of fungal myositis may be responsible for the misdiagnosis.Although the origin of pathogenic fungi is still unknown,we believe that both hematogenous spread and local invasion could be the most likely sources.To the best of our knowledge,this is therst case in the literature that reported fungal myositis in pterygoid muscles as the only reason that results in trismus.CONCLUSION Surgeons should remain vigilant to the possibility of trismus originating from fungal myositis.展开更多
Myositis ossificans circumscribed is a bone and cartilage heterotopic non neoplastic proliferation inside the soft tissues. It is a benign focal heterotopic ossification process of soft tissues, and a rare disorder th...Myositis ossificans circumscribed is a bone and cartilage heterotopic non neoplastic proliferation inside the soft tissues. It is a benign focal heterotopic ossification process of soft tissues, and a rare disorder that occurs spontaneously or after local trauma. Clinical and radiographic appearances are quite hustler. A careful histological examination of biopsy straightens diagnosis is necessary. There is no consensus in support (surgical or/and medical). From Benign prognosis, evolution of this pathology is usually favorable. The authors report a case of giant myositis ossificans circumscribed post-traumatic localized on gluteus and adductor muscles of the right hip on a 26-year-old man. Through a review of literature the mechanism, the diagnostic methods and therapeutic will be discussed.展开更多
The transobturator tape (TOT) procedure is generally felt to be a safer surgical alternative to the ten sion-free vaginal tape procedure for women with stress urinary incontinence. We report a case of adductor internu...The transobturator tape (TOT) procedure is generally felt to be a safer surgical alternative to the ten sion-free vaginal tape procedure for women with stress urinary incontinence. We report a case of adductor internus myositis not associated with infection following the TOT procedure. To our knowledge this is the first case of this type reported in the literature. A 43 year old lady underwent a straightforward elective TOT procedure. There were no intraoperative complications. Immediately following the procedure she complained of pain in her right thigh. MRI confirmed abnormal oedema within the antero-inferior aspect of the right obturator internus muscle consistent with myositis secondary to tape insertion. The tape was removed the following day in theatre, following which her pain resolved. This case highlights a previously unreported complication as a result of the TOT procedure.展开更多
Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokin...Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokine variables in rituximab-treated refractory myositis patients. Methods: We analyzed data for 200 myositis patients (76 with adult polymyositis (PM), 76 with adult dermatomyositis (DM), and 48 with juvenile (DM)) in the rituximab in myositis trial. Clinical improvement is defined as the change from baseline to 24 weeks in Physician Global Visual Analog Scale (VAS). We analyze the association of baseline variables with improvements: demographics, myositis subtype, clinical and laboratory parameters, autoantibody status, and interferon (IFN)- regulated chemokines. Multivariable linear regression models are developed by using stepwise variable selection methods. Results: A “base” multivariable model to predict improvement with clinical and laboratory variablesonly is built with modest predictive ability (adjusted R2 = 0.21). This model includes two significant factors at baseline: Physician Global VAS and Muscle Disease Activity VAS. A “final” multivariable model to predict improvement including non-standard laboratory measures is developed and demonstrated better predictive ability (adjusted R2 = 0.32). This model includes Physician Global VAS, IFN chemokine score and IL-2 levels. The “final” model explained 11% more variability than the “base” model. Conclusions: Changes in disease activity over time following treatment with rituximab in refractory myositis can be predicted. These models can be clinically useful to optimize treatment selection in myositis.展开更多
Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of...Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of mastication. The patient was a 70-year-old Japanese woman who presented with a tumorous mass in the right parotid area and no history of trauma. The surgically excised tumorous lesion consisted of proliferating fibroblastic cells with no atypia in the central portion, and a formation of trabecular osteoid and/or bony tissue in the peripheral portion as the so-called “zonal phenomenon”. The final histological diagnosis was MO, and the lesion was located in the posterior belly of the digastric muscle on computer tomography. A review of the literature showed that many cases of MO predominantly affected the medial pterygoid and masseter muscle and showed a history of previous trauma. The present case represents a rare case of nontraumatic MO in the uncommon location of the posterior belly of the digastric muscle.展开更多
