Modern Rheumatology Case Reports最新文献

{"title":"Giant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity in a patient with systemic sclerosis.","authors":"Yoshihiro Kitahara,&nbsp;Rie Nakamura,&nbsp;Makiko Kawai,&nbsp;Tetsu Hirakawa,&nbsp;Arisa Hamada,&nbsp;Mineyo Mito,&nbsp;Kikuo Nakano","doi":"10.1093/mrcr/rxad006","DOIUrl":"https://doi.org/10.1093/mrcr/rxad006","url":null,"abstract":"<p><p>To the best of our knowledge, systemic sclerosis with overlapping characteristics of both microscopic polyangiitis and giant cell arteritis (i.e. microscopic polyangiitis involving the superficial temporal artery or giant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity) has not been reported previously. An 82-year-old woman with diffuse cutaneous systemic sclerosis experienced dyspnoea on exertion and fever. No signs of infection were observed on computed tomography. Her fever persisted despite antibiotic treatment for occult bacterial infection and secondary Clostridioides difficile-associated diarrhoea. Microscopic polyangiitis was suspected because of myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity, and giant cell arteritis was suspected as a differential diagnosis due to swelling of the superficial temporal artery. Arterial biopsy revealed inflammatory cell infiltration with granuloma formation. Based on the presence of granulomatous inflammation in the superficial temporal artery, we concluded that giant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity occurred as a complication. After glucocorticoid therapy, her fever and dyspnoea on exertion improved with a gradual decline in the serum myeloperoxidase anti-neutrophil cytoplasmic antibody levels. It is possible that vasculitis occurs as a complication in patients with systemic sclerosis in cases where the fever persists and cannot be explained by systemic sclerosis itself, infectious disease, or malignancy. Clinicians must be careful not to prematurely diagnose microscopic polyangiitis based on myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity or giant cell arteritis based on the swelling of the superficial temporal artery. Careful evaluation of the presence of granulomatous inflammation in an arterial biopsy specimen is essential to differentiate between microscopic polyangiitis and giant cell arteritis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"458-463"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724088","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 Behçet's-like disease associated with trisomy 8-positive myelodysplastic syndrome carrying MEFV E148Q variant presented with periodic fever.","authors":"Yukiko Ishikawa,&nbsp;Ryo Sasaki,&nbsp;Akira Ishiwata,&nbsp;Shuji Hatakeyama,&nbsp;Masami Matsumura,&nbsp;Takeo Sato","doi":"10.1093/mrcr/rxad015","DOIUrl":"https://doi.org/10.1093/mrcr/rxad015","url":null,"abstract":"<p><p>Behçet's-like disease, which incompletely fulfils the criteria of Behçet's disease, is often associated with trisomy 8-positive myelodysplastic syndrome (MDS). We report a case of an 82-year-old man with these conditions carrying the E148Q variant of MEFV gene who presented with periodic fever. The patient presented with joint pain, muscle pain, and episodes of periodic fever every 2 weeks for the past 3 months. On admission, painful erythema and fever were observed. Colonoscopy revealed erosion in the caecum and ascending colon. The patient had bicytopenia, and a bone marrow biopsy showed findings compatible with trisomy 8-positive unclassifiable MDS. Because the patient incompletely fulfilled the criteria for Behçet's disease, he was diagnosed with Behçet's-like disease associated with trisomy 8-positive MDS. Positron emission tomography-computed tomography performed during the fever revealed multiple muscle lesions consistent with the sites of pain. To examine the cause of the periodic fever attacks, MEFV gene was analysed, and the results revealed an E148Q variant. Steroids were ineffective against periodic fever attacks. A daily dose of 0.5 mg colchicine was prescribed, but the effect was minimal, probably, because of the insufficient dose due to renal dysfunction. Based on the diagnosis of atypical familial Mediterranean fever, canakinumab was added, which partially mitigated the periodic fever. This case suggests the importance of ruling out MDS when physicians see an elderly patient who present with Behçet's-like disease. Although the significance of the E148Q variant in the pathogenesis of periodic fever remains controversial, it may act as a disease modifier in accordance with trisomy 8-positive MDS.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"470-474"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9670217","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":"Overlap syndrome involving anti-OJ antibody-positive polymyositis, systemic lupus erythematosus and Sjögren's syndrome: A case report and literature review.","authors":"Yumiko Mizuhashi,&nbsp;Shinya Hirata,&nbsp;Komei Sakata,&nbsp;Eiko Miyagawa,&nbsp;Mikako Iwakura,&nbsp;Masayuki Murai,&nbsp;Tsuneo Sasai,&nbsp;Masao Matsuoka","doi":"10.1093/mrcr/rxac087","DOIUrl":"https://doi.org/10.1093/mrcr/rxac087","url":null,"abstract":"<p><p>Anti-OJ antibody is relatively rarely detected in patients with the anti-synthetase syndrome, which is polymyositis (PM)/dermatomyositis (DM) with anti-aminoacyl transfer ribonucleic acid (RNA) synthetase antibodies. There have been few case reports of anti-OJ antibody-positive PM/DM complicated by other connective tissue disorders. Herein, we report the case of a 33-year-old woman who was admitted to our hospital with fever, muscle weakness, and dyspnoea on exertion. She was diagnosed with anti-OJ antibody-positive PM, overlapping systemic lupus erythematosus, and Sjögren's syndrome (SS). Her symptoms and clinical findings improved after treatment with prednisolone 1 mg/kg/day without immunosuppressive agents. This is the first case of overlap syndrome with anti-OJ antibody-positive PM, systemic lupus erythematosus, and Sjögren's syndrome.