Modern rheumatology case reports最新文献

{"title":"Successful salvage treatment with baricitinib for macrophage activation syndrome complicating adult-onset Still's disease during interleukin-6 inhibition: A case report and literature review.","authors":"Kazuma Nishisaka, Yo Ueda, Iku Shirasugi, Hirotaka Yamada, Takaichi Okano, Keisuke Nishimura, Sho Sendo, Jun Saegusa","doi":"10.1093/mrcr/rxag072","DOIUrl":"10.1093/mrcr/rxag072","url":null,"abstract":"<p><p>Adult-onset Still's disease (AOSD) is a systemic autoinflammatory disorder, and macrophage activation syndrome is a life-threatening hyperinflammatory complication. Interleukin-1 and interleukin-6 inhibitors have improved outcomes in refractory AOSD, but optimal management of macrophage activation syndrome, particularly when it develops during biologic therapy, remains uncertain. We report a case of a 49-year-old woman with articular-predominant AOSD who developed fulminant macrophage activation syndrome while receiving high-dose glucocorticoids, tacrolimus, and the interleukin-6 receptor inhibitor tocilizumab. At the onset of macrophage activation syndrome, she had persistent fever, cytopenia, hyperferritinemia, and liver dysfunction, and bone marrow examination revealed haemophagocytosis. Macrophage activation syndrome persisted despite two courses of intravenous methylprednisolone pulse therapy and continuation of tocilizumab. Tocilizumab was discontinued, and treatment was switched to the oral Janus kinase 1/2 inhibitor baricitinib in combination with glucocorticoids and tacrolimus. After this change, the patient experienced rapid defervescence, marked improvement in blood counts and ferritin levels, and sustained control of articular and systemic disease activity. Glucocorticoids were successfully tapered without relapse, and the patient remained in remission for more than 2 years without serious infections. To contextualise this case, we reviewed published reports on Janus kinase inhibition in AOSD, including cases complicated by macrophage activation syndrome. Multi-cytokine blockade through Janus kinase 1/2 inhibition, targeting overlapping interleukin-6, interferon-gamma, and granulocyte-macrophage colony-stimulating factor pathways, may simultaneously suppress macrophage activation and systemic inflammation. This case highlights the potential of baricitinib as a therapeutic option for refractory AOSD-associated macrophage activation syndrome during interleukin-6 inhibition.</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":"148427617","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}

{"title":"Reconstruction preserving the talonavicular and subtalar joints for flexible rocker-bottom foot deformity in rheumatoid arthritis.","authors":"Takuya Kurihara, Takaaki Noguchi, Yuki Etani, Atsushi Sugimoto, Seiji Okada, Kosuke Ebina","doi":"10.1093/mrcr/rxag066","DOIUrl":"10.1093/mrcr/rxag066","url":null,"abstract":"<p><p>A patient with rheumatoid arthritis and a flexible rocker-bottom foot deformity underwent surgery, including a flexor digitorum longus transfer, a spring ligament reconstruction, and a lateral column lengthening using calcaneocuboid distraction arthrodesis, while preserving the talonavicular and subtalar joints. Correction was achieved with the restoration of the medial longitudinal arch, and clinical outcome scores had improved at the final follow-up (1 year postoperatively). This case suggests that, even in patients with inflammatory joint diseases presenting with a rocker-bottom foot deformity, reconstruction preserving the talonavicular and subtalar joints may be considered as a treatment option when disease activity is adequately controlled with pharmacotherapy and the talonavicular and subtalar joints are not destroyed, as in the present case.</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":"148407554","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}

{"title":"Salvage surgery for talar component subsidence after total ankle arthroplasty using the FINE Total Ankle System: A case report.","authors":"Takaaki Noguchi, Yuki Etani, Gensuke Okamura, Makoto Hirao, Jun Hashimoto, Atsushi Sugimoto, Seiji Okada, Kosuke Ebina","doi":"10.1093/mrcr/rxag058","DOIUrl":"10.1093/mrcr/rxag058","url":null,"abstract":"<p><p>In recent years, total ankle arthroplasty (TAA) has been increasingly selected for the treatment of ankle arthropathy associated with rheumatoid arthritis because it allows early weight-bearing and preservation of ankle range of motion. However, salvage strategies for talar component subsidence after TAA depend on the implant system used, and ankle arthrodesis is frequently selected for cases treated with the FINE Total Ankle System (NAKASHIMA HEALTHFORCE CO.). Implant removal followed by ankle arthrodesis inevitably results in loss of ankle motion and substantial bone defects. Furthermore, removal of a cemented implant often requires extensive bone debridement, which may further increase the risk of non-union and deterioration of activities of daily living due to prolonged non-weight-bearing. We report a case of talar component subsidence of approximately 10 mm after TAA treated with the FINE Total Ankle System, successfully managed by revision TAA using the largest available talar component (size 6L) combination with bone grafting and medial malleolar osteotomy. This approach preserved ankle range of motion and was associated with favourable mid-term clinical and radiographic outcomes in the present case. Revision TAA using the largest available talar component with bone grafting may be considered a potential salvage option for carefully selected patients with talar component subsidence after TAA using the FINE Total Ankle System.</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":"148427635","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}

