Modern rheumatology case reports最新文献

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Organ-limited ANCA-associated orbital inflammatory disease: a diagnostic challenge. 器官受限的anca相关眼窝炎性疾病:一个诊断挑战。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag045
Aron M Sebhat, Adam G Hidad, Grant Moore, Kirsten Da Silva, Tamim Sultani, Jessica Wong, Anna M Stagner, Trent Smith, Chandana Keshavamurthy
{"title":"Organ-limited ANCA-associated orbital inflammatory disease: a diagnostic challenge.","authors":"Aron M Sebhat, Adam G Hidad, Grant Moore, Kirsten Da Silva, Tamim Sultani, Jessica Wong, Anna M Stagner, Trent Smith, Chandana Keshavamurthy","doi":"10.1093/mrcr/rxag045","DOIUrl":"10.1093/mrcr/rxag045","url":null,"abstract":"<p><p>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that cause inflammation and necrosis of small- to medium-sized blood vessels. It most commonly affects the kidneys and lungs, leading to complications such as rapidly progressive glomerulonephritis and pulmonary haemorrhage. Ocular involvement occurs in up to 50% of patients with AAV, most commonly presenting as scleritis, episcleritis, or orbital inflammatory disease (also known as orbital pseudotumor). Other ocular findings may include keratitis, uveitis, or retinal vasculitis. Eyelid necrosis with destructive orbital inflammation, however, is extremely rare and poses a significant diagnostic challenge because its presentation can mimic infections, malignancies, or other inflammatory orbital conditions. Prompt recognition is critical to prevent irreversible tissue damage and vision loss. We report the case of a 74-year-old Hispanic woman with multiple vascular risk factors who presented with recurrent pustules of the right upper eyelid that progressed to full-thickness eyelid necrosis, medial orbital mass effect, and corneal compromise. Initial management was directed toward presumed orbital cellulitis; however, orbital imaging revealed a destructive extraconal mass with mass effect and associated venous stasis. Sequential biopsies demonstrated extensive necrosis with histiocytoid granulomatous inflammation and focal features of chronic vasculitis. Negative stains and cultures excluded infectious and neoplastic causes. After thorough evaluation and exclusion of alternative etiologies, the findings were most consistent with ANCA-negative ANCA-AAV. The patient was treated with high-dose intravenous corticosteroids and rituximab induction, resulting in stabilization of orbital disease and preservation of vision. This case highlights the potential for destructive orbital vasculitis to masquerade as infection or malignancy and underscores the importance of considering AAV in atypical orbital presentations. Prompt recognition and initiation of immunosuppressive therapy are critical to prevent irreversible morbidity.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148311097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Progressive macrocytic anaemia over 14 years leading to the diagnosis of VEXAS syndrome in a patient initially diagnosed with adult-onset Still's disease. 进行性大细胞性贫血超过14年导致诊断为VEXAS综合征的患者最初诊断为成人发病斯蒂尔氏病:一个病例报告。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag067
Kazuya Abe, Kentaro Takahashi, Ayako Matsuki, Yoshitaka Zaimoku, Takeshi Umibe
{"title":"Progressive macrocytic anaemia over 14 years leading to the diagnosis of VEXAS syndrome in a patient initially diagnosed with adult-onset Still's disease.","authors":"Kazuya Abe, Kentaro Takahashi, Ayako Matsuki, Yoshitaka Zaimoku, Takeshi Umibe","doi":"10.1093/mrcr/rxag067","DOIUrl":"10.1093/mrcr/rxag067","url":null,"abstract":"<p><p>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently recognised autoinflammatory disease caused by somatic mutations in UBA1. Its clinical features include recurrent fever, systemic inflammation affecting multiple organs, and progressive haematologic abnormalities such as macrocytic anaemia. Because these manifestations overlap with other autoinflammatory diseases, including adult-onset Still's disease, the diagnosis can be challenging. In this case report, we describe a patient initially diagnosed with adult-onset Still's disease who was ultimately found to have VEXAS syndrome 5 years later due to progressive macrocytic anaemia. The patient developed recurrent fever and skin rash but did not have macrocytic anaemia at onset. Over the subsequent 5 years, his mean corpuscular volume progressively increased. Bone marrow biopsy revealed no evidence of malignancy but showed vacuoles in myeloid and erythroid progenitor cells. Based on these findings, VEXAS syndrome was suspected, and UBA1 testing identified the pathogenic p.Met41Thr variant, thereby confirming the diagnosis. This case highlights that macrocytic anaemia may not be present in the early phase of VEXAS syndrome. In patients diagnosed with adult-onset Still's disease who later develop or progressively worsen macrocytic anaemia, clinicians should consider VEXAS syndrome and perform UBA1 variant testing.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148427651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful prosthesis salvage using continuous local antibiotic perfusion for prosthetic joint infection following revision total elbow arthroplasty in a patient with rheumatoid arthritis. 类风湿性关节炎患者全肘关节置换术后使用持续局部抗生素灌注(CLAP)成功修复假体:一例报告。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag077
