{"title":"A case of SARS-CoV-2 infection during the course of SLE difficult to differentiate NPSLE from multisystem inflammatory syndrome in adults (MIS-A).","authors":"Takeru Sonoda, Shigeru Iwata, Katsunori Tanaka, Ryo Matsumiya, Kayoko Tabata, Nobuo Kuramoto, Takao Fujii","doi":"10.1093/mrcr/rxaf061","DOIUrl":"https://doi.org/10.1093/mrcr/rxaf061","url":null,"abstract":"<p><p>The global COVID-19 pandemic has also brought attention to multisystem inflammatory syndrome in adults (MIS-A), a post-COVID-19 complication. Here, we present a case of MIS-A caused by COVID-19 after the patient had systemic lupus erythematosus (SLE). A woman in her 30s developed SLE. Two years later, she became aware of arthralgia and febrile. On admission two weeks later, she had three episodes of generalized clonic seizure and a SARS-CoV-2 PCR test was positive. Blood tests showed elevated C-reactive protein (CRP) level. Cerebrospinal fluid was negative for bacterial culture but showed elevated IL-6. MRI FLAIR showed high signal in the brain surface from the right frontal to the temporal lobes. Antimicrobial agents, methylprednisolone pulse therapy and anticonvulsants were started on admission day. The seizures resolved, but the fever persisted and the CRP elevated again. Other conditions that might explain the severely elevated CRP were negative but the CDC 2020 diagnostic criteria for MIS-A was met. After a second methylprednisolone pulse and 3-day intravenous immunoglobulin therapy,her fever resolved, the CRP level decreased, and the MRI abnormalities and cerebrospinal fluid findings improved. Differential diagnosis is important when central nervous system symptoms are present in patients with SLE. If a patient develops neuropsychiatric manifestation after SARS-CoV-2 infection, possibility of MIS-A should be considered even in SLE patients.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202660","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":"Adult-onset Still's disease following COVID-19: A case report and literature review.","authors":"Tsubasa Maeda, Goshi Komine, Ryosuke Noda, Mikiya Komatsu, Mariko Sakai, Yukiko Takeyama, Sachiko Soejima, Akihito Maruyama, Mitsuteru Akahoshi, Syuichi Koarada, Yoshifumi Tada","doi":"10.1093/mrcr/rxaf059","DOIUrl":"https://doi.org/10.1093/mrcr/rxaf059","url":null,"abstract":"<p><p>A 59-year-old man developed adult-onset Still's disease 11 days after contracting COVID-19. He presented with high fever, polyarthritis, erythema, sore throat, and high levels of C-reactive protein and ferritin; treatment with glucocorticoids and methotrexate led to disease remission. We reviewed the clinical characteristics of 12 cases (11 from the literature and the present case) of adult-onset Still's disease following COVID-19. Eight cases involved females, with a median age of 54 years (19-59 years), and the median time from COVID-19 to Still's disease onset was 12.5 days. Frequencies of high fever, arthralgia, typical skin lesion, sore throat, liver damage, and increased neutrophil count did not differ from cases of non-COVID-related adult-onset Still's disease. Serum ferritin levels were increased in all cases (median 6,354 ng/mL). Complications were infrequent, with macrophage activation syndrome reported in one case. Immunosuppressive drugs and biologic agents were used in five and three cases, respectively, and all cases had good outcomes. Our review suggests that adult-onset Still's disease develops early after COVID-19, presenting with clinical findings similar to non-COVID-19-related cases, and has few severe complications and a good prognosis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145093329","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":"Fatal intrahepatic haemorrhage in a patient with rheumatoid vasculitis: an autopsy case report.","authors":"Ayako Makiyama, Yoshiyuki Abe, Mariko Hara, Takashi Kyomoto, Ryo Wakana, Takumi Saito, Masahiro Kogami, Kurisu Tada, Ayako Ura, Yuki Fukumura, Ken Yamaji, Naoto Tamura","doi":"10.1093/mrcr/rxaf034","DOIUrl":"10.1093/mrcr/rxaf034","url":null,"abstract":"<p><p>Rheumatoid vasculitis (RV) is an extra-articular complication characterised by small-to-medium vessel vasculitis associated with rheumatoid arthritis, leading to various organ involvements. However, there are few reports of RV associated with aneurysms causing intra-abdominal haemorrhage. Although the incidence of RV has recently decreased, its prognosis remains poor. We herein report a case of RV in a patient with a 1.5-year history of treatment for late-onset rheumatoid arthritis. The patient died of intrahepatic haemorrhage caused by the rupture of a hepatic artery aneurysm. RV can be challenging to diagnose clinically and is sometimes only identified at autopsy. When inflammatory findings arise that do not correspond to the activity of arthritis, careful differential diagnosis is essential.