Modern rheumatology case reports最新文献

{"title":"Benralizumab for eosinophilic granulomatosis with polyangiitis and subclinical cardiac involvement: A fertility-preserving case report.","authors":"Takashi Yamane, Ayaka Inoue, Mari Nishio","doi":"10.1093/mrcr/rxag086","DOIUrl":"10.1093/mrcr/rxag086","url":null,"abstract":"<p><p>Cardiac involvement, which may remain clinically silent, is a major determinant of prognosis in eosinophilic granulomatosis with polyangiitis, and remission induction generally requires glucocorticoids (GCs) combined with cyclophosphamide or rituximab. In contrast, treatment selection may be challenging in women wishing to preserve fertility because cyclophosphamide carries a risk of gonadal toxicity. We report the case of a 40-year-old woman with a history of asthma, eosinophilic chronic rhinosinusitis, and eosinophilic otitis media who developed postpartum-onset eosinophilic granulomatosis with polyangiitis and presented with neurological and cutaneous manifestations. Elevated cardiac troponin I levels and newly developed electrocardiographic abnormalities suggested subclinical myocardial injury despite the absence of cardiac symptoms. Intravenous methylprednisolone pulse therapy was initiated immediately. Because cardiac troponin I levels showed no early decline after GC initiation, and GC monotherapy was considered potentially insufficient to control eosinophil-mediated myocardial injury, additional eosinophil-targeted therapy was selected after shared decision-making that balanced fertility preservation with the need for prompt disease control. Benralizumab was chosen because of its potent eosinophil-depleting effect. Peripheral eosinophils rapidly decreased to zero, troponin I normalised by Day 26, and GCs were successfully discontinued within 5 months. To our knowledge, this is the first report of benralizumab used as part of a fertility-preserving remission-induction strategy to avoid cyclophosphamide in a woman with eosinophilic granulomatosis with polyangiitis and cardiac involvement. This approach may represent an individualised therapeutic option for carefully selected patients in whom fertility preservation is an important treatment consideration.</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":"148868514","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":"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":"Primary erythromelalgia associated with an SCN9A variant can mimic systemic lupus erythematosus in skin manifestations: A case report.","authors":"Atsuko Tomikawa, Kazuyuki Meguro, Keishi Etori, Tomoki Suichi, Makoto Yamamoto, Rena Oguma, Yoshihide Okano, Eri Sato, Kazutaka Noda, Keisuke Matsusaka, Jun-Ichiro Ikeda, Hiroshi Nakajima","doi":"10.1093/mrcr/rxag089","DOIUrl":"10.1093/mrcr/rxag089","url":null,"abstract":"<p><p>Primary erythromelalgia (PEM) is a rare autosomal dominant disorder characterised by episodic erythema and burning pain of the extremities, typically triggered by exercise or fatigue. PEM usually begins in childhood and is caused by gain-of-function variants in SCN9A, which encodes the voltage-gated sodium channel Nav1.7. The disease concept of PEM, however, is largely unrecognised among rheumatologists. Here, we report a 20-year-old woman who had experienced recurrent burning pain and redness of the extremities since around age 10. She also exhibited livedo-like rash, leukopenia, and hypocomplementemia and tested positive for antinuclear antibodies. Nerve conduction studies showed abnormalities interpreted as compatible with multifocal mononeuropathy. Based on these findings, she was diagnosed with systemic lupus erythematosus and treated with 20 mg of prednisolone; however, the painful erythema of the extremities did not improve. Given a family history of similar episodic symptoms in her father, PEM was suspected. Genetic testing identified a heterozygous SCN9A p.G856D variant, supporting the diagnosis of autosomal dominant PEM. Direct immunofluorescence/lupus band testing of the biopsied lower-leg lesion was interpreted as negative. This case highlights that SCN9A-associated PEM may present with lupus-like cutaneous erythema in young patients, potentially leading to misdiagnosis and ineffective immunosuppression. PEM should be considered in patients with childhood-onset, trigger-induced painful erythema of the extremities, particularly when there is family history or poor response to steroids.</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":"149121217","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}