{"title":"Skin vasculitis and cardiac eosinophil extracellular trap formation in eosinophilic granulomatosis with polyangiitis: A case report.","authors":"Hisashi Kamido, Yuki Oba, Masayuki Yamanouchi, Tatsuya Suwabe, Kei Kono, Kenichi Ohashi, Keinosuke Hizuka, Shigeharu Ueki, Naoki Sawa, Yoshifumi Ubara","doi":"10.1093/mrcr/rxag046","DOIUrl":"10.1093/mrcr/rxag046","url":null,"abstract":"<p><p>The pathophysiology of organ involvement in eosinophilic granulomatosis with polyangiitis is characterised by vasculitis and eosinophilic inflammation, which can be accompanied by eosinophil extracellular trap cell death (EETosis). Clinically, eosinophilic inflammation is often observed in the lungs, gastrointestinal tract, and heart, whereas vasculitis more frequently affects the peripheral nerves, skin, and kidneys. However, histopathological confirmation of vasculitis and EETosis in different organs within the same patient has rarely been reported. We report a patient with EGPA presenting with both cutaneous and cardiac involvement. Skin biopsy findings were consistent with cutaneous fibrinoid vasculitis with prominent eosinophilic infiltration. Prednisolone therapy led to improvement of the skin lesion and inflammatory markers within 1 month. In contrast, endomyocardial biopsy demonstrated marked eosinophilic infiltration with cytolytic degranulation and extracellular deposition of eosinophil granule proteins, without evidence of fibrinoid vasculitis. Immunofluorescence findings supported the presence of EETosis. Despite prompt methylprednisolone pulse therapy, the cardiac lesion showed limited response and was complicated by refractory arrhythmias. This case suggests that distinct pathogenic mechanisms, namely vasculitis and EETosis, may predominate in different organs even within the same patient, potentially leading to discrepant treatment responses. When EETosis-driven pathology is dominant, conventional immunosuppressive therapy may have limited efficacy, highlighting the need for therapeutic strategies targeting EETosis.</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":"148311074","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}

{"title":"Idiopathic multicentric Castleman disease complicated by unilateral pleural thickening and massive pleural effusion: A case report.","authors":"Yuki Aitani, Shion Kachi, Ryosuke Hiwa, Yoshihiro Nishino, Ryota Sumitomo, Reiko Hidaka, Hironori Haga, Masataka Kitajima, Nobuhisa Okada, Mirei Shirakashi, Hideaki Tsuji, Shuji Akizuki, Ran Nakashima, Hajime Yoshifuji, Akira Onishi, Masao Tanaka, Akio Morinobu","doi":"10.1093/mrcr/rxag056","DOIUrl":"10.1093/mrcr/rxag056","url":null,"abstract":"<p><p>Idiopathic multicentric Castleman disease (iMCD) is a benign lymphoproliferative disease characterised by generalised lymphadenopathy and systemic inflammatory symptoms, occurring in individuals without an infection with human immunodeficiency virus or Kaposi sarcoma-associated herpesvirus. iMCD is typically subclassified into iMCD-TAFRO, which is characterised by thrombocytopenia, ascites, fever, reticulin fibrosis, and organomegaly; iMCD with idiopathic plasmacytic lymphadenopathy, which follows a chronic disease course with persistent lymphadenopathy, marked polyclonal hypergammaglobulinemia, and prominent plasma cell infiltration in the lymph nodes; and iMCD-not otherwise specified, which lacks features of both TAFRO syndrome and the idiopathic plasmacytic lymphadenopathy phenotype. Pleural thickening and effusion are extremely rare manifestations of iMCD-not otherwise specified. Herein, we present a rare case of iMCD-not otherwise specified presenting with unilateral pleural thickening and pleural effusion. A 76-year-old Japanese man was referred for further evaluation of a massive left-sided pleural effusion with tracheal compression. Fluorodeoxyglucose positron emission tomography/computed tomography showed increased uptake in the thickened pleura and multiple lymph nodes. Histopathological examination of a mediastinal lymph node demonstrated medullary and lymphoid follicular hyperplasia without structural destruction, while a biopsy of the thickened pleura showed infiltration of lymphocytes and plasma cells without dysplasia. The patient was treated with corticosteroids and tocilizumab, resulting in a marked improvement in his symptoms and pleural effusion. This case highlights the importance of considering pleural and lymph node biopsies for an accurate diagnosis and of not excluding iMCD in patients with unilateral pleural thickening accompanied by multiple lymphadenopathies.</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":"148378866","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}

