BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatou...BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.展开更多
Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally co...Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally compatible with the causative agent (whether there is a deep venous catheter or not). On the other hand and according to the 2012 Revised Chapel Hill Classification, granulomatosis with polyangiitis GPA is classified as a vasculitis associated with antineutrophil cytoplasmic antibodies ANCA. It is a systemic disease characterized by the anatomopathological aspect of granuloma. We report the case of a patient who presented an atypical and a very rare revealing mode of GPA which was a bronchopulmonary candidiasis complicated by candidemia. Despite its controversy, the combination in the acute phase of antifungal treatment based on intravenous voriconazole and glucocorticoid therapy has made it possible to control candidemia and calm vasculitis.展开更多
BACKGROUND Tocilizumab is a humanized monoclonal antibody against the interleukin-6(IL-6)receptor that is commonly used to treat large vessel vasculitis and antineutrophil cytoplasmic antibody-related small vessel vas...BACKGROUND Tocilizumab is a humanized monoclonal antibody against the interleukin-6(IL-6)receptor that is commonly used to treat large vessel vasculitis and antineutrophil cytoplasmic antibody-related small vessel vasculitis.However,tocilizumab in combination with glucocorticoids for successfully treating granulomatosis with polyangiitis(GPA)has rarely been reported.CASE SUMMARY Here,we report a 40-year-old male patient who suffered GPA for 4 years.He was treated with multiple rounds of drugs,including cyclophosphamide,Tripterygium wilfordii,mycophenolate mofetil,and belimumab,with no improvement.In addition,he exhibited persistently high IL-6 levels.After tocilizumab treatment,his symptoms improved,and his inflammatory marker levels returned to normal.CONCLUSION Tocilizumab may be effective for treating GPA.展开更多
目的嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)中以血管炎为主要特征和以嗜酸性粒细胞浸润为主要特征的两种亚型分类标准尚未明确,本研究探讨EGPA两种亚型的准确、简明分类方式。方法回顾性收集2000...目的嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)中以血管炎为主要特征和以嗜酸性粒细胞浸润为主要特征的两种亚型分类标准尚未明确,本研究探讨EGPA两种亚型的准确、简明分类方式。方法回顾性收集2000年1月—2023年11月北京协和医院呼吸内科、全科医学科、风湿免疫科等多科确诊的EGPA患者临床资料,将患者根据单纯抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibodies,ANCA)、ANCA与组织病理活检结果相结合以及MIRRA标准三种分类方法分为不同的阳性组和阴性组,分析患者的临床特征。结果共纳入245例患者。根据ANCA结果分类时,阳性组51例,阴性组194例,相较于阴性组,阳性组患者的年龄(60.9比55.4)、NEUT%(59.12比51.87)、PLT(312.94比264.64)、ESR(55.40比27.62)、CRP(46.51比23.57)均较高,上述指标均达显著统计学意义;阳性组肾脏系统(47.1%比25.8%)和神经系统(72.5%比58.2%)的受累比例也显著高于阴性组。根据ANCA结果和组织病理活检结果分类时,阳性组88例,阴性组157例,两组间的分析结果与第一种分类方法一致。根据MIRRA标准分类时,MIRRA标准组患者161例,MIRRA非标准组患者84例,MIRRA标准组的NEUT%(55.39比49.58)显著高于MIRRA非标准组,而LY%(18.57比21.30)和HGB(128.80比136.36)显著低于MIRRA非标准组。结论根据ANCA结果可将EGPA患者分为血管炎亚型和嗜酸性粒细胞浸润亚型;尽管ANCA联合组织病理活检结果也可明确区分血管炎亚型与嗜酸性粒细胞浸润亚型,但由于病理活检存在风险,需根据患者的具体情况进行个体化评估;而MIRRA标准不能准确区分两类亚型。展开更多
BACKGROUND Toxic epidermal necrolysis(TEN)is a life-threatening dermatological emergency mainly induced by drug hypersensitivity reactions.Standard management includes discontinuation of culprit drug and application o...BACKGROUND Toxic epidermal necrolysis(TEN)is a life-threatening dermatological emergency mainly induced by drug hypersensitivity reactions.Standard management includes discontinuation of culprit drug and application of immunomodulatory therapy.However,mortality remains high due to complications like septic shock and multiorgan failures.Innovative approaches for skin care are crucial.This report introduces borneol-gypsum,a traditional Chinese drug but a novel dressing serving as an adjuvant of TEN therapy,might significantly improve skin conditions and patient outcomes in TEN.CASE SUMMARY A 38-year-old woman diagnosed with eosinophilic granulomatosis with polyangiitis experienced gangrenous complications and motor nerve involvement.After initial treatment of high-dose corticosteroids and cyclophosphamide,symptom of foot drop improved,absolute eosinophil counts decreased,while limb pain sustained.Duloxetine was added to alleviate her symptom.Subsequently,TEN developed.Additional topical application of borneol-gypsum dressing not only protected the skin lesions from infection but also significantly eased localized pain.This approach demonstrated its merit in TEN management by promoting skin healing and potentially reducing infection risks.CONCLUSION Borneol-gypsum dressing is a promising adjuvant that could significantly improve TEN management,skin regeneration,and patient comfort.展开更多
AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRIS...AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRISMA guidelines.We searched Medline,LILACS,Virtual Health Library,and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range(<18 years),with no language limitations.All relevant articles were accessed in full text.The manual search included references of retrieved articles.We extracted data on patients’characteristics,disease characteristics,association with other diseases,and treatment.We analyzed the data and reported the results in tables and text.RESULTS:We retrieved 173 reports of OFG in children.Mean age at onset was 11.1±3.8 years(range:2.0-18years).Prevalence in males was significant higher than in females(P<0.001),with a male:female ratio of 2:1.Gastrointestinal signs or symptoms were present in 26.0%of children at the time of OFG diagnosis.Overall,70/173(40.4%)children received a concomitant diagnosis of Crohn’s disease.In about half(51.4%)of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease,with a mean time between the two diagnoses of 13.1±11.6 mo(range:3-36 mo).Overall,21/173(12.1%)of the children with OFG had perianal disease,while 11/173(6.4%)had a family history of Crohn’s disease.Both perianal disease and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG[relative risk(RR)=3.10,95%confidence interval(CI):2.46-3.90;RR=2.74,95%CI:2.24-3.36,P<0.0001 for both).Treatment of OFG included steroids(70.8%of children)and other immunosuppressive drugs(42.7%),such as azathioprine,thalidomide and infliximab.CONCLUSION:High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease.展开更多
We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pa...We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.展开更多
A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener&#...A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.展开更多
Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease...Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.展开更多
BACKGROUND Although cyclophosphamide(CPA)is the key drug for the treatment of autoimmune diseases including vasculitides,it has some well-known adverse effects,such as myelosuppression,hemorrhagic cystitis,infertility...BACKGROUND Although cyclophosphamide(CPA)is the key drug for the treatment of autoimmune diseases including vasculitides,it has some well-known adverse effects,such as myelosuppression,hemorrhagic cystitis,infertility,and infection.However,CPA-associated severe enteritis is a rare adverse effect,and only one case with a lethal clinical course has been reported.Therefore,the appropriate management of patients with CPA-associated severe enteritis is unclear.CASE SUMMARY We present the case of a 61-year-old woman diagnosed with granulomatosis with polyangiitis based on the presence of symptoms in ear,lung,and,kidney with positive myeloperoxidase-antineutrophil cytoplasmic antibody.She received pulsed methylprednisolone followed by prednisolone 55 mg/d and intravenous CPA at a dose of 500 mg/mo.Ten days after the second course of intravenous CPA,she developed nausea,vomiting,and diarrhea,and was admitted to the hospital.Laboratory testing revealed hypoalbuminemia,suggesting proteinlosing enteropathy.Computed tomography revealed wall thickening of the stomach,small intestine,and colon with contrast enhancement on the lumen side.Antibiotics and immunosuppressive therapy were not effective,and the patient’s enteritis did not improve for>4 mo.Because her condition became seriously exhausted,corticosteroids were tapered and supportive therapies including intravenous hyperalimentation,replenishment of albumin and gamma globulin,plasma exchange,and infection control were continued.These supportive therapies improved her condition,and her enteritis gradually regressed.She was finally discharged 7 mo later.CONCLUSION Immediate discontinuation of CPA and intensive supportive therapy are crucial for the survival of patients with CPA-associated severe enteritis.展开更多
A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of ...A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.展开更多
BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel...BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.展开更多
BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulm...BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.展开更多