Using a rabbit model leading to myositis in response to exercise-induced muscle overuse, we have previously observed that TNF-alpha is involved in the exercised muscle in early developing myositis as well as both ipsi...Using a rabbit model leading to myositis in response to exercise-induced muscle overuse, we have previously observed that TNF-alpha is involved in the exercised muscle in early developing myositis as well as both ipsiand contralaterally in the myositis which develops in response to a lengthened period of overuse. It is unknown if TNF-alpha can also be engaged contralaterally in early stages of myositis. The hypothesis was that this is the case. It was therefore evaluated whether the TNF-alpha system is early involved contralaterally. An experimental model of 1 week of overuse of the soleus muscle on one side leading to myositis was used, and in situ hybridization and immunohistochemistry were applied to study the expression patterns of TNF-alpha in the soleus muscle in the contralateral side. TNF-alpha was expressed in the myositis process which occurred contralaterally. There were thus TNF-alpha mRNA reactions in the cells of the inflammatory infiltrates, in blood vessel walls and in certain of the muscle fibers. Parts of the latter were necrotic fibers, whereas others were interpreted to be in a regenerative stage. TNF-alpha immunoreactions were seen for infiltrating white blood cells. The observations show that the TNF-alpha system is early involved in the cross-over effects that occur in response to unilateral muscle overuse leading to myositis bilaterally. TNF-alpha is likely to have pro-inflammatory and destructive effects but also to have effects in the muscle regenerative processes. The occurrence of an early involvement of the TNF-alpha system contralaterally to the injury side shows a new aspect of importance of this system in inflammation.展开更多
To the Editor:Dermatomyositis(DM)is a rare autoimmune disease characterized by specific cutaneous manifestations and proximal muscle weakness,in which abnormalities of nailfold capillary(NFC)and serum levels of vascul...To the Editor:Dermatomyositis(DM)is a rare autoimmune disease characterized by specific cutaneous manifestations and proximal muscle weakness,in which abnormalities of nailfold capillary(NFC)and serum levels of vascular biomarkers are common.Dynamic changes in vascular lesions play an important role in the occurrence and development of DM and reflect the disease activity and treatment efficacy.Due to the diversity of clinical manifestations in DM patients,vascular involvement is often under-evaluated,causing the treatment of refractory vascular damage challenging.This review aims to explore the mechanisms,clinical manifestations,and treatment options of vasculopathy in DM to achieve early recognition and improve prognosis.展开更多
Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characte...Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characterized by myositis,arthritis,interstitial lung disease,and Raynaud phenomenon.We present a case of a unique combination of deep morphea in a patient with anti-U1RNP myositis.Case presentation:A 64-year-old male with 5-year history of proximal muscle weakness,polyarthritis,Raynaud phenomenon,and dyspnea on multiple immunosuppressives presented with localized infiltrated,tight,and hyperpigmented plaques over the posterior thighs and mid-to-lower back developing over the last 2 years and limiting his movement.Autoimmune workup revealed a positive ANA,anti-U1RNP antibody,anti-Jo1 antibody,and anti-Ro52 antibody.Further workup showed restrictive lung disease,kidney disease,and arthritis.Patient was diagnosed with anti-U1RNP myositis.Skin biopsy of the back lesion showed deep morphea.Discussion:Association of deep morphea with anti-U1RNP myositis is not described prior in the literature.Treatment of morphea is challenging since the patient is already on immunosuppressive medications.The patient failed methotrexate prior and is currently on Mycophenolate mofetil and Deflazacort which are reported as potential treatment for morphea.Therefore,physical therapy plus topical Tacrolimus were suggested as an initial measure to preserve the range of motion of his posterior thighs and back.This is a case of progressive deep morphea developing in a patient with a unique autoimmune profile on immunosuppressive drugs.Conclusion:Anti-U1RNP myositis is a challenging diagnosis and should be always thought of in patients with positive anti-U1RNP,myositis,interstitial lung disease,arthritis,kidney disease,and Raynaud phenomenon.Moreover,deep morphea treatment in immunosuppressed patients is challenging and different measures should be considered.展开更多