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"399-403"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9668290","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":"Distraction arthroplasty combined with autologous bone grafting for diffuse-type tenosynovial giant cell tumour with articular cartilage defect and subchondral bone cysts: A case report.","authors":"Yasunari Ikuta,&nbsp;Tomoyuki Nakasa,&nbsp;Junichi Sumii,&nbsp;Akinori Nekomoto,&nbsp;Shingo Kawabata,&nbsp;Nobuo Adachi","doi":"10.1093/mrcr/rxac092","DOIUrl":"https://doi.org/10.1093/mrcr/rxac092","url":null,"abstract":"<p><p>Tenosynovial giant cell tumour (TGCT) encompasses a group of lesions that present with synovial differentiation and most commonly occur in the joint synovium, bursae, and tendon sheaths. Diffuse-type TGCT (Dt-TGCT), previously known as pigmented villonodular synovitis, is one of the most common benign soft-tissue tumours of the foot and ankle and usually affects young adults. The differential diagnosis of Dt-TGCTs remains a clinical problem because their clinical symptoms are similar to those of inflammatory arthritis, including rheumatoid arthritis. Moreover, persistent Dt-TGCTs can lead to articular deterioration, including osseous erosions and subchondral bone cysts. Joint-preserving procedures are considered optimal for treating younger patients with ankle osteoarthritis because the indication of ankle arthrodesis and total ankle arthroplasty is limited. Thus, ankle distraction arthroplasty could be an alternative for treating Dt-TGCT with articular deterioration in young patients. Here, we report about a woman in her early 30s who presented with ankle pain owing to a Dt-TGCT with an articular cartilage defect and subchondral bone cysts. We performed ankle distraction arthroplasty combined with an autologous bone graft. A follow-up examination at 2 years revealed preservation of physical function and pain alleviation. These findings suggest that distraction arthroplasty is a viable treatment option for remedying the destruction of the articular cartilage and subchondral bone owing to Dt-TGCTs in young adults.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"483-487"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9670501","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 systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura.","authors":"Ryo Motoyama,&nbsp;Tomoaki Higuchi,&nbsp;Shinya Hirahara,&nbsp;Naoko Konda,&nbsp;Risa Yamada,&nbsp;Kotaro Watanabe,&nbsp;Mayuko Fujisaki,&nbsp;Rei Yamaguchi,&nbsp;Yasuhiro Katsumata,&nbsp;Yasushi Kawaguchi,&nbsp;Masayoshi Harigai","doi":"10.1093/mrcr/rxad011","DOIUrl":"https://doi.org/10.1093/mrcr/rxad011","url":null,"abstract":"<p><p>An 18-year-old Japanese woman with systemic lupus erythematosus experienced dyspnoea, headache, tinnitus, and purpura for 2 weeks and was admitted to our hospital. The patient had been diagnosed with systemic lupus erythematosus and secondary immune thrombocytopenia 8 years before and treated with high-dose prednisolone and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45 mg/day prednisolone; however, the patient's platelet count did not normalise. Based on a low level of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for 6 consecutive days, the patient's platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"383-387"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9674045","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 neuropathic arthropathy of the elbow with early loosening after total elbow arthroplasty.","authors":"Hiroshi Nakamura,&nbsp;Takuji Iwamoto,&nbsp;Hiroo Kimura,&nbsp;Taku Suzuki,&nbsp;Noboru Matsumura,&nbsp;Morio Matsumoto,&nbsp;Masaya Nakamura","doi":"10.1093/mrcr/rxac094","DOIUrl":"https://doi.org/10.1093/mrcr/rxac094","url":null,"abstract":"<p><p>We reported the case of a 65-year-old woman who presented with neuropathic arthropathy of the elbow and had undergone cervical spinal cord tumour resection at 32 years of age. Open synovectomy with free-body resection was performed; however, the instability of the elbow joint rapidly progressed. Therefore, we performed total elbow arthroplasty (TEA) using a linked-type prosthesis. However, the humeral stem became severely loosened 1 year after arthroplasty. Furthermore, a periprosthetic humeral fracture developed due to a minor trauma. A revision TEA using a long-stem prosthesis was performed 4 years after the primary TEA. Radiographs taken 2 years after the revision surgery showed no evidence of implant loosening. In this case, early postoperative loosening occurred despite the use of a linked-type prosthesis and an appropriate cementing technique, suggesting that normal implants may not provide sufficient fixation for neuropathic arthropathy of the elbow. Since surgery for neuropathic arthropathy generally has a poor prognosis, surgical interventions including TEA should be carefully considered.