BACKGROUND Granulomatosis with polyangiitis is a necrotizing inflammation of small andmedium-sized vessels accompanied by formation of granuloma, involvement ofprimary granulomatous upper and lower respiratory tracts,...BACKGROUND Granulomatosis with polyangiitis is a necrotizing inflammation of small andmedium-sized vessels accompanied by formation of granuloma, involvement ofprimary granulomatous upper and lower respiratory tracts, glomerulonephritis,and vasculitis of small vessels.CASE SUMMARY Herein, we described a case of a 52-year-old man admitted with pulmonarynodules and high fever. Autoantibody workup revealed that the patient waspositive for c-anti-neutrophil cytoplasmic antibodies and proteinase-3 antineutrophilcytoplasmic antibodies. Pulmonary biopsies revealed a localgranulomatous structure. The patient received therapy with methylprednisoloneand intravenous immunoglobulin, and his clinical symptoms improved.CONCLUSION Intravenous immunoglobulin may act on granulomatosis with polyangiitis similarto immunosuppressants.展开更多
BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the...BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the arterioles and venules.When the coronary arteries are invaded,it can lead to acute myocardial infarction(AMI),acute heart failure,and other manifestations that often lead to death in the absence of timely treatment.CASE SUMMARY A 69-year-old man was admitted to the emergency department due to chest pain for more than 1 h.He had a past history of bronchial asthma and chronic obstructive pulmonary disease and was diagnosed with AMI and heart failure.Thrombus aspiration of the left circumflex artery and percutaneous transluminal coronary angioplasty were performed immediately.After surgery,the patient was admitted to the intensive care unit.The patient developed eosinophilia,and medical history taking revealed fatigue of both thighs 1 mo prior.Local skin numbness and manifestations of peripheral nerve involvement were found on the lateral side of the right thigh.Skin biopsy of the lower limbs pathologically confirmed EGPA.The patient was treated with methylprednisolone combined with intravenous immunoglobulin and was discharged after 21 d.On follow-up at 7 d after discharge,heart failure recurred.The condition improved after cardiotonic and diuretic treatment,and the patient was discharged.CONCLUSION Asthma,impaired cardiac function,and eosinophilia are indicative of EGPA.Delayed diagnosis often leads to heart involvement and death.展开更多
BACKGROUND Eosinophilic granulomatosis polyangiitis(EGPA)is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma;however,it can rarely manifest with cardiac involvement su...BACKGROUND Eosinophilic granulomatosis polyangiitis(EGPA)is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma;however,it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade.Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare.Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes.CASE SUMMARY 52-year-old woman with no past medical history presented with progressive dyspnea and dry cough.On physical exam she had a pericardial friction rub and bilateral rales.Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89%oxygen saturation.On laboratory exam,she had 45%peripheral eosinophilia,troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL.TTE confirmed a large pericardial effusion and tamponade physiology.She underwent urgent pericardial window procedure.Pericardial and lung biopsy demonstrated eosinophilic infiltration.Based on the American College of Radiology guidelines,the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade.She was treated with steroid taper and mepolizumab.CONCLUSION This case highlights that when isolated pericardial involvement occurs in EGPA,diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.展开更多
基金Supported by The Research Project of Zhejiang Chinese Medical University,No.2023JKZKTS33.
文摘BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.
文摘Candidemia is defined as being a yeast infection confirmed by the presence of at least one positive Candida blood culture. It is a life threatening infection causing high mortality. The clinical signs are generally compatible with the causative agent (whether there is a deep venous catheter or not). On the other hand and according to the 2012 Revised Chapel Hill Classification, granulomatosis with polyangiitis GPA is classified as a vasculitis associated with antineutrophil cytoplasmic antibodies ANCA. It is a systemic disease characterized by the anatomopathological aspect of granuloma. We report the case of a patient who presented an atypical and a very rare revealing mode of GPA which was a bronchopulmonary candidiasis complicated by candidemia. Despite its controversy, the combination in the acute phase of antifungal treatment based on intravenous voriconazole and glucocorticoid therapy has made it possible to control candidemia and calm vasculitis.