Introduction:Statin-associated immune-mediated necrotizing myositis(IMNM)is a rare but distinct idiopathic inflammatory myopathy(IIM)that requires early recognition and intervention to prevent irreversible muscle dama...Introduction:Statin-associated immune-mediated necrotizing myositis(IMNM)is a rare but distinct idiopathic inflammatory myopathy(IIM)that requires early recognition and intervention to prevent irreversible muscle damage.It is typically characterized by active statin use,elevated creatinine kinase(CK)levels,proximal muscle weakness,and at times,a positive 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase(HMGCR)antibody.Treatment includes immediate discontinuation of the statin and may include corticosteroids,intravenous immunoglobulin(IVIG),and/or immunosuppressive therapy.Inclusion body myositis(IBM),another distinct IIM,also presents with elevated CK levels but with insidious onset of distal upper and proximal lower extremity weakness and is typically refractory to treatment.Case Description:A 64-year-old female patient presented with proximal muscle weakness,elevated CK levels,and a positive HMGCR antibody in the setting of statin use with muscle pathology suggestive of both statinassociated IMNM and IBM.She responded to subcutaneous methotrexate and a slow prednisone taper over several months,however,will require close monitoring for symptoms associated with either disease.Conclusion:In conclusion,we report a case of muscle weakness with muscle pathology demonstrating both statin-associated IMNM and IBM.This case highlights the importance of understanding the clinical and pathological features of statin-associated IMNM and IBM.展开更多
Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly ass...Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly associated with rapid progressive ILD.The aim of this study was to identify risk factors for prediction of different types of ILD in CADM.Methods:In this study,data of 108 inpatients with CADM were collected,including 87 with ILD.The baseline clinical data and laboratory parameters,including myositis-specific and associated antibodies and tumor-associated antigens were analyzed to identify risk factors for acute or subacute interstitial pneumonitis(A/SIP)and chronic interstitial pneumonitis(CIP).Results:In 87 patients with CADM-ILD,39(36.1%)were A/SIP,and 48(44.4%)were CIP.There were 22(20.4%)patients with asymptomatic ILD who were detected by routine high resolution computed tomography.Cytokeratin-19 fragment(CYFRA21-1)was significantly higher in CADM-ILD than that in CADM patients without ILD;carcinoembryonic antigen and neuron-specific enolase were significantly elevated in A/SIP than that in CIP.Patients with A/SIP had a higher positive rate of anti-melanoma differentiation-associated gene 5(MDA5),while patients with CIP had a higher positive rate of anti PL-12 and anti-Ro-52.Logistic regression analysis indicated that elevation of CYFRA21-1 was a risk factor for ILD,higher titer of anti-MDA5 indicated increased likelihood for A/SIP,and higher titer of anti-Ro-52 was also clearly associated with CIP.Conclusions:This study indicated that the prevalence of ILD was high in CADM.Asymptomatic ILD has been previously underestimated.Anti-MDA5 was a risk factor for the presence of A/SIP,and CYFRA21-1 was a risk factor for ILD.展开更多
基金Supported by Gansu Provincial Science and Technology Program Project,No.008108491108。
文摘BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized rehabilitation are also crucial.Here,we present a surgical strategy to prevent the recurrence of MO.CASE SUMMARY A 28-year-old female patient was admitted for the first time for a comminuted fracture of the left olecranon.However,incorrect postoperative rehabilitation resulted in the development of elbow joint stiffness with ectopic ossification,causing a loss of normal range of motion.The patient was diagnosed with MO based on physical examination,X-ray findings,and clinical presentation.We devised a surgical strategy to remove MO,followed by fixation with an Ilizarov frame,and implemented a scientifically reasonable rehabilitation plan.The surgery lasted for 3 h with an estimated blood loss of 45 mL.A drainage tube was placed after surgery,and fluid was aspirated through ultrasound-guided puncture.The patient experienced a significant reduction in joint stiffness after surgery.In the final follow-up at 9 mouths,there was evident improvement in the range of motion of the elbow joint,and no other symptoms were reported.CONCLUSION The Ilizarov frame is an advantageous surgical technique for facilitating rehabilitation after MO removal.It offers benefits such as passive recovery,individualized treatment,and prompt recovery.
文摘Rationale:Fever with myositis and rhabdomyolysis is a medical emergency requiring prompt diagnosis and management.Scrub typhus associated myositis with rhabdomyolysis is rare.Patient concerns:A 36-year-old female presented with intermittent fever up to 38.6℃,jaundice and progressive weakness of all four limbs.Diagnosis:Scrub typhus associated myositis and rhabdomyolysis.Intervention:Doxycycline 100 mg twice daily and injection of ceftriaxone 1 gm twice daily along with continuous intravenous fluids.Outcome:Fever resolved with normalization of liver function and recovery of muscle power.Lessons:Presence of myositis and rhabdomyolysis is uncommon in scrub typhus;high clinical suspicion should be kept in patients with atypical manifestations of scrub typhus.
