BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurre...BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.展开更多
Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM an...Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.展开更多
BACKGROUND Natural killer(NK)/T cell lymphoma is a rare and highly aggressive malignant tumor,and is a special form of non-Hodgkin's lymphoma.Although extranodal involvement is frequently found in tissues such as ...BACKGROUND Natural killer(NK)/T cell lymphoma is a rare and highly aggressive malignant tumor,and is a special form of non-Hodgkin's lymphoma.Although extranodal involvement is frequently found in tissues such as the skin,testicular and gastrointestinal tract etc,its presence in skeletal muscle has scarcely been reported in the literature.CASE SUMMARY We report a case of extranodal NK/T cell lymphoma with muscle swelling as the first clinical manifestation.A 42-year-old man,who initially presented with localized swelling in the double lower extremities,demonstrated gradual facial and eyelid swelling,and his imaging results showed multiple sites of muscle damage throughout the body.The final pathological results suggested NK/T cell lymphoma,and immunohistochemistry showed CD20(-),CD3(+),CD30(+),CD56(-),EBER(+),Ki67(60%),TIA-1(+)and CD68(±)staining.The muscle swelling significantly improved after treatment with chemotherapy regimens.CONCLUSION This disease is difficult to diagnose and highly invasive,and should be included in the differential diagnosis of unexplained muscle swelling.展开更多
Introduction: Polymyositis (PM) is a type of inflammatory myopathy that is associated with a broad range of malignant disorders. An association of PM with synchronous carcinoma of the bladder and prostate is extremely...Introduction: Polymyositis (PM) is a type of inflammatory myopathy that is associated with a broad range of malignant disorders. An association of PM with synchronous carcinoma of the bladder and prostate is extremely rare. Case: A 65-year-old man admitted to hematology with complaints of severe progressive weakness of lower extremities, hematuria and irritative urinary symptoms lasting for a month. The hemogram and erythrocyte sedimentation rate were normal. ALT was normal but AST was 405.56 U/l. There was marked elevation of serum creatine kinase (CK) and lactate dehydrogenase, which were 14,065.15 U/l and 1267.50 U/l, respectively. PSA was 4.28 and DRE was positive. The abdominal ultrasound revealed a 24 × 20 mm soft tissue echogenicity lesion at the right wall and a 35 × 21 mm soft tissue echogenicity lesion at the left wall of the bladder. The rest of the abdominal viscera were normal. Computed tomography found, two 6 mm solid lesions at left anterolateral and a 18 × 12 mm solid lesion at inferoanterior bladder wall in addition to the above findings. Patient counseled to us. We resected all of the bladder masses with transurethral (TUR-BT) way and pathology revealed T2 high grade bladder tumor. Two days after TUR-BT, we performed a trans rectal ultrasonography guided prostate biopsy and pathology revealed a Gleason 3 + 4 prostate cancer. EMG showed sensorimotor polyneuropathy at the lower extremities, sustaining polymyozitis. Biopsy of the right peroneus brevis muscle showed no vasculitis with low grade neurologic changes. We offered to perform a radical cystoprostatectomy operation but the patient prefered chemotherapy. Two months after his initial presentation at the second cure of the chemotherapy all muscle weaknesses showed a dramatic regression. Conclusion: This case report indicates that both bladder carcinoma and prostate cancer should be kept in mind in elderly PM patients presenting with lower urinary tract symptoms and hematuria.展开更多
Diffuse myositis with progression to rhabdomynlysis has been reported in association with wide range of viral infections.We report a case of polvmvosilis-like svndrome complicated by rhabdomyolysis secondary to brucel...Diffuse myositis with progression to rhabdomynlysis has been reported in association with wide range of viral infections.We report a case of polvmvosilis-like svndrome complicated by rhabdomyolysis secondary to brucellosis.This case report thus contributes yet another atypical presentation to a disease already infamous for its protean manifestations.展开更多
Polymyositis is an illness characterized by inflammation of the muscle(s) with uncertain etiological causes. Most of the physicians think that viral infection, disturbances of immune functions and/or pathological vasc...Polymyositis is an illness characterized by inflammation of the muscle(s) with uncertain etiological causes. Most of the physicians think that viral infection, disturbances of immune functions and/or pathological vascular changes might be responsible for the condition.展开更多