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144562491","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":"The importance of dietary history: A case of scurvy mimicking vasculitis.","authors":"Tatsuo Mori, Makiko Kimura, Masanori Hanaoka, Mutsuto Tateishi","doi":"10.1093/mrcr/rxaf035","DOIUrl":"10.1093/mrcr/rxaf035","url":null,"abstract":"<p><p>Scurvy, a disease caused by vitamin C deficiency, is now uncommon in developed countries with ample food resources. We present the case of a 28-year-old man with no significant past medical history who presented with lower extremity petechiae, initially raising suspicion for vasculitis. Although his skin biopsy findings were consistent with vasculitis, based on the characteristic perifollicular distribution of the purpura, the presence of corkscrew hairs, and the finding of a subfascial haematoma of the gastrocnemius muscle, which raised suspicion for a bleeding tendency, led us to suspect scurvy. A detailed dietary history revealed that he had consumed an imbalanced diet with no intake of fresh fruits or vegetables for more than 6 months. Serum ascorbic acid concentration was measured to be < 0.2 μg/ml, confirming the diagnosis of scurvy. In conclusion, scurvy can occur even in healthy young individuals without prior medical history living in developed countries and can present to rheumatologists as a mimic of vasculitis. It should be considered in the differential diagnosis of vasculitis, and a detailed dietary history should be obtained when suspected.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144562492","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":"Other iatrogenic immunodeficiency-associated lymphoproliferative disorders after multidrug immunosuppressive therapy for anti-melanoma differentiation association gene 5 antibody-positive dermatomyositis: a case report.","authors":"Shungo Mochizuki, Toshiki Nakajima, Shota Ohsumi, Aiko Ogura, Nozomi Akatsu, Noriyoshi Takebe, Kentaro Odani, Toshiyuki Kitano, Yoshitaka Imura","doi":"10.1093/mrcr/rxaf043","DOIUrl":"10.1093/mrcr/rxaf043","url":null,"abstract":"<p><p>Although other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs) are rare, they are important adverse effects of immunosuppressive therapies. Even though anti-melanoma differentiation association gene 5 (MDA5) antibody-positive dermatomyositis requires multidrug immunosuppressive therapy for interstitial pneumonia control, OIIA-LPD has rarely been reported. Moreover, central nervous system (CNS) OIIA-LPD has never been documented. Here, we report a case of CNS OIIA-LPD that may have been caused by treatment for MDA5 antibody-positive dermatomyositis. A 53-year-old woman was diagnosed with MDA5 dermatomyositis and treated for rapidly progressive interstitial lung disease using multidrug immunosuppressive therapy with prednisolone (PSL), tacrolimus, and intravenous cyclophosphamide pulse therapy. Seven months after treatment initiation, vomiting led to the discovery of a cerebellar tumour. The cerebellar tumour was histologically Epstein-Barr virus (EBV)-encoded small RNA-positive diffuse large B-cell lymphoma, with EBV-DNA being positive in the blood. The patient was diagnosed with OIIA-LPDs due to EBV reactivation. Chemotherapy, including high-dose methotrexate (MTX) and rituximab, prevented tumour recurrence without exacerbating interstitial lung disease. This is the first reported case of CNS OIIA-LPD with multidrug immunosuppression in a patient with MDA5 dermatomyositis. Chemotherapy, including high-dose MTX and rituximab, can be used for central OIIA-LPD without aggravating settled interstitial lung disease. The activity of MDA5 dermatomyositis during OIIA-LPD treatment may be managed with low-dose PSL.