文摘BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could be difficult to understand and a wrong diagnosis could lead to an improper cure.CASE SUMMARY We herein report the case of IMO in a healthy 69-year-old man.The patient presented with an abdominal mass in the left lower quadrant.A computed tomography scan showed an inhomogeneous mass with multiple calcifications.The patient underwent radical excision of the mass.Histopathological findings were compatible with MO.Five months later the patient showed a recurrence causing hemorrhagic shock due to intractable intralesional bleeding.The patients eventually died within three months since recurrence.CONCLUSION The case described could be classified as post-traumatic MO that developed close to the previously fractured iliac bone.The subsequent surgical procedure was ineffective and the disease rapidly recurred.The misleading intraoperative diagnosis led to improper surgical treatment with a dramatic evolution.
基金This work was supported by:Federico Foundation and FundacaoFaculdade de Medicina to S.K.S.
文摘Objective: With scant studies in the literature, little is known about the risk factors for tuberculosis in patients with dermatomyositis/polymyositis. Therefore, the aim of the present study was to analyze the predictive factors for tuberculosis development in dermatomyositis/polymyositis. Methods: This single-center, retrospective, cohort study initially included 290 patients with dermatomyositis/polymyositis, from 2002 to 2016. Tuberculosis (pulmonary and/or extra-pulmonary) was confirmed after dermatomyositis/polymyositis diagnosis in 12 patients (4.1%) (Tuberculosis+ group). For the control group (Tuberculosis−), 24 patients without tuberculosis were arbitrarily selected in the same period and matched for age, ethnicity, gender, age at disease diagnosis, disease duration and type (dermatomyositis or polymyositis). Results: Tuberculosis occurred for a median of 16 months after dermatomyositis/polymyositis diagnosis. Clinical, laboratory and treatment features were similar in Tuberculosis+ and Tuberculosis−groups (P > 0.05). However, previous pulmonary fibrosis in dermatomyositis/polymyositis was more prevalent in the Tuberculosis+ group (41.7 vs. 8.3%;P = 0.029). Moreover, on a multivariate logistic regression model, pulmonary fibrosis was significantly associated with Tuberculosis (Odds ratio: 9.59, 95% confidence interval: 1.17 - 78.82). Tuberculosis affected 3 dermatomyositis cases for every 1 polymyositis case, with predominantly pulmonary followed by extra-pulmonary involvement (pleura, cutaneous, muscular, joint, soft tissue and hematologic). Two or more sites were affected in 41.7% of cases. Conclusions: Previous pulmonary fibrosis in dermatomyositis/polymyositis was a predictive factor associated with tuberculosis development. Further studies are needed to confirm these results.
文摘BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history and nonspecific early clinical manifestations,which may lead to the failure of timely and effective diagnosis and treatment in some patients.We report the diagnosis and treatment of a child with nontraumatic myositis ossificans and summarize the clinical characteristics and diagnosis and treatment of the disease.CASE SUMMARY An 8-year-old girl first came to our hospital for more than a week with pain in the right lower limb.There was no history of trauma or strenuous activities.On physical examination,no mass on the right thigh was found,and the movement of the right lower extremity was limited.Ultrasonography showed synovitis of the hip,and bed rest was recommended.Three days later,the child’s pain persisted and worsened,accompanied by fever and other discomforts.She came to our hospital again and a mass was found on the right thigh with redness and swelling on the surface.The images showed a soft tissue tumor on the right thigh with calcification.Routine blood tests revealed that the inflammation index was significantly increased.In case of infection,the patient was given antibiotics,and the pain was relieved soon after,without fever.However,the right thigh mass persisted and hardened.The patient underwent incision biopsy more than 1 mo later,and the postoperative pathology showed nontraumatic myositis ossificans.After approximately 9 mo of observation,the tumor still persisted,which affected the life of the child,and then resection was performed.Since follow-up,there has been no recurrence.CONCLUSION Due to the difficulty in discerning a child’s medical history and the diverse early manifestations,it is difficult to diagnose nonossifying muscle disease in children in its early stage.Measures such as timely follow-up and periodic image monitoring are conducive to early diagnosis of the disease.The disease has a certain degree of self-limitation,and it can be observed and treated first.If the tumor persists in the later stage or affects functioning,then surgery is considered.
文摘In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with tofacitinib. Case Report: An adult female patient presented with definite dermatomyositis and with refractoriness to high doses of intravenous and oral glucocorticoids, intravenous human immunoglobulin, several immunosuppressive drugs (methotrexate, azathioprine, and leflunomide) and two previous immunobiological drugs (rituximab and abatacept). However, the patient had a good outcome with tofacitinib. Conclusions: Tofacitinib appears to be a promising alternative therapy for refractory dermatomyositis.