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"480-482"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724063","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":"Hereditary angio-oedema with normal C1-INH, developing recurrent acute abdomen after taking low-dose oestrogen-progestin: A case report.","authors":"Tsuyoshi Nakayama,&nbsp;Yasuhiro Tamimoto,&nbsp;Yutaka Shimomura,&nbsp;Hiroshi Tsukamoto","doi":"10.1093/mrcr/rxad017","DOIUrl":"https://doi.org/10.1093/mrcr/rxad017","url":null,"abstract":"<p><p>Hereditary angio-oedema (HAE) is a rare genetic disease characterised by repeated episodes of temporary organ swelling. Three types of HAE are known, of which HAE with normal C1 inactivator is difficult to be diagnosed due to its lack of laboratory abnormalities. Here, we describe a case of HAE with normal C1 inactivator and recurrent acute abdomen following low-dose oestrogen-progestin therapy. Notably, genetic analysis by Sanger sequencing led to the identification of a recurrent heterozygous missense mutation c.988A > G (p.K330E) in the plasminogen (PLG) gene of the patient. Prophylactic tranexamic acid and on-demand selective bradykinin B2 receptor blockers are used to treat her symptoms.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"491-494"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724596","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":"Aseptic meningitis as an initial manifestation of primary Sjögren's syndrome.","authors":"Misako Higashida-Konishi,&nbsp;Mitsuhiro Akiyama,&nbsp;Tatsuya Shimada,&nbsp;Satoshi Hama,&nbsp;Tatsuhiro Oshige,&nbsp;Keisuke Izumi,&nbsp;Hisaji Oshima,&nbsp;Yutaka Okano","doi":"10.1093/mrcr/rxac095","DOIUrl":"https://doi.org/10.1093/mrcr/rxac095","url":null,"abstract":"<p><p>Aseptic meningitis is a rare life-threatening complication of primary Sjögren's syndrome (pSS), and its characteristics and prognosis remain unknown. We present our case of aseptic meningitis associated with pSS and reviewed the published literature to elucidate their characteristics and prognosis. An 84-year-old man was admitted to our hospital for fever and disturbance of consciousness. Acute aseptic meningitis was diagnosed based on the results for cerebrospinal fluid and head imaging tests. As an aetiological investigation for his aseptic meningitis, serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies were found to be positive, and the biopsy specimen of his labial salivary gland revealed lymphocytic sialadenitis, confirming a diagnosis of pSS. Treatment with moderate-dose glucocorticoid completely improved his aseptic meningitis. Relapse of the disease was not observed during his clinical course over 12 months. Our present case and literature review suggest that aseptic meningitis can be an initial manifestation of pSS and be treatable by immunosuppressive therapy. Thus, early recognition and treatment initiation are critical to prevent the irreversible damage of central nervous system in pSS-associated aseptic meningitis. In aseptic meningitis of unknown origin, pSS should be included in differential diagnoses, and testing for serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen A antibodies may be useful as an initial screening.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"388-393"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9670504","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":"Reactive arthritis after Gam-COVID-Vac vaccination: A case report.","authors":"Kamyar Shokraee,&nbsp;Somaye Sadat Rezaei,&nbsp;Shahriar Shahriarian,&nbsp;Maryam Masoumi,&nbsp;Amirhossein Parsaei,&nbsp;Behnam Amini","doi":"10.1093/mrcr/rxad002","DOIUrl":"https://doi.org/10.1093/mrcr/rxad002","url":null,"abstract":"<p><p>Although the safety and efficacy of vaccinations have been evaluated through clinical trials, medical experts and authorities are very interested in the reporting and investigation of adverse events following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) immunisation in the general public. This article reports about a 41-year-old man without a history of underlying diseases, complaining of continuous morning stiffness and acute discomfort in his left elbow joint, 20 days after taking the first dosage of Sputnik V. The case was extensively studied, and a possible diagnosis of reactive arthritis was made.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"368-372"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724086","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":"Refractory PR3-positive ANCA-associated vasculitis with eosinophilia requiring anti-IL-5 therapy: A case report.","authors":"Elizabeth Anderson,&nbsp;Marcia A Friedman","doi":"10.1093/mrcr/rxac096","DOIUrl":"https://doi.org/10.1093/mrcr/rxac096","url":null,"abstract":"<p><p>This report describes a highly unusual and refractory case of proteinase-3-positive antineutrophil cytoplasmic autoantibody-associated vasculitis, which was associated with marked eosinophilia and only achieved remission after interleukin-5 blockade was added to maximal induction therapy. The patient was a healthy 18-year-old woman who presented with scleritis, otitis, constitutional symptoms, skin and mucosal lesions, and diffuse alveolar haemorrhage and found to have refractory eosinophilia of unclear aetiology. The case did not meet classification criteria for a diagnosis of granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis. Despite induction therapy with glucocorticoids, cyclophosphamide, and rituximab, she did not achieve remission or normalisation of eosinophilia until the addition of mepolizumab, which corresponded to the suppression of peripheral blood eosinophils and clinical remission. This case highlights the limitations of classification criteria and success of interleukin-5 in a multimodal treatment of severe vasculitis with eosinophilia.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"431-434"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9669753","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}