OBJECTIVE： Polymyositis （PM） mainly involves proximal limb and trunk muscles. Ocular muscles are not affected, except in rare cases with both PM and myasthenia gravis （MG）. Thus, the results of laboratory examina...OBJECTIVE： Polymyositis （PM） mainly involves proximal limb and trunk muscles. Ocular muscles are not affected, except in rare cases with both PM and myasthenia gravis （MG）. Thus, the results of laboratory examinations in such a patient deserve to be reported. METHODS： To analyze the clinical, imaging and pathology datas on a 65-year-old woman patient with PM with complex symptoms, who presented mainly ophthalmoplegia. The patient consented to all examinations and the hospital Ethics Committee approved the study. The laboratory examinations included creatine kinase （CK）, ENA, tumor marker, function of thyroid, cranial MRI, and electromyogram （EMG）. Biopsy of the left quadriceps femoris was performed, frozen specimens were stained with hematoxylin and eosin, ATPase, NADH tetrazolium reductase, periodic acid Schiff, oil red O, modified Gomory trichrome and MHC-I, to investigate the pathology of muscle fibers RESULTS： Laboratory results showed： CK, 108.32μ kat/L; antinuclear antibody： （＋）; ENA, （-）; tumor marker, （-）; normal thyroid function, MRI showed no abnormal signals in brain and extraocular muscles. Electromyography of the bilateral deltoid, biceps brachii, musculus quadriceps fexoris, anterior tibialis showed fibrillation potentials, positive potentials and short-duration, small-amplitude polyphasic potentials on voluntary movements with a full interference pattern on mild exertion. Repetitive stimulation did not result in any increment or decrement in these potentials. A muscle biopsy of the left quadriceps femoris showed many small round muscle fibers without peripheral bundle distribution and apparent myofiber degeneration, necrosis and phagocytosis. There were several focal lymphocyte infiltrations. MHC-I immunohistochemical staining was positive in most fibers revealing inflammatory infiltration of normal fibers with MHC-I expression. CONCLUSION： This patient showed increased CK, typical triad of myopathy in EMG, and apparent degeneration and necrosis in biopsy of quadriceps femoris. Therefore, the diagnosis of PM and involvement of extraocular muscles were definite.展开更多
AIM:To evaluate detailed anterior segment parameters of patients with idiopathic inflammatory myopathies(IIM),including polymyositis(PM),and dermatomyositis(DM),and to clarify the associations between these data and c...AIM:To evaluate detailed anterior segment parameters of patients with idiopathic inflammatory myopathies(IIM),including polymyositis(PM),and dermatomyositis(DM),and to clarify the associations between these data and clinical variables of IIM.METHODS:Totally 57 PM,41 DM patients and 62 controls were enrolled in this cross-sectional,observational,case-control study.All study participants underwent Pentacam evaluation.Laboratory investigations consisted of different antibody assays,while extramuscular clinical assessments included Raynaud’s phenomenon,dysphagia,interstitial lung disease,arthritis/arthralgia,and weight loss.Objective signs and subjective symptoms of dry eye disease(DED)were also evaluated.RESULTS:All pachymetric parameters[center,apex,thinnest and maximal keratometry(Kmax)]and corneal volume(CV)of both sides of PM patients proved to be significantly lower.Some pachymetric data were also noticed as significantly decreased compared to those of controls.Several significant differences were traced between anterior segment values and extramuscular manifestations of myositis,largely in case of arthritis/arthralgia and weight loss,whereas associations between anterior segment parameters and antibodies were weak.Objective clinical tests of DED were also significantly decreased in IIM patients.CONCLUSION:The results suggest that all IIM patients have thinner corneas compared with those of controls,and decreased corneal parameters are significantly associated with the occurrence of some extramuscular manifestations.In addition,IIM patients tend to develop objective signs of DED.展开更多