{"title":"Gastrointestinal perforation due to lupus enteritis in early-stage systemic lupus erythematosus.","authors":"Shu Sugimoto, Jun-Ichi Kurashina, Naoki Tanomogi, Tatsuya Kobayashi, Takanori Ichikawa, Dai Kishida, Yasuhiro Shimojima, Yoshiki Sekijima","doi":"10.1093/mrcr/rxag074","DOIUrl":"10.1093/mrcr/rxag074","url":null,"abstract":"<p><p>Lupus enteritis (LE) is an uncommon gastrointestinal (GI) manifestation of systemic lupus erythematosus (SLE) and GI perforation is a rare, but a severe complication of LE that is typically found in patients with high disease activity and a long disease duration. Herein, we present the case of a previously healthy 31-year-old Thai man who developed SLE complicated by LE. He was admitted to our hospital with refractory thrombocytopenia despite initial treatment with high-dose prednisolone, mycophenolate mofetil, and plasma exchange. Although the abdominal pain initially improved, residual pain persisted in the left side of the abdomen at the time of transfer. The abdominal pain gradually worsened despite starting rituximab therapy. Colonoscopy revealed extensive necrosis of the descending colon, and contrast-enhanced abdominal computed tomography revealed colonic perforation. Emergency surgery revealed ischaemia extending from the distal transverse, to an entirely perforated descending colon. Histopathological findings suggested that the colonic perforation was attributable to SLE-associated mesenteric vasculitis. The LE did not recur postoperatively. Because LE might lead to GI perforation even during the early stages of SLE, patients with high disease activity and persistent abdominal pain should be closely monitored, and treatment should be promptly escalated given the potential risk of perforation.</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":"148427354","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}

{"title":"Incidental detection of spondyloarthritis on FDG-PET/CT performed for unintentional weight loss.","authors":"Haruki Matsumoto, Hiroki Nibu, Shuhei Yoshida, Tomoyuki Asano, Shuzo Sato, Yasuhiro Shimojima","doi":"10.1093/mrcr/rxag073","DOIUrl":"10.1093/mrcr/rxag073","url":null,"abstract":"<p><p>18F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography (PET/CT) is frequently used to evaluate malignancy. However, FDG uptake is not cancer-specific and may also be seen in inflammatory rheumatic diseases. Recognition of musculoskeletal uptake patterns can prevent misdiagnosis and prompt targeted evaluation. A 60-year-old woman developed unintentional weight loss. Malignant lymphoma was suspected based on elevated serum C-reactive protein, axillary lymphadenopathy, and elevated soluble interleukin-2 receptor. FDG-PET/CT demonstrated prominent uptake in the sternoclavicular joints, both shoulders, wrists, and the sacroiliac joints. She presented with long-standing inflammatory back pain, and examination showed reduced lumbar mobility. Pelvic radiography revealed grade 3 sacroiliitis in the right sacroiliac joint, and cervical radiography showed bulky asymmetric paravertebral ossification changes. Magnetic resonance imaging demonstrated short tau inversion recovery hyperintensity in the sacroiliac joints. Because FDG uptake was also observed in the sternoclavicular joints, synovitis-acne-pustulosis-hyperostosis-osteitis syndrome was considered; however, no characteristic skin lesions were present. She was diagnosed with spondyloarthritis. Ixekizumab was initiated, resulting in rapid clinical improvement. This case illustrates that FDG-PET/CT performed for suspected malignancy can incidentally reveal a characteristic musculoskeletal uptake pattern of spondyloarthritis. That prompt recognition of this pattern enables timely initiation of effective interleukin-17A inhibition, leading to rapid improvement in systemic inflammation and clinical symptoms.</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":"148427398","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}