{"title":"Baricitinib in two paediatric patients with coatomer subunit alpha (COPA) syndrome: A case series and literature review.","authors":"Enrique G Villarreal, Dilan Dissanayake, Ronald M Laxer, Kenneth W Yip, Fiona Kritzinger","doi":"10.1093/mrcr/rxag052","DOIUrl":"10.1093/mrcr/rxag052","url":null,"abstract":"<p><p>Coatomer subunit α (COPA) syndrome is a rare, autosomal dominant inborn error of immunity driven by dysregulated type I interferon signalling. It typically presents in early childhood with interstitial lung disease, arthritis, and systemic inflammation. Current treatments are largely extrapolated from adult protocols, and disease management remains challenging. Recent evidence suggests that Janus kinase inhibitors (JAKi), which block interferon signalling, may offer a targeted therapeutic approach. We report on two paediatric patients with genetically confirmed COPA syndrome who demonstrated sustained clinical improvement following the initiation of baricitinib, a JAKi. Both cases exhibited early onset interstitial lung disease and systemic inflammation. Genetic testing identified pathogenic variants in the COPA gene. Conventional immunosuppressants provided only partial and temporary relief with disease flares occurring when immunosuppression was tapered. Baricitinib was initiated, and both patients showed a rapid clinical response, improved pulmonary function, reduced systemic inflammation, lung parenchymal radiographic stabilisation, and improvement of interstitial lung disease symptoms. Baricitinib was well tolerated overall and enabled the tapering and discontinuation of corticosteroids and other immunosuppressants. These cases align with the limited existing literature showing the efficacy of JAKi in COPA syndrome and further support the role of precision medicine targeting type I interferonopathies. While long-term safety data remain limited, early findings suggest that JAK inhibition represents a promising therapeutic strategy in COPA syndrome. Careful monitoring for viral reactivation and infection is essential. Larger studies and longer follow-up are needed to validate these results and to optimise the management of this complex paediatric autoimmune disease.</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":"148310988","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":"Metatarsal-shortening offset osteotomy as salvage for painful plantar callosities following resection arthroplasty in patients with rheumatoid arthritis: A report of three cases.","authors":"Atsushi Goshima, Takaaki Noguchi, Yuki Etani, Hideki Tsuboi, Takashi Kaito, Motoki Iwasaki, Atsushi Sugimoto, Yuji Fukuda, Seiji Okada, Kosuke Ebina","doi":"10.1093/mrcr/rxag047","DOIUrl":"10.1093/mrcr/rxag047","url":null,"abstract":"<p><p>Surgical treatment for forefoot deformities in patients with rheumatoid arthritis has recently shifted toward joint-preserving procedures for all toes. In contrast, resection arthroplasty was previously more commonly performed for the lesser toes. Advances in drug therapy have improved life expectancy, resulting in an increased number of long-term survivors who had previously underwent resection arthroplasty. With increased activity levels, the recurrence of plantar callosities in the forefoot has become more frequent, often leading to gait disturbances. In general, revision surgery for such cases often involves additional bone resection; however, this approach may result in further shortening of the metatarsals and increase the risk of further recurrence. Therefore, we applied a metatarsal-shortening offset osteotomy, which we typically perform as a primary procedure, as a salvage procedure to reduce the risk of recurrence. We report three cases treated using this strategy. In all cases, the second to fifth metatarsal heads had been resected, but the resection stumps were covered with pseudo-cartilaginous tissue. This allowed a metatarsal-shortening offset osteotomy to be performed as a procedure similar to that used in primary surgery, achieving the planned correction. Consequently, both radiographic and clinical improvements were observed. These findings suggest that a metatarsal-shortening offset osteotomy may be a possible salvage option after primary resection arthroplasty for forefoot deformities in patients with rheumatoid arthritis.</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":"148311084","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}