An immunocytochemical study was performed in 6 peripheral nerve specimens from 6 cases of polymyositis. The results revealed that depositions of IgG, IgM, IgA and C3 were found in the epineurium, perineurium and the w...An immunocytochemical study was performed in 6 peripheral nerve specimens from 6 cases of polymyositis. The results revealed that depositions of IgG, IgM, IgA and C3 were found in the epineurium, perineurium and the walls of capillaries. These findings demonstrated that depositions of immunoglobulins and the complement-mediated immunoreaction may play an important role in pathogenesis of polymyositis with peripheral nerve involvements.展开更多
BACKGROUND Idiopathic inflammatory myopathies(IIM)are systemic autoimmune disorders such as dermatomyositis(DM),polymyositis(PM),inclusion body myopathy,and autoimmune necrotizing myopathy that,similar to osteoarthrit...BACKGROUND Idiopathic inflammatory myopathies(IIM)are systemic autoimmune disorders such as dermatomyositis(DM),polymyositis(PM),inclusion body myopathy,and autoimmune necrotizing myopathy that,similar to osteoarthritis,affect quality of life and activities of daily living.Moreover,these patients are often burdened with chronic pain and disability;however,the outcomes and risk of total hip arthroplasty(THA)in this patient population remain unclear.AIM To evaluate 90-d complications and costs in patients with these conditions.METHODS A retrospective case control study was designed by accessing data from the Medicare dataset available on the PearlDiver server.Patients with IIM,here,those with DM and PM were matched based on possible confounding variables to a cohort without these diseases and with the same 10-year risk of mortality as defined by the Charlson Comorbidity Index Score(CCI).Univariate and multivariate analysis were performed to evaluate complications and t-tests to evaluate 90-d Medicare reimbursements as markers of costs after THA.RESULTS The total sample was 1090 patients with each cohort comprised of 545.Females were 74.9%of the population.The mean CCI was 5.89(SD 2.11).Those with IIM had increased rates of pneumonia[odds ratio(OR)1.45,P<0.001]and pulmonary embolism(OR 1.46,P=0.035)and decreased hematoma risks(OR 0.58,P=0.00).90-d costs were on average$1411 greater for those with IIM yet not significantly different(P=0.034).CONCLUSION Patients with IIM have an increased 90-d rate of pneumonia and pulmonary embolism concomitant with a decreased hematoma rate consistent with their procoagulatory state.Further attention to increased resource utilization in these patients is also warranted.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
Amyloidosis is the result of fibrous and insoluble amyloid protein deposition in extracellular spaces of tissues and organs, including the skin. Through the analysis of medical records, clinical condition and laborato...Amyloidosis is the result of fibrous and insoluble amyloid protein deposition in extracellular spaces of tissues and organs, including the skin. Through the analysis of medical records, clinical condition and laboratory tests, this article proposes a rare case report of an adult patient with symptoms and diagnosis of polymyositis, histopathology of nodular amyloidosis with skin lesions suggestive of lupus. The patient is still being followed at the dermatology clinic of Valença Medical School, in order to improve the prognosis and prevent the progression of existing symptoms.展开更多
文摘BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.
文摘Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.
基金Supported by the National Natural Science Foundation of China,No.81400978
文摘BACKGROUND Natural killer(NK)/T cell lymphoma is a rare and highly aggressive malignant tumor,and is a special form of non-Hodgkin's lymphoma.Although extranodal involvement is frequently found in tissues such as the skin,testicular and gastrointestinal tract etc,its presence in skeletal muscle has scarcely been reported in the literature.CASE SUMMARY We report a case of extranodal NK/T cell lymphoma with muscle swelling as the first clinical manifestation.A 42-year-old man,who initially presented with localized swelling in the double lower extremities,demonstrated gradual facial and eyelid swelling,and his imaging results showed multiple sites of muscle damage throughout the body.The final pathological results suggested NK/T cell lymphoma,and immunohistochemistry showed CD20(-),CD3(+),CD30(+),CD56(-),EBER(+),Ki67(60%),TIA-1(+)and CD68(±)staining.The muscle swelling significantly improved after treatment with chemotherapy regimens.CONCLUSION This disease is difficult to diagnose and highly invasive,and should be included in the differential diagnosis of unexplained muscle swelling.