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144661475","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":"Successful treatment with tocilizumab in a case of familial Mediterranean fever with Takayasu arteritis.","authors":"Saeka Kondo, Natsuka Umezawa, Yasuhiro Tagawa, Shinsuke Yasuda","doi":"10.1093/mrcr/rxaf027","DOIUrl":"10.1093/mrcr/rxaf027","url":null,"abstract":"<p><p>Familial Mediterranean fever (FMF) is an autoinflammatory disease associated with mutations in MEFV, which encodes pyrin. Patients with FMF present intermittent high fever with elevated inflammatory markers during periodic attacks. While some forms of vasculitis, including immunoglobulin A (IgA) vasculitis and polyarteritis nodosa have been reported in some patients with FMF, Takayasu arteritis (TAK) rarely associated with FMF. In addition, little has been known about the clinical features and pathogenesis of vasculitis with FMF. Here we report a case of FMF with TAK. Our case is remarkable on his clinical course of neck pain with low-grade elevation of serum C-reactive protein during interictal periods of fever attacks. He possessed the dual genetic background of a pathogenic variant of p.M694V in MEFV and HLA-B*52:01, which is susceptible to TAK. Although he was refractory to the combination therapy with colchicine, corticosteroids, and methotrexate, tocilizumab was effective for both recurrent fever attacks and vasculitis. Previous four reports of FMF with TAK as well as our case suggest the pathogenic MEFV mutation could be a predisposing or additional factor that modify the development of TAK. Since both the activity of FMF and TAK responded to tocilizumab in our case, the pathogenesis shared between FMF and TAK was indicated.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144164420","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":"Life-threatening anaphylaxis after first dose of Iguratimod.","authors":"Bodhisatwa Choudhuri","doi":"10.1093/mrcr/rxaf053","DOIUrl":"10.1093/mrcr/rxaf053","url":null,"abstract":"<p><p>Iguratimod is a novel oral disease-modifying antirheumatic drug (DMARD) utilised for rheumatoid arthritis, characterised by a favourable safety profile and infrequent instances of hypersensitivity, predominantly mild and cutaneous in nature. This report describes what appears to be the first reported case of severe, noncutaneous anaphylaxis following a first oral dose of iguratimod. A 37-year-old woman with seropositive rheumatoid arthritis, previously stable on methotrexate, experienced acute respiratory distress, hypotension, and new-onset atrial fibrillation within 3 hours of her initial iguratimod dose. She had never experienced a medication allergy before. Examination indicated significant hypoxia and cardiovascular instability. Anaphylaxis was validated by increased serum tryptase levels. Immediate treatment included injectable epinephrine, corticosteroids, fluid resuscitation, and mechanical ventilation. Electrical cardioversion was necessary to treat atrial fibrillation. The patient was stabilised with intensive care and was discharged without complications. This case demonstrates a rare but dramatic adverse reaction to iguratimod, emphasising the necessity of including anaphylaxis in the differential diagnosis of acute cardiorespiratory collapse, even in the absence of skin signs. Clinicians must recognise that novel immunomodulatory drugs may provoke severe allergic reactions and ensure that suitable precautions and emergency protocols are established prior to commencing such therapies.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857294","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":"Unexpected adverse event caused by avacopan: a case of drug-induced hypersensitivity syndrome in microscopic polyangiitis.","authors":"Hiroki Nibu, Haruki Matsumoto, Yuto Ishizaki, Eisuke Kameoka, Hiroki Irie, Naoki Konno, Yukiko Kanno, Kenta Kodama, Masahito Kuroda, Yoshikazu Motoki, Kazuhiro Tasaki, Tomoyuki Asano, Shuzo Sato, Masayuki Miyata","doi":"10.1093/mrcr/rxaf054","DOIUrl":"10.1093/mrcr/rxaf054","url":null,"abstract":"<p><p>Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody-associated vasculitis characterised by inflammation in small vessels. Avacopan, an oral C5a receptor inhibitor, has demonstrated efficacy in inducing and sustaining remission in MPA, with the added benefit of reducing glucocorticoid exposure and associated toxicities. Among adverse effects, liver injury is the most common, occurring in 16.7-40.9% of cases in the Japanese cohorts. Drug-induced