基金Supported by JSPS Grants-in-Aid for Scientific Research,No.16K19332Takeda medical research grants
文摘AIM To define clinical criteria to differentiate eosinophilic gastrointestinal disorder(Eo GD) in the esophagus. METHODS Our criteria were defined based on the analyses of the clinical presentation of eosinophilic esophagitis(Eo E), subepithelial eosinophilic esophagitis(s Eo E) and eosinophilic esophageal myositis(Eo EM), identified by endoscopy, manometry and serum immunoglobulin E levels(s-Ig E), in combination with histological and polymerase chain reaction analyses on esophageal tissue samples.RESULTS In five patients with Eo E, endoscopy revealed longitudinal furrows and white plaques in all, and fixed rings in two. In one patient with s Eo E and four with Eo EM, endoscopy showed luminal compression only. Using manometry, failed peristalsis was observed in patients with Eo E and s Eo E with some variation, while Eo EM was associated with hypercontractile or hypertensive peristalsis, with elevated s-Ig E. Histology revealed the following eosinophils per high-power field values. Eo E = 41.4 ± 7.9 in the epithelium and 2.3 ± 1.5 in the subepithelium; s Eo E = 3 in the epithelium and 35 in the subepithelium(conventional biopsy); Eo EM = none in the epithelium, 10.7 ± 11.7 in the subepithelium(conventional biopsy or endoscopic mucosal resection) and 46.8 ± 16.5 in the muscularis propria(peroral esophageal muscle biopsy). Presence of dilated epithelial intercellular space and downward papillae elongation were specific to Eo E. Eotaxin-3, IL-5 and IL-13 were overexpressed in Eo E.CONCLUSION Based on clinical and histological data, we identified criteria, which differentiated between Eo E, s Eo E and Eo EM, and reflected a different pathogenesis between these esophageal Eo GDs.
文摘Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis. A 33-year-old Moroccan woman presented with a 2-month history of left inguinal inflammatory pain with limping gait, MRI examination suggested a malignant neoplasm such as soft tissue osteosarcoma. A diagnosis of myositis ossificans was made by incisional biopsy. Conservative management with clinical and radiological follow up of 19 months confirm the diagnosis. The symptoms resolved within seven months. Myositis ossificans should be considered by clinicians as a possible diagnosis for a soft tissue lesion.
基金Supported by Yonghua Mou’s Famous Doctor Studio,No.2020-42.
文摘BACKGROUND Proliferative myositis is a rare benign tumor that is typically self-limiting and does not become malignant.It can be cured by simple resection without reported recurrence.Due to its rapid growth,hard structure and ill-defined borders,it can however be mistaken for malignant tumors such as sarcomas.CASE SUMMARY We investigate the case of a 64-year-old male with proliferative myositis of the abdominal wall,who was preoperatively administered a needle aspiration biopsy and given a simple excision and patch repair.We then compared it with other similar cases to determine the effectiveness of this treatment method.CONCLUSION Resection with follow-up observation has shown to be an effective treatment method for proliferative myositis.To avoid unnecessarily extended or destructive resection,a thorough and conclusive diagnosis is crucial,which requires adequate imaging and pathological knowledge.
文摘BACKGROUND: Infective myositis is rare. The retrospective report of clinical data and symptoms from one patient with infective myositis will hopefully provide more information for clinicians in the diagnosis of this disease. METHODS: A male patient, 65 years old, was admitted with "fever and muscle pain since four days ago, accompanied by inertia of all limbs for one day", to the First Department of Neurology, Guangdong Provincial Hospital of Traditional Chinese Medicine on April 25th, 2005. Following admission, a history of diseases was record, and detailed physical and neurological examinations were performed. During the examination, symmetrical myasthenia appeared, tendon reflex disappeared, and creatine kinase levels were increased 500 times higher than normal. The patient was primarily diagnosed with hypokalemic periodic paralysis, myositis, Guillain-Barre syndrome, and upper respiratory infection. Subsequently, the patient was treated with the following: cefuroxime for infection, potassium supplements, breviscapin for promoting blood circulation through the removal of stasis, and ATP/CO-A for myocardial nutrition. Antiviral drugs were not administered. However, laboratory samples were continuously monitored. Creatine kinase levels decreased to normal, and muscle pain was obviously relieved following antibiotics treatment. The results led to a final diagnosis of infective myositis. RESULTS: Four days after treatment (April 29th), muscular tenderness and throat congestion were obviously improved (+/-). The neurological examination showed the patient was conscious, cooperated with treatment, and had normal intellect. No abnormalities of the cranial nerve were observed upon examination. Proximal and distal muscle strength and muscular tensions of the four limbs were all normal. Reflexes of the right biceps brachii muscle and its tendon were decreased, and knee tendon and Achilles tendon reflex were not induced. A Babinski reflex was not detected. The neurological examination presented no abnormalities, and the related creatine kinases were within normal range by re-examination at 2 weeks, and l and 3 months after discharge. CONCLUSION: A diagnosis of infective myositis should be considered for patients with clear prodromes of infection, that include symptoms of periodic paralysis and elevated creatine kinase levels. Treatment with sufficient antibiotics can obtain good results.