文摘Introduction: Polymyositis (PM) is a type of inflammatory myopathy that is associated with a broad range of malignant disorders. An association of PM with synchronous carcinoma of the bladder and prostate is extremely rare. Case: A 65-year-old man admitted to hematology with complaints of severe progressive weakness of lower extremities, hematuria and irritative urinary symptoms lasting for a month. The hemogram and erythrocyte sedimentation rate were normal. ALT was normal but AST was 405.56 U/l. There was marked elevation of serum creatine kinase (CK) and lactate dehydrogenase, which were 14,065.15 U/l and 1267.50 U/l, respectively. PSA was 4.28 and DRE was positive. The abdominal ultrasound revealed a 24 × 20 mm soft tissue echogenicity lesion at the right wall and a 35 × 21 mm soft tissue echogenicity lesion at the left wall of the bladder. The rest of the abdominal viscera were normal. Computed tomography found, two 6 mm solid lesions at left anterolateral and a 18 × 12 mm solid lesion at inferoanterior bladder wall in addition to the above findings. Patient counseled to us. We resected all of the bladder masses with transurethral (TUR-BT) way and pathology revealed T2 high grade bladder tumor. Two days after TUR-BT, we performed a trans rectal ultrasonography guided prostate biopsy and pathology revealed a Gleason 3 + 4 prostate cancer. EMG showed sensorimotor polyneuropathy at the lower extremities, sustaining polymyozitis. Biopsy of the right peroneus brevis muscle showed no vasculitis with low grade neurologic changes. We offered to perform a radical cystoprostatectomy operation but the patient prefered chemotherapy. Two months after his initial presentation at the second cure of the chemotherapy all muscle weaknesses showed a dramatic regression. Conclusion: This case report indicates that both bladder carcinoma and prostate cancer should be kept in mind in elderly PM patients presenting with lower urinary tract symptoms and hematuria.
文摘Diffuse myositis with progression to rhabdomynlysis has been reported in association with wide range of viral infections.We report a case of polvmvosilis-like svndrome complicated by rhabdomyolysis secondary to brucellosis.This case report thus contributes yet another atypical presentation to a disease already infamous for its protean manifestations.
文摘Polymyositis is an illness characterized by inflammation of the muscle(s) with uncertain etiological causes. Most of the physicians think that viral infection, disturbances of immune functions and/or pathological vascular changes might be responsible for the condition.
文摘OBJECTIVE： Polymyositis （PM） mainly involves proximal limb and trunk muscles. Ocular muscles are not affected, except in rare cases with both PM and myasthenia gravis （MG）. Thus, the results of laboratory examinations in such a patient deserve to be reported. METHODS： To analyze the clinical, imaging and pathology datas on a 65-year-old woman patient with PM with complex symptoms, who presented mainly ophthalmoplegia. The patient consented to all examinations and the hospital Ethics Committee approved the study. The laboratory examinations included creatine kinase （CK）, ENA, tumor marker, function of thyroid, cranial MRI, and electromyogram （EMG）. Biopsy of the left quadriceps femoris was performed, frozen specimens were stained with hematoxylin and eosin, ATPase, NADH tetrazolium reductase, periodic acid Schiff, oil red O, modified Gomory trichrome and MHC-I, to investigate the pathology of muscle fibers RESULTS： Laboratory results showed： CK, 108.32μ kat/L; antinuclear antibody： （＋）; ENA, （-）; tumor marker, （-）; normal thyroid function, MRI showed no abnormal signals in brain and extraocular muscles. Electromyography of the bilateral deltoid, biceps brachii, musculus quadriceps fexoris, anterior tibialis showed fibrillation potentials, positive potentials and short-duration, small-amplitude polyphasic potentials on voluntary movements with a full interference pattern on mild exertion. Repetitive stimulation did not result in any increment or decrement in these potentials. A muscle biopsy of the left quadriceps femoris showed many small round muscle fibers without peripheral bundle distribution and apparent myofiber degeneration, necrosis and phagocytosis. There were several focal lymphocyte infiltrations. MHC-I immunohistochemical staining was positive in most fibers revealing inflammatory infiltration of normal fibers with MHC-I expression. CONCLUSION： This patient showed increased CK, typical triad of myopathy in EMG, and apparent degeneration and necrosis in biopsy of quadriceps femoris. Therefore, the diagnosis of PM and involvement of extraocular muscles were definite.