Risa Arai, Yuya Takakubo, Juji Ito, Tomohiro Uno, Masahiro Maruyama, Yasushi Naganuma, Ryuta Oishi, Jun Nagai, Yoshihiro Wanezaki, Akemi Suzuki, Hiroshi Satake, Michiaki Takagi
{"title":"Successful prosthesis salvage using continuous local antibiotic perfusion for prosthetic joint infection following revision total elbow arthroplasty in a patient with rheumatoid arthritis.","authors":"Risa Arai, Yuya Takakubo, Juji Ito, Tomohiro Uno, Masahiro Maruyama, Yasushi Naganuma, Ryuta Oishi, Jun Nagai, Yoshihiro Wanezaki, Akemi Suzuki, Hiroshi Satake, Michiaki Takagi","doi":"10.1093/mrcr/rxag077","DOIUrl":"10.1093/mrcr/rxag077","url":null,"abstract":"<p><strong>Introduction: </strong>Prosthetic joint infection (PJI) represents a serious and challenging complication in a patient with rheumatoid arthritis. We report a case of PJI following revision total elbow arthroplasty with rheumatoid arthritis that was successfully managed with debridement, antibiotics, and implant retention augmented by continuous local antibiotic perfusion (CLAP).</p><p><strong>Case presentation: </strong>A 69-year-old woman with a 39-year history of rheumatoid arthritis previously underwent revision total elbow arthroplasty for aseptic loosening. Two years following the revision surgery, she developed left elbow swelling, pain, and erythema, resulting in a diagnosis of PJI. Because the implant remained stable without evidence of loosening, an emergency debridement, antibiotics, and implant retention procedure was performed in conjunction with CLAP in attempt to salvage the prosthesis. Intraoperative cultures were positive for methicillin-susceptible Staphylococcus aureus. Following surgery, the patient received targeted systemic antibiotic therapy alongside continuous local gentamicin perfusion via the CLAP system. Local perfusion was discontinued on postoperative Day 18, and oral antibiotic suppressive therapy was continued for 3 months. At the 2-year follow-up examination, the patient showed no clinical evidence of recurrent infection and had maintained her preoperative elbow range of motion.</p><p><strong>Conclusion: </strong>The integration of CLAP with the debridement, antibiotics, and implant retention procedure represents a promising, prosthesis-preserving therapeutic strategy for managing PJI following revision total elbow arthroplasty.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148671790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis: A case report and literature review. 慢性非细菌性骨髓炎/慢性复发性多灶性骨髓炎1例报告并文献复习。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag081
Christophe Persad, Niamh Donaldson, Simona Huica
{"title":"Chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis: A case report and literature review.","authors":"Christophe Persad, Niamh Donaldson, Simona Huica","doi":"10.1093/mrcr/rxag081","DOIUrl":"10.1093/mrcr/rxag081","url":null,"abstract":"<p><p>Chronic non-bacterial osteomyelitis/chronic recurrent multifocal osteomyelitis is an autoinflammatory, sterile bone disorder that predominantly affects children but can also occur in adults. The nonspecific nature of its presentation, as well as a relative lack of awareness of the condition, often results in significant diagnostic delay. In this article, we present the case of a female patient who began experiencing sternal pain at the age of 14, initially thought to be due to costochondritis but ultimately discovered to be secondary to chronic non-bacterial osteomyelitis/chronic recurrent multifocal osteomyelitis. We demonstrate her long and tortuous diagnostic journey, including key imaging and bone biopsy findings. We also report good clinical and radiographic response to tumour necrosis factor-α inhibitors, despite having to switch from adalimumab to etanercept and then to methotrexate due to side effects and anti-drug antibody production.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148803885","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Planned pregnancy after remission of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated interstitial lung disease: More than 4 years of postpartum follow-up. 抗mda5抗体阳性皮肌炎相关间质性肺病缓解后计划妊娠:产后随访4年以上1例
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag085