文摘BACKGROUND Tocilizumab is a humanized monoclonal antibody against the interleukin-6(IL-6)receptor that is commonly used to treat large vessel vasculitis and antineutrophil cytoplasmic antibody-related small vessel vasculitis.However,tocilizumab in combination with glucocorticoids for successfully treating granulomatosis with polyangiitis(GPA)has rarely been reported.CASE SUMMARY Here,we report a 40-year-old male patient who suffered GPA for 4 years.He was treated with multiple rounds of drugs,including cyclophosphamide,Tripterygium wilfordii,mycophenolate mofetil,and belimumab,with no improvement.In addition,he exhibited persistently high IL-6 levels.After tocilizumab treatment,his symptoms improved,and his inflammatory marker levels returned to normal.CONCLUSION Tocilizumab may be effective for treating GPA.
文摘BACKGROUND Toxic epidermal necrolysis(TEN)is a life-threatening dermatological emergency mainly induced by drug hypersensitivity reactions.Standard management includes discontinuation of culprit drug and application of immunomodulatory therapy.However,mortality remains high due to complications like septic shock and multiorgan failures.Innovative approaches for skin care are crucial.This report introduces borneol-gypsum,a traditional Chinese drug but a novel dressing serving as an adjuvant of TEN therapy,might significantly improve skin conditions and patient outcomes in TEN.CASE SUMMARY A 38-year-old woman diagnosed with eosinophilic granulomatosis with polyangiitis experienced gangrenous complications and motor nerve involvement.After initial treatment of high-dose corticosteroids and cyclophosphamide,symptom of foot drop improved,absolute eosinophil counts decreased,while limb pain sustained.Duloxetine was added to alleviate her symptom.Subsequently,TEN developed.Additional topical application of borneol-gypsum dressing not only protected the skin lesions from infection but also significantly eased localized pain.This approach demonstrated its merit in TEN management by promoting skin healing and potentially reducing infection risks.CONCLUSION Borneol-gypsum dressing is a promising adjuvant that could significantly improve TEN management,skin regeneration,and patient comfort.
文摘AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRISMA guidelines.We searched Medline,LILACS,Virtual Health Library,and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range(<18 years),with no language limitations.All relevant articles were accessed in full text.The manual search included references of retrieved articles.We extracted data on patients’characteristics,disease characteristics,association with other diseases,and treatment.We analyzed the data and reported the results in tables and text.RESULTS:We retrieved 173 reports of OFG in children.Mean age at onset was 11.1±3.8 years(range:2.0-18years).Prevalence in males was significant higher than in females(P<0.001),with a male:female ratio of 2:1.Gastrointestinal signs or symptoms were present in 26.0%of children at the time of OFG diagnosis.Overall,70/173(40.4%)children received a concomitant diagnosis of Crohn’s disease.In about half(51.4%)of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease,with a mean time between the two diagnoses of 13.1±11.6 mo(range:3-36 mo).Overall,21/173(12.1%)of the children with OFG had perianal disease,while 11/173(6.4%)had a family history of Crohn’s disease.Both perianal disease and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG[relative risk(RR)=3.10,95%confidence interval(CI):2.46-3.90;RR=2.74,95%CI:2.24-3.36,P<0.0001 for both).Treatment of OFG included steroids(70.8%of children)and other immunosuppressive drugs(42.7%),such as azathioprine,thalidomide and infliximab.CONCLUSION:High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease.
文摘We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.
文摘A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.
文摘Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.
基金Funding for Scientific Research(Funding for Academic Research),No.18K16136.