hypersensitivity syndrome (DIHS) is a rare adverse effect caused by avacopan. We describe a case of a 77-year-old woman with MPA who was initiated on prednisolone 30 mg/day and avacopan as the induction therapy. Disease activity of MPA improved with this induction therapy. However, 7 weeks after initiating avacopan, she developed significant liver dysfunction. Despite the discontinuation of avacopan, she subsequently presented with fever and a generalised rash, leading to a diagnosis of DIHS. Laboratory data revealed reactivation of human herpesvirus 6. Despite the discontinuation of avacopan, liver injury persisted, and liver biopsy findings were consistent with drug-induced hepatitis. Long-term hospitalisation was required for improvement in skin symptoms and liver function. This case highlights a rare but serious adverse event of avacopan in MPA. During avacopan therapy, it is necessary to monitor for delayed severe skin symptoms such as DIHS.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144857295","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}
Rajaie Namas, Sarah Al Qassimi, Jawahir Alameri, Fatema Alawadhi, Esat Memisoglu, Ahlam Almarzooqi
{"title":"Young onset chronic inflammatory back pain: A diagnostic dilemma between axial spondylitis and diffuse idiopathic skeletal hyperostosis.","authors":"Rajaie Namas, Sarah Al Qassimi, Jawahir Alameri, Fatema Alawadhi, Esat Memisoglu, Ahlam Almarzooqi","doi":"10.1093/mrcr/rxaf002","DOIUrl":"10.1093/mrcr/rxaf002","url":null,"abstract":"<p><p>Diffuse idiopathic skeletal hyperostosis (DISH) and axial spondyloarthritis (axSpA) share similarities in both clinical presentation and radiological findings, making the diagnostic process challenging. We report the case of a 30-year-old male with a long-standing history of back pain with an initial diagnosis of young-onset DISH. However, a diagnosis of axSpA was ultimately pursued based on his age and clinical presentation. This was further supported by improvement in both Ankylosing Spondylitis Disease Activity Score with Erythrocyte Sedimentation Rate (ASDAS-ESR) and Ankylosing Spondylitis Disease Activity Score with C-Reactive Protein (ASDAS-CRP) scores at his 6- and 12-month follow-ups on ixekizumab. Early and accurate diagnosis of axSpA, followed by appropriate treatment, is essential in preventing complications and improving patient outcomes.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019147","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 high tibial osteotomy in a patient with psoriatic arthritis.","authors":"Mikiro Koga, Akira Maeyama, Tetsuro Ishimatsu, Tomonobu Hagio, Terufumi Shibata, Yutaro Yamasaki, Yuki Sugino, Takuaki Yamamoto","doi":"10.1093/mrcr/rxaf007","DOIUrl":"10.1093/mrcr/rxaf007","url":null,"abstract":"<p><p>Traditionally, patients with rheumatic diseases, such as rheumatoid arthritis (RA), were considered unsuitable for joint-sparing surgery. In the present study, we report on bilateral knee joints affected by psoriatic arthritis coexisting with osteoarthritis, with good, albeit short-term, results. A 62-year-old woman was treated for psoriatic arthritis with a biologic (adalimumab). The Disease Activity in Psoriatic Arthritis index was 7.24, indicating low disease activity. She had been suffering from bilateral knee pain for some time and was treated conservatively by her local doctor, but the pain persisted, and she came to visit us. At the initial visit, tenderness in the medial joint line of both knees and hydrarthrosis in the right knee were observed. Preoprative radiographs at the time of the initial examination showed medial-type osteoarthritis in both knees. First, interlocking closed wedge high tibial osteotomy (CWHTO) was performed on the right knee. This was followed 1 year later by right knee implant removal and interlocking CWHTO on the left knee, with implant removal on the left knee 1 year after that. In both knees preoperatively and postoperatively, the joint range of motion and the Knee Injury and Osteoarthritis Outcome Score total improved Considering the patient's background, we considered high tibial osteotomy if the disease activity was controlled. However, if the disease worsens in the future, joint destruction may occur, so careful follow-up is necessary.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143030659","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}