Daisuke Kobayashi, Sayuri Takamura, Shunsuke Sakai, Yoko Wada, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Kuroda, Suguru Yamamoto
{"title":"Planned pregnancy after remission of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated interstitial lung disease: More than 4 years of postpartum follow-up.","authors":"Daisuke Kobayashi, Sayuri Takamura, Shunsuke Sakai, Yoko Wada, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Kuroda, Suguru Yamamoto","doi":"10.1093/mrcr/rxag085","DOIUrl":"10.1093/mrcr/rxag085","url":null,"abstract":"<p><p>Pregnancy after anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated interstitial lung disease is rarely described, and preconception management remains uncertain. A 29-year-old woman presented with fever, arthralgia, cough, characteristic Gottron's and inverse Gottron's papules, normal muscle enzyme levels, mild interstitial lung abnormalities on high-resolution computed tomography, and positivity for anti-melanoma differentiation-associated gene 5 antibody. Due to the potential risk of rapid pulmonary deterioration, she received prednisolone, a calcineurin inhibitor, and intravenous cyclophosphamide, followed by maintenance immunosuppression. Mycophenolate mofetil was discontinued as part of preconception planning 24 months after disease onset. After two biochemical pregnancies that ended before ultrasonographic confirmation of a gestational sac, a subsequent pregnancy was confirmed at ~36 months after disease onset while she was receiving prednisolone 10 mg/day and tacrolimus. The pregnancy was not complicated by relapse of dermatomyositis or interstitial lung disease, a hypertensive disorder, gestational diabetes, foetal growth restriction, or non-reassuring foetal status. She delivered a healthy female infant at 38 weeks and 3 days of gestation by vaginal delivery. At >4 years postpartum, she remained relapse-free, and the antibody remained negative. This case suggests that a planned pregnancy may be feasible in carefully selected patients after sustained preconception disease control, the discontinuation of teratogenic drugs, and the continuation of pregnancy-compatible immunosuppression under multidisciplinary surveillance; serial anti-melanoma differentiation-associated gene 5 antibody measurements may provide adjunctive information when interpreted alongside clinical, pulmonary function, and imaging assessments.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148852243","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Add-on anifrolumab for residual mucocutaneous and systemic manifestations during cyclophosphamide therapy in severe neuropsychiatric lupus: a case report. 重症神经精神性狼疮环磷酰胺治疗期间残留的粘膜和全身表现的附加Anifrolumab: 1例报告。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag092
Mariko Hoshino, Toshihiko Shiga, Yumi Morimoto, Jinhai Li, Yuji Nozaki, Koji Kinoshita, Shinya Rai
{"title":"Add-on anifrolumab for residual mucocutaneous and systemic manifestations during cyclophosphamide therapy in severe neuropsychiatric lupus: a case report.","authors":"Mariko Hoshino, Toshihiko Shiga, Yumi Morimoto, Jinhai Li, Yuji Nozaki, Koji Kinoshita, Shinya Rai","doi":"10.1093/mrcr/rxag092","DOIUrl":"10.1093/mrcr/rxag092","url":null,"abstract":"<p><p>A 29-year-old woman presented with fever, malar rash, and alopecia. Laboratory evaluation demonstrated a high-titre antinuclear antibody of 1:5120, hypocomplementaemia, and positivity for anti-double-stranded DNA and anti-Smith antibodies, leading to a diagnosis of systemic lupus erythematosus (SLE). During the clinical course, she developed progressive peripheral neuropathy with inability to walk. Intravenous methylprednisolone pulse therapy and intravenous immunoglobulin were administered, but neurological improvement was limited, and intravenous cyclophosphamide (IVCY) therapy was initiated. Subsequently, she developed central nervous system manifestations of neuropsychiatric SLE and received an additional course of intravenous methylprednisolone. Central nervous system manifestations gradually improved during ongoing IVCY therapy; however, fever, rash, and alopecia persisted at the time of transfer to our hospital. Because fever persisted despite antibiotic therapy and mucocutaneous manifestations and hypocomplementaemia progressively worsened, residual SLE activity was considered clinically relevant. Anifrolumab (ANF) was initiated on hospital Day 28, coinciding with completion of antibiotic therapy. Fever resolved the following day, while the mucocutaneous manifestations and hypocomplementaemia subsequently showed gradual and sustained improvement. No serious infectious or other adverse events were observed after ANF initiation during ongoing IVCY therapy. Reports describing add-on ANF during ongoing IVCY therapy for severe SLE are extremely limited. This case highlights the potential role of ANF as an adjunctive therapy for persistent systemic and mucocutaneous manifestations suggestive of interferon-associated disease activity during ongoing cyclophosphamide-based intensive immunosuppressive treatment.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149077359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute respiratory deterioration in rheumatoid arthritis-associated interstitial lung disease associated with pulmonary Mycobacterium abscessus infection: A case report. 类风湿性关节炎相关肺间质性疾病伴肺脓肿分枝杆菌感染的急性呼吸系统恶化1例报告