基金Supported by Natural Science Foundation of Zhejiang Province,China,No.LQ19H160019Zhejiang Provincial Basic Public Welfare Research Project,China,No.LGF19H140006+1 种基金Zhejiang TCM Science and Technology Plan,China,No.2018ZA071,and No.2019ZA069Medical Scientific Research Foundation of Zhejiang Province,China,No.2019KY379.
文摘BACKGROUND Trismus is a common problem with various causes.Any abnormal conditions of relevant anatomic structures that disturb the free movement of the jaw might provoke trismus.Trismus has a detrimental effect on the quality of life.The outcome of this abnormality is critically dependent on timely diagnosis and treatment,and it is difficult to identify the true origin in some cases.We present a rare case of trismus due to fungal myositis in the pterygoid muscle,excluding any other possible pathogenesis.CASE SUMMARY The patient presented with a 2-mo history of restricted mouth opening.Computed tomography showed obvious enlargement of the left pterygoid muscles.Furthermore,the patient had trismus without obvious predisposing causes.The primary diagnosis was pterygoid myosarcoma.Consequently,lesionectomy of the left pterygoid muscle was performed.Intraoperative frozen biopsy implied the possibility of an uncommon infection.Postoperative pathologic examination confirmed myositis and necrosis in the pterygoid muscle.Fungi were detected in both muscle tissue and surrounding necrotic tissue.The patient recovered well with antifungal therapy and mouth opening exercises.The rarity of fungal myositis may be responsible for the misdiagnosis.Although the origin of pathogenic fungi is still unknown,we believe that both hematogenous spread and local invasion could be the most likely sources.To the best of our knowledge,this is therst case in the literature that reported fungal myositis in pterygoid muscles as the only reason that results in trismus.CONCLUSION Surgeons should remain vigilant to the possibility of trismus originating from fungal myositis.
文摘Myositis ossificans circumscribed is a bone and cartilage heterotopic non neoplastic proliferation inside the soft tissues. It is a benign focal heterotopic ossification process of soft tissues, and a rare disorder that occurs spontaneously or after local trauma. Clinical and radiographic appearances are quite hustler. A careful histological examination of biopsy straightens diagnosis is necessary. There is no consensus in support (surgical or/and medical). From Benign prognosis, evolution of this pathology is usually favorable. The authors report a case of giant myositis ossificans circumscribed post-traumatic localized on gluteus and adductor muscles of the right hip on a 26-year-old man. Through a review of literature the mechanism, the diagnostic methods and therapeutic will be discussed.
文摘The transobturator tape (TOT) procedure is generally felt to be a safer surgical alternative to the ten sion-free vaginal tape procedure for women with stress urinary incontinence. We report a case of adductor internus myositis not associated with infection following the TOT procedure. To our knowledge this is the first case of this type reported in the literature. A 43 year old lady underwent a straightforward elective TOT procedure. There were no intraoperative complications. Immediately following the procedure she complained of pain in her right thigh. MRI confirmed abnormal oedema within the antero-inferior aspect of the right obturator internus muscle consistent with myositis secondary to tape insertion. The tape was removed the following day in theatre, following which her pain resolved. This case highlights a previously unreported complication as a result of the TOT procedure.