文摘AIM:To evaluate detailed anterior segment parameters of patients with idiopathic inflammatory myopathies(IIM),including polymyositis(PM),and dermatomyositis(DM),and to clarify the associations between these data and clinical variables of IIM.METHODS:Totally 57 PM,41 DM patients and 62 controls were enrolled in this cross-sectional,observational,case-control study.All study participants underwent Pentacam evaluation.Laboratory investigations consisted of different antibody assays,while extramuscular clinical assessments included Raynaud’s phenomenon,dysphagia,interstitial lung disease,arthritis/arthralgia,and weight loss.Objective signs and subjective symptoms of dry eye disease(DED)were also evaluated.RESULTS:All pachymetric parameters[center,apex,thinnest and maximal keratometry(Kmax)]and corneal volume(CV)of both sides of PM patients proved to be significantly lower.Some pachymetric data were also noticed as significantly decreased compared to those of controls.Several significant differences were traced between anterior segment values and extramuscular manifestations of myositis,largely in case of arthritis/arthralgia and weight loss,whereas associations between anterior segment parameters and antibodies were weak.Objective clinical tests of DED were also significantly decreased in IIM patients.CONCLUSION:The results suggest that all IIM patients have thinner corneas compared with those of controls,and decreased corneal parameters are significantly associated with the occurrence of some extramuscular manifestations.In addition,IIM patients tend to develop objective signs of DED.
文摘An immunocytochemical study was performed in 6 peripheral nerve specimens from 6 cases of polymyositis. The results revealed that depositions of IgG, IgM, IgA and C3 were found in the epineurium, perineurium and the walls of capillaries. These findings demonstrated that depositions of immunoglobulins and the complement-mediated immunoreaction may play an important role in pathogenesis of polymyositis with peripheral nerve involvements.
文摘BACKGROUND Idiopathic inflammatory myopathies(IIM)are systemic autoimmune disorders such as dermatomyositis(DM),polymyositis(PM),inclusion body myopathy,and autoimmune necrotizing myopathy that,similar to osteoarthritis,affect quality of life and activities of daily living.Moreover,these patients are often burdened with chronic pain and disability;however,the outcomes and risk of total hip arthroplasty(THA)in this patient population remain unclear.AIM To evaluate 90-d complications and costs in patients with these conditions.METHODS A retrospective case control study was designed by accessing data from the Medicare dataset available on the PearlDiver server.Patients with IIM,here,those with DM and PM were matched based on possible confounding variables to a cohort without these diseases and with the same 10-year risk of mortality as defined by the Charlson Comorbidity Index Score(CCI).Univariate and multivariate analysis were performed to evaluate complications and t-tests to evaluate 90-d Medicare reimbursements as markers of costs after THA.RESULTS The total sample was 1090 patients with each cohort comprised of 545.Females were 74.9%of the population.The mean CCI was 5.89(SD 2.11).Those with IIM had increased rates of pneumonia[odds ratio(OR)1.45,P<0.001]and pulmonary embolism(OR 1.46,P=0.035)and decreased hematoma risks(OR 0.58,P=0.00).90-d costs were on average$1411 greater for those with IIM yet not significantly different(P=0.034).CONCLUSION Patients with IIM have an increased 90-d rate of pneumonia and pulmonary embolism concomitant with a decreased hematoma rate consistent with their procoagulatory state.Further attention to increased resource utilization in these patients is also warranted.
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Amyloidosis is the result of fibrous and insoluble amyloid protein deposition in extracellular spaces of tissues and organs, including the skin. Through the analysis of medical records, clinical condition and laboratory tests, this article proposes a rare case report of an adult patient with symptoms and diagnosis of polymyositis, histopathology of nodular amyloidosis with skin lesions suggestive of lupus. The patient is still being followed at the dermatology clinic of Valença Medical School, in order to improve the prognosis and prevent the progression of existing symptoms.