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag091
Tomoko Oshima, Yutaro Nakamura, Eriko Iwaizumi, Kentaro Yamamoto, Manabu Ato, Tatsuru Eifuku, Yasuhiro Ito, Hisano Ohba, Kaoru Fujita, Miho Kanai, Seiichi Miwa, Masahiro Shirai
{"title":"Acute respiratory deterioration in rheumatoid arthritis-associated interstitial lung disease associated with pulmonary Mycobacterium abscessus infection: A case report.","authors":"Tomoko Oshima, Yutaro Nakamura, Eriko Iwaizumi, Kentaro Yamamoto, Manabu Ato, Tatsuru Eifuku, Yasuhiro Ito, Hisano Ohba, Kaoru Fujita, Miho Kanai, Seiichi Miwa, Masahiro Shirai","doi":"10.1093/mrcr/rxag091","DOIUrl":"10.1093/mrcr/rxag091","url":null,"abstract":"<p><p>Nontuberculous mycobacteria (NTM) are important pathogens in patients with chronic lung diseases, including rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Although acute exacerbations of ILD associated with NTM infection have been reported sporadically, cases involving pulmonary Mycobacterium abscessus infection remain extremely limited. We describe a fatal case of acute respiratory deterioration in a patient with RA-ILD following pulmonary infection with M. abscessus. A 76-year-old man with longstanding RA-ILD developed pulmonary NTM disease initially caused by Mycobacterium avium complex and subsequently complicated by M. abscessus infection. Intensive multidrug induction therapy led to transient clinical and radiographic improvement. However, 2 weeks after discharge, he was readmitted with rapidly progressive hypoxemic respiratory failure and newly developed bilateral diffuse ground-glass opacities on chest computed tomography. A comprehensive evaluation did not identify alternative infectious aetiologies, and the overall findings were considered consistent with clinically suspected acute exacerbation of RA-ILD associated with pulmonary M. abscessus infection, although progressive pulmonary M. abscessus disease could not be completely excluded. Despite corticosteroid pulse therapy and broad antimicrobial coverage, the patient died 6 days after readmission. Subsequent analysis confirmed M. abscessus subsp. abscessus with resistance to macrolides and imipenem/cilastatin. This case highlights the diagnostic challenges in distinguishing acute exacerbation of RA-ILD from progressive pulmonary M. abscessus disease, raises the possibility that M. abscessus infection may be associated with acute respiratory deterioration in patients with underlying RA-ILD, and underscores the importance of early recognition and optimal management of NTM infections in this high-risk population.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083362","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anti-centromere antibody-positive primary Sjögren's disease with exudative pleural effusion: A case report and literature review. 抗着丝粒抗体阳性的原发性Sjögren病伴渗出性胸腔积液1例报告并文献复习。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag078
Masashi Uchikawa, Masako Tsukamoto, Kiichi Sugito, Shinya Asatani, Masahiro Nishihara, Kumiko Akiya, Miho Oshima, Hirotake Inomata, Noboru Kitamura, Junki Kurita, Hiroyuki Hao, Hideki Nakamura
{"title":"Anti-centromere antibody-positive primary Sjögren's disease with exudative pleural effusion: A case report and literature review.","authors":"Masashi Uchikawa, Masako Tsukamoto, Kiichi Sugito, Shinya Asatani, Masahiro Nishihara, Kumiko Akiya, Miho Oshima, Hirotake Inomata, Noboru Kitamura, Junki Kurita, Hiroyuki Hao, Hideki Nakamura","doi":"10.1093/mrcr/rxag078","DOIUrl":"10.1093/mrcr/rxag078","url":null,"abstract":"<p><p>Sjögren's disease (SjD) is a systemic autoimmune disorder with glandular and extra-glandular manifestations. Pleural effusion is a rare complication of SjD among different types of pulmonary involvement. Herein, we report a case of primary anti-centromere antibody (ACA)-positive primary SjD with pleural effusion. A 79-year-old woman was admitted to our hospital with a 3-month history of leg oedema. She was diagnosed with primary SjD based on positive Schirmer and Saxon tests, and her anti-Sjögren syndrome-related antigen/Ro antibody test was negative, but ACA and anti-Sjögren syndrome-related antigen B/La tests were positive. The patient's symptoms promptly improved following prednisolone treatment. This is the first reported case of ACA-positive primary SjD complicated with pleural effusion, and the possible presence of pleural effusion should be considered in SjD, even in patients with ACA-positive primary SjD.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Critical ischemia of multiple organ systems in a patient with systemic lupus erythematosus complicated by catastrophic antiphospholipid syndrome: Potential pathogenetic role of non-inhibitory anti-ADAMTS13 autoantibodies. 系统性红斑狼疮合并灾难性抗磷脂综合征患者多器官系统严重缺血:非抑制性抗adamts13自身抗体的潜在致病作用