文摘Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokine variables in rituximab-treated refractory myositis patients. Methods: We analyzed data for 200 myositis patients (76 with adult polymyositis (PM), 76 with adult dermatomyositis (DM), and 48 with juvenile (DM)) in the rituximab in myositis trial. Clinical improvement is defined as the change from baseline to 24 weeks in Physician Global Visual Analog Scale (VAS). We analyze the association of baseline variables with improvements: demographics, myositis subtype, clinical and laboratory parameters, autoantibody status, and interferon (IFN)- regulated chemokines. Multivariable linear regression models are developed by using stepwise variable selection methods. Results: A “base” multivariable model to predict improvement with clinical and laboratory variablesonly is built with modest predictive ability (adjusted R2 = 0.21). This model includes two significant factors at baseline: Physician Global VAS and Muscle Disease Activity VAS. A “final” multivariable model to predict improvement including non-standard laboratory measures is developed and demonstrated better predictive ability (adjusted R2 = 0.32). This model includes Physician Global VAS, IFN chemokine score and IL-2 levels. The “final” model explained 11% more variability than the “base” model. Conclusions: Changes in disease activity over time following treatment with rituximab in refractory myositis can be predicted. These models can be clinically useful to optimize treatment selection in myositis.
文摘Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of mastication. The patient was a 70-year-old Japanese woman who presented with a tumorous mass in the right parotid area and no history of trauma. The surgically excised tumorous lesion consisted of proliferating fibroblastic cells with no atypia in the central portion, and a formation of trabecular osteoid and/or bony tissue in the peripheral portion as the so-called “zonal phenomenon”. The final histological diagnosis was MO, and the lesion was located in the posterior belly of the digastric muscle on computer tomography. A review of the literature showed that many cases of MO predominantly affected the medial pterygoid and masseter muscle and showed a history of previous trauma. The present case represents a rare case of nontraumatic MO in the uncommon location of the posterior belly of the digastric muscle.
基金Financial support has been obtained from the Faculty of Medicine,Umea University,and the J.C.Kempe and Seth M.Kempe Memorial Foundations,Ornskoldsvik
文摘Using a rabbit model leading to myositis in response to exercise-induced muscle overuse, we have previously observed that TNF-alpha is involved in the exercised muscle in early developing myositis as well as both ipsiand contralaterally in the myositis which develops in response to a lengthened period of overuse. It is unknown if TNF-alpha can also be engaged contralaterally in early stages of myositis. The hypothesis was that this is the case. It was therefore evaluated whether the TNF-alpha system is early involved contralaterally. An experimental model of 1 week of overuse of the soleus muscle on one side leading to myositis was used, and in situ hybridization and immunohistochemistry were applied to study the expression patterns of TNF-alpha in the soleus muscle in the contralateral side. TNF-alpha was expressed in the myositis process which occurred contralaterally. There were thus TNF-alpha mRNA reactions in the cells of the inflammatory infiltrates, in blood vessel walls and in certain of the muscle fibers. Parts of the latter were necrotic fibers, whereas others were interpreted to be in a regenerative stage. TNF-alpha immunoreactions were seen for infiltrating white blood cells. The observations show that the TNF-alpha system is early involved in the cross-over effects that occur in response to unilateral muscle overuse leading to myositis bilaterally. TNF-alpha is likely to have pro-inflammatory and destructive effects but also to have effects in the muscle regenerative processes. The occurrence of an early involvement of the TNF-alpha system contralaterally to the injury side shows a new aspect of importance of this system in inflammation.
基金supported by grants from the China International Medical Foundation(No.Z-2018-40)the Nantong Science and Technology Bureau Project(Nos.MSZ18108,JCZ20058,and JCZ2022026)the Special Clinical Basic Research Key Project of Nantong University(No.2019JZ001)
文摘To the Editor:Dermatomyositis(DM)is a rare autoimmune disease characterized by specific cutaneous manifestations and proximal muscle weakness,in which abnormalities of nailfold capillary(NFC)and serum levels of vascular biomarkers are common.Dynamic changes in vascular lesions play an important role in the occurrence and development of DM and reflect the disease activity and treatment efficacy.Due to the diversity of clinical manifestations in DM patients,vascular involvement is often under-evaluated,causing the treatment of refractory vascular damage challenging.This review aims to explore the mechanisms,clinical manifestations,and treatment options of vasculopathy in DM to achieve early recognition and improve prognosis.