文摘BACKGROUND Although cyclophosphamide(CPA)is the key drug for the treatment of autoimmune diseases including vasculitides,it has some well-known adverse effects,such as myelosuppression,hemorrhagic cystitis,infertility,and infection.However,CPA-associated severe enteritis is a rare adverse effect,and only one case with a lethal clinical course has been reported.Therefore,the appropriate management of patients with CPA-associated severe enteritis is unclear.CASE SUMMARY We present the case of a 61-year-old woman diagnosed with granulomatosis with polyangiitis based on the presence of symptoms in ear,lung,and,kidney with positive myeloperoxidase-antineutrophil cytoplasmic antibody.She received pulsed methylprednisolone followed by prednisolone 55 mg/d and intravenous CPA at a dose of 500 mg/mo.Ten days after the second course of intravenous CPA,she developed nausea,vomiting,and diarrhea,and was admitted to the hospital.Laboratory testing revealed hypoalbuminemia,suggesting proteinlosing enteropathy.Computed tomography revealed wall thickening of the stomach,small intestine,and colon with contrast enhancement on the lumen side.Antibiotics and immunosuppressive therapy were not effective,and the patient’s enteritis did not improve for>4 mo.Because her condition became seriously exhausted,corticosteroids were tapered and supportive therapies including intravenous hyperalimentation,replenishment of albumin and gamma globulin,plasma exchange,and infection control were continued.These supportive therapies improved her condition,and her enteritis gradually regressed.She was finally discharged 7 mo later.CONCLUSION Immediate discontinuation of CPA and intensive supportive therapy are crucial for the survival of patients with CPA-associated severe enteritis.
文摘A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
文摘BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.
基金Supported by Science and Technology department of Sichuan Province,No.2020YFS0105West China Second University Hospital of Sichuan University,No.KL036.
文摘BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.
文摘BACKGROUND Granulomatosis with polyangiitis is a necrotizing inflammation of small andmedium-sized vessels accompanied by formation of granuloma, involvement ofprimary granulomatous upper and lower respiratory tracts, glomerulonephritis,and vasculitis of small vessels.CASE SUMMARY Herein, we described a case of a 52-year-old man admitted with pulmonarynodules and high fever. Autoantibody workup revealed that the patient waspositive for c-anti-neutrophil cytoplasmic antibodies and proteinase-3 antineutrophilcytoplasmic antibodies. Pulmonary biopsies revealed a localgranulomatous structure. The patient received therapy with methylprednisoloneand intravenous immunoglobulin, and his clinical symptoms improved.CONCLUSION Intravenous immunoglobulin may act on granulomatosis with polyangiitis similarto immunosuppressants.
文摘BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the arterioles and venules.When the coronary arteries are invaded,it can lead to acute myocardial infarction(AMI),acute heart failure,and other manifestations that often lead to death in the absence of timely treatment.CASE SUMMARY A 69-year-old man was admitted to the emergency department due to chest pain for more than 1 h.He had a past history of bronchial asthma and chronic obstructive pulmonary disease and was diagnosed with AMI and heart failure.Thrombus aspiration of the left circumflex artery and percutaneous transluminal coronary angioplasty were performed immediately.After surgery,the patient was admitted to the intensive care unit.The patient developed eosinophilia,and medical history taking revealed fatigue of both thighs 1 mo prior.Local skin numbness and manifestations of peripheral nerve involvement were found on the lateral side of the right thigh.Skin biopsy of the lower limbs pathologically confirmed EGPA.The patient was treated with methylprednisolone combined with intravenous immunoglobulin and was discharged after 21 d.On follow-up at 7 d after discharge,heart failure recurred.The condition improved after cardiotonic and diuretic treatment,and the patient was discharged.CONCLUSION Asthma,impaired cardiac function,and eosinophilia are indicative of EGPA.Delayed diagnosis often leads to heart involvement and death.
文摘BACKGROUND Eosinophilic granulomatosis polyangiitis(EGPA)is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma;however,it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade.Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare.Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes.CASE SUMMARY 52-year-old woman with no past medical history presented with progressive dyspnea and dry cough.On physical exam she had a pericardial friction rub and bilateral rales.Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89%oxygen saturation.On laboratory exam,she had 45%peripheral eosinophilia,troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL.TTE confirmed a large pericardial effusion and tamponade physiology.She underwent urgent pericardial window procedure.Pericardial and lung biopsy demonstrated eosinophilic infiltration.Based on the American College of Radiology guidelines,the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade.She was treated with steroid taper and mepolizumab.CONCLUSION This case highlights that when isolated pericardial involvement occurs in EGPA,diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.