{"title":"A case of rheumatoid neutrophilic dermatosis complicated by neutrophilic tubulointerstitial nephritis.","authors":"Hirotsugu Nohara, Yurie Satoh-Kanda, Satoshi Kubo, Natsuko Sasaki, Yu Sawada, Shumpei Kosaka, Aya Nawata, Shingo Nakayamada","doi":"10.1093/mrcr/rxag075","DOIUrl":"10.1093/mrcr/rxag075","url":null,"abstract":"<p><p>As an extra-articular manifestation of rheumatoid arthritis (RA), neutrophilic dermatosis may occur. Here, we report a rare case of RA complicated by both neutrophilic dermatosis and neutrophilic tubulointerstitial nephritis. In addition to polyarthritis, the patient exhibited purpura and erythema with haemorrhagic crusts and ulceration accompanied by marked neutrophilic infiltration, extending from the fingers to the forearms and from both lower legs to the toes, as well as interstitial lung disease, leading to a diagnosis of RA. After the initiation of tocilizumab, the arthritis improved. However, the skin lesions and renal function worsened. Histopathological examination of renal and skin biopsy specimens revealed prominent neutrophilic infiltration in both tissues. After ruling out infectious disease and vasculitis, the patient was diagnosed with neutrophilic dermatosis and neutrophilic acute tubulointerstitial nephritis. Following the introduction of dapsone, both the skin manifestations and renal impairment improved. This case represents a rare instance of rheumatoid neutrophilic dermatosis complicated by tubulointerstitial nephritis, highlighting the importance of targeting neutrophils in the treatment strategy based on the underlying pathophysiology.</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":"148564153","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}

{"title":"Immunoglobulin A vasculitis in association with invasive meningococcal disease.","authors":"Joseph Danicic, Burcu Isler, Jennifer Ng","doi":"10.1093/mrcr/rxag054","DOIUrl":"10.1093/mrcr/rxag054","url":null,"abstract":"<p><p>Immunoglobulin A vasculitis is uncommon in adults and is frequently associated with an identifiable trigger, most often infection. Invasive meningococcal disease is a rare but life-threatening infection caused by Neisseria meningitidis. We report the case of an 18-year-old Indigenous Australian woman who presented with fever, purpuric rash, ankle synovitis, abdominal pain, and systemic inflammation. A skin biopsy demonstrated leukocytoclastic vasculitis with immunoglobulin A deposition on immunohistochemistry, and blood cultures subsequently identified N. meningitidis serogroup B. Cerebrospinal fluid studies were unremarkable. The patient was treated with intravenous ceftriaxone, with complete clinical resolution and no recurrence at a 6-month follow-up. This case highlights a previously unreported association between immunoglobulin A vasculitis and invasive meningococcal disease in an adult patient, and it expands the spectrum of infectious triggers associated with adult-onset immunoglobulin A vasculitis.</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":"148341713","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}

{"title":"Bilateral diaphragmatic paralysis as clinical onset of eosinophilic granulomatosis with polyangiitis: A case report.","authors":"Federico Pasqualotto, Antonio Fabozzi, Maria Elisabetta Cardini, Arianna Sanna, Fausta Viccaro, Letizia D'Antoni, Paolo Palange","doi":"10.1093/mrcr/rxag043","DOIUrl":"10.1093/mrcr/rxag043","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis is a rare small-vessel vasculitis that may affect different organs, leading to a highly variable and often challenging initial clinical presentation. We report the case of a 67-year-old man presenting with acute non-exertional dyspnoea and chest tightness. He had a history of adult-onset asthma, chronic rhinosinusitis and peripheral eosinophilia. Imaging revealed bilateral diaphragmatic elevation, and needle electromyography confirmed diaphragmatic paralysis. His medical history, combined with the presence of an eosinophilic exudative pleural effusion, transient pulmonary ground-glass opacities, and turbinate eosinophilic extravascular inflammation led us to a diagnosis of eosinophilic granulomatosis with polyangiitis characterised by bilateral diaphragmatic paralysis. Eosinophilic granulomatosis with polyangiitis should be considered in patients with unexplained diaphragmatic paralysis associated with an appropriate clinical and laboratory picture, as prompt recognition is crucial for the prognosis.</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":"148220858","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}