文摘Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characterized by myositis,arthritis,interstitial lung disease,and Raynaud phenomenon.We present a case of a unique combination of deep morphea in a patient with anti-U1RNP myositis.Case presentation:A 64-year-old male with 5-year history of proximal muscle weakness,polyarthritis,Raynaud phenomenon,and dyspnea on multiple immunosuppressives presented with localized infiltrated,tight,and hyperpigmented plaques over the posterior thighs and mid-to-lower back developing over the last 2 years and limiting his movement.Autoimmune workup revealed a positive ANA,anti-U1RNP antibody,anti-Jo1 antibody,and anti-Ro52 antibody.Further workup showed restrictive lung disease,kidney disease,and arthritis.Patient was diagnosed with anti-U1RNP myositis.Skin biopsy of the back lesion showed deep morphea.Discussion:Association of deep morphea with anti-U1RNP myositis is not described prior in the literature.Treatment of morphea is challenging since the patient is already on immunosuppressive medications.The patient failed methotrexate prior and is currently on Mycophenolate mofetil and Deflazacort which are reported as potential treatment for morphea.Therefore,physical therapy plus topical Tacrolimus were suggested as an initial measure to preserve the range of motion of his posterior thighs and back.This is a case of progressive deep morphea developing in a patient with a unique autoimmune profile on immunosuppressive drugs.Conclusion:Anti-U1RNP myositis is a challenging diagnosis and should be always thought of in patients with positive anti-U1RNP,myositis,interstitial lung disease,arthritis,kidney disease,and Raynaud phenomenon.Moreover,deep morphea treatment in immunosuppressed patients is challenging and different measures should be considered.
文摘Introduction:Statin-associated immune-mediated necrotizing myositis(IMNM)is a rare but distinct idiopathic inflammatory myopathy(IIM)that requires early recognition and intervention to prevent irreversible muscle damage.It is typically characterized by active statin use,elevated creatinine kinase(CK)levels,proximal muscle weakness,and at times,a positive 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase(HMGCR)antibody.Treatment includes immediate discontinuation of the statin and may include corticosteroids,intravenous immunoglobulin(IVIG),and/or immunosuppressive therapy.Inclusion body myositis(IBM),another distinct IIM,also presents with elevated CK levels but with insidious onset of distal upper and proximal lower extremity weakness and is typically refractory to treatment.Case Description:A 64-year-old female patient presented with proximal muscle weakness,elevated CK levels,and a positive HMGCR antibody in the setting of statin use with muscle pathology suggestive of both statinassociated IMNM and IBM.She responded to subcutaneous methotrexate and a slow prednisone taper over several months,however,will require close monitoring for symptoms associated with either disease.Conclusion:In conclusion,we report a case of muscle weakness with muscle pathology demonstrating both statin-associated IMNM and IBM.This case highlights the importance of understanding the clinical and pathological features of statin-associated IMNM and IBM.
基金This work was supported by grants from the National Natural Science Foundation of China(Nos.81801615 and 81871289).
文摘Background:Clinically amyopathic dermatomyositis(CADM)is a unique sub-type of idiopathic inflammatory myopathies with a high prevalence of interstitial lung disease(ILD).Poor prognosis of the patients was strongly associated with rapid progressive ILD.The aim of this study was to identify risk factors for prediction of different types of ILD in CADM.Methods:In this study,data of 108 inpatients with CADM were collected,including 87 with ILD.The baseline clinical data and laboratory parameters,including myositis-specific and associated antibodies and tumor-associated antigens were analyzed to identify risk factors for acute or subacute interstitial pneumonitis(A/SIP)and chronic interstitial pneumonitis(CIP).Results:In 87 patients with CADM-ILD,39(36.1%)were A/SIP,and 48(44.4%)were CIP.There were 22(20.4%)patients with asymptomatic ILD who were detected by routine high resolution computed tomography.Cytokeratin-19 fragment(CYFRA21-1)was significantly higher in CADM-ILD than that in CADM patients without ILD;carcinoembryonic antigen and neuron-specific enolase were significantly elevated in A/SIP than that in CIP.Patients with A/SIP had a higher positive rate of anti-melanoma differentiation-associated gene 5(MDA5),while patients with CIP had a higher positive rate of anti PL-12 and anti-Ro-52.Logistic regression analysis indicated that elevation of CYFRA21-1 was a risk factor for ILD,higher titer of anti-MDA5 indicated increased likelihood for A/SIP,and higher titer of anti-Ro-52 was also clearly associated with CIP.Conclusions:This study indicated that the prevalence of ILD was high in CADM.Asymptomatic ILD has been previously underestimated.Anti-MDA5 was a risk factor for the presence of A/SIP,and CYFRA21-1 was a risk factor for ILD.