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag053
Shintaro Yamamoto, Yuichiro Shirai, Takahisa Gono, Mitsuhiro Takeno, Masanori Matsumoto, Masataka Kuwana
{"title":"Critical ischemia of multiple organ systems in a patient with systemic lupus erythematosus complicated by catastrophic antiphospholipid syndrome: Potential pathogenetic role of non-inhibitory anti-ADAMTS13 autoantibodies.","authors":"Shintaro Yamamoto, Yuichiro Shirai, Takahisa Gono, Mitsuhiro Takeno, Masanori Matsumoto, Masataka Kuwana","doi":"10.1093/mrcr/rxag053","DOIUrl":"10.1093/mrcr/rxag053","url":null,"abstract":"<p><p>We report a 33-year-old woman with systemic lupus erythematosus who developed fulminant multiorgan ischemic manifestations and hematologic abnormalities. She presented with persistent fever, newly developed painful fingertip cyanosis, and acute kidney injury. Laboratory tests showed severe thrombocytopenia, hemolytic anaemia with fragmented red blood cells, positive direct and indirect Coombs tests, hypocomplementemia, and positivity for multiple autoantibodies, including a triple-positive antiphospholipid antibody profile. Within the first week of hospitalisation, in addition to digital ischemia, she developed multiple cerebral infarctions and acalculous cholecystitis, findings consistent with catastrophic antiphospholipid syndrome. Unexpectedly, the activity of a disintegrin and metalloproteinase with thrombospondin type 1 motifs member 13 (ADAMTS13) was severely reduced to 5%, whereas ADAMTS13 inhibitor was not detected by the Bethesda assay. There was no past medical or family history suggestive of congenital thrombotic thrombocytopenic purpura. After treatment with high-dose glucocorticoids and nine sessions of plasma exchange, abdominal pain resolved and digital ischemia improved, accompanied by improvement in hematologic and renal abnormalities and disappearance of fragmented red blood cells. Subsequently, ADAMTS13 activity normalised to 63% and remained stable after completion of plasma exchange. Later, non-inhibitory anti-ADAMTS13 autoantibodies were detected by enzyme-linked immunosorbent assay in a stored serum sample obtained on admission. ADAMTS13 deficiency due to non-inhibitory anti-ADAMTS13 autoantibodies may modify the complications of systemic lupus erythematosus and antiphospholipid syndrome by promoting thrombotic microangiopathy.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148311109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sulfasalazine-induced aseptic meningitis. 磺胺嘧啶致无菌性脑膜炎。
IF 0.9
Modern rheumatology case reports Pub Date : 2026-06-29 DOI: 10.1093/mrcr/rxag071
Emily McHale, Andrew Dermawan, Raymond Tang, Lisa Redgrave
{"title":"Sulfasalazine-induced aseptic meningitis.","authors":"Emily McHale, Andrew Dermawan, Raymond Tang, Lisa Redgrave","doi":"10.1093/mrcr/rxag071","DOIUrl":"10.1093/mrcr/rxag071","url":null,"abstract":"<p><p>Aseptic meningitis is a clinical syndrome of meningeal inflammation without clear infectious aetiology. Drug-induced aseptic meningitis is a well-documented but poorly understood side effect of a variety of medications, and has rarely been described with the immunosuppressive and anti-inflammatory drug sulfasalazine. This report describes the case of a man in his 60s presenting with aseptic meningitis after commencing sulfasalazine for seronegative arthritis 1 month prior. Analysis of cerebrospinal fluid showed a predominantly mononuclear leucocytosis, slightly elevated protein, and normal glucose. Cerebrospinal fluid microscopy, culture, sensitivities, and polymerase chain reaction testing were negative for bacterial and viral causes of meningitis. The patient improved with cessation of sulfasalazine. Antimicrobial treatment was ceased with ongoing resolution of symptoms, supporting a diagnosis of sulfasalazine-induced aseptic meningitis. Clinicians should consider a diagnosis of sulfasalazine-induced aseptic meningitis when other aetiologies of meningitis have been excluded, particularly in the first weeks to months of commencing sulfasalazine.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148427735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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