{"title":"Primary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.","authors":"Michail Papantoniou, Stefanos Korfias, Theodoros Argyrakos, Panagiotis Aggelidakis, Antonios Tavernarakis","doi":"10.1093/mrcr/rxad034","DOIUrl":"10.1093/mrcr/rxad034","url":null,"abstract":"<p><p>Primary angiitis of the central nervous system (PACNS) refers to a rare form of vasculitis of unknown cause, with a challenging diagnostic work-up. We report the case of a 57-year-old patient who presented with transient episodes of headache and global aphasia. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with moderate elevated protein and normal glucose. CSF and serum tests for infections and autoimmune/paraneoplastic antibodies were negative, except CSF polymerase chain reaction testing that detected Epstein-Barr virus (EBV). Magnetic resonance imaging of the brain with intravenous gadolinium showed meningeal enhancement and pachymeningitis. Due to continuous relapsing episodes of aphasia, a leptomeningeal and brain tissue biopsy was performed and revealed lesions of granulomatous necrotising vasculitis of medium-sized leptomeningeal and intracranial vessels, as well as negative in situ hybridism for EBV. A diagnosis of primary granulomatous necrotising angiitis of the central nervous system was made, and the patient was treated with intravenous methylprednisolone and oral cyclophosphamide, showing excellent response to treatment. Diversity in clinical and laboratory features makes it difficult for PACNS to be distinguished by other systemic vasculitides. Laboratory tests and neuroimaging can provide guidance in evaluation of the patients and exclude other possible causes, but tissue biopsy remains the gold standard for a definite diagnosis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"117-120"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9973806","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}
Yu Mori, Takuya Izumiyama, Kazuaki Otsuka, Ryuichi Kanabuchi, Jun Yamamoto, Mana Sekine, Naoko Mori, Yoshihide Asano, Toshimi Aizawa
{"title":"Pyoderma gangrenosum after surgery for forefoot deformity in a patient with rheumatoid arthritis: A case report.","authors":"Yu Mori, Takuya Izumiyama, Kazuaki Otsuka, Ryuichi Kanabuchi, Jun Yamamoto, Mana Sekine, Naoko Mori, Yoshihide Asano, Toshimi Aizawa","doi":"10.1093/mrcr/rxad051","DOIUrl":"10.1093/mrcr/rxad051","url":null,"abstract":"<p><p>Pyoderma gangrenosum (PG) is a rare inflammatory skin disease characterised by skin ulcers that are associated with autoimmune diseases. Although the effectiveness of immunosuppression with glucocorticoids and tumour necrosis factor inhibitors in treating PG has been reported, the utility of negative-pressure wound therapy (NPWT) for severe ulcerative lesions in patients with PG remains controversial. Herein, we report the case of a 76-year-old woman with rheumatoid arthritis who developed PG after undergoing surgery for a forefoot deformity. The patient showed improvement in deep ulcer lesions through NPWT while receiving treatment with abatacept and systemic glucocorticoids. Subsequent topical glucocorticoid therapy led to the remission of the PG. This case suggests that NPWT, when used under immunosuppressive conditions, does not exacerbate the pathergy and may be beneficial for treating severe ulcerative PG.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"11-15"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10139777","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":"Secondary fibromyalgia: An entity to be remembered-A case series with axial spondyloarthritis.","authors":"Ebru Yilmaz","doi":"10.1093/mrcr/rxad023","DOIUrl":"10.1093/mrcr/rxad023","url":null,"abstract":"<p><p>Fibromyalgia (FM) is a clinical syndrome characterised by chronic widespread musculoskeletal pain, stiffness, and tenderness in addition to a variety of physical and mental symptoms such as fatigue, sleep disturbances, depression, anxiety, cognitive dysfunction, headaches, and digestive problems. FM can be associated with or coexist with other inflammatory rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, osteoarthritis, and spondyloarthritis. This phenomenon is called secondary FM. Although FM cannot be considered an autoimmune disease, it may in some cases be an early sign of an autoimmune disease. Therefore, clinicians should be cautious in these situations. This case series presents three patients diagnosed with axial spondyloarthritis coexisted with FM symptoms.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"26-32"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9545336","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 belimumab for immune thrombocytopenia associated with systemic lupus erythematosus: A report of two cases.","authors":"Kyohei Nakayama, Yasuhiro Tamimoto, Tsuyoshi Nakayama","doi":"10.1093/mrcr/rxad055","DOIUrl":"10.1093/mrcr/rxad055","url":null,"abstract":"<p><p>Immune thrombocytopenia (ITP) is a common complication of systemic lupus erythematosus (SLE). Glucocorticoids (GCs) and hydroxychloroquine are first-line therapy for SLE-associated ITP (SLE-ITP). SLE-ITP in most of patients is less severe and well controlled with GCs, but some of the patients are GC resistant and require additional immunosuppressants including calcineurin inhibitors, azathioprine, and rituximab. We present two cases of SLE-ITP patients treated with belimumab who were resistant to GCs and achieved remission. For severe SLE-ITP, belimumab has emerged as a novel induction therapeutic option.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"69-73"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10285996","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 peficitinib monotherapy for the new-onset skin manifestations of rheumatoid vasculitis after long-term treatment with tocilizumab for rheumatoid arthritis.","authors":"Yuki Oba, Naoki Sawa, Daisuke Ikuma, Hiroki Mizuno, Noriko Inoue, Akinari Sekine, Eiko Hasegawa, Masayuki Yamanouchi, Tatsuya Suwabe, Yuko Yamaguchi, Yutaka Takasawa, Yoshifumi Ubara","doi":"10.1093/mrcr/rxad025","DOIUrl":"10.1093/mrcr/rxad025","url":null,"abstract":"<p><p>Rheumatoid vasculitis (RV) is a severe extra-articular systemic manifestation of rheumatoid arthritis (RA). Its prevalence has been decreasing for decades because of improved early diagnosis of RA and advances in RA treatment, but it remains a life-threatening disease. The standard treatment for RV has been a glucocorticoid and disease-modifying antirheumatic drugs. Biological agents, including antitumour necrosis factor inhibitors, are also recommended for refractory cases. However, there are no reports of Janus kinase (JAK) inhibitor use in RV. We experienced a case of an 85-year-old woman with a 57-year history of RA who had been treated with tocilizumab for 9 years after receiving three different biological agents over 2 years. Her RA seemed to be in remission in her joints, and her serum C-reactive protein had decreased to 0.0 mg/dL, but she developed multiple cutaneous leg ulcers associated with RV. Because of her advanced age, we changed her RA treatment from tocilizumab to the JAK inhibitor peficitinib in monotherapy, after which the ulcers improved within 6 months. This is the first report to indicate that peficitinib is a potential treatment option for RV that can be used in monotherapy without glucocorticoids or other immunosuppressants.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"5-10"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9493441","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 in which baricitinib was effective for both rheumatoid arthritis and essential thrombocythemia.","authors":"Rioko Migita, Atsushi Tanaka, Kazuki Tanimoto, Junki Hiura, Yasutaka Kimoto, Takahiko Horiuchi, Yasushi Inoue","doi":"10.1093/mrcr/rxad012","DOIUrl":"10.1093/mrcr/rxad012","url":null,"abstract":"<p><p>We experienced a case of rheumatoid arthritis and JAK2V617F mutation-positive essential thrombocythemia treated with baricitinib. The patient was a 72-year-old male. He was diagnosed with rheumatoid arthritis at a local clinic in April 2018. Methotrexate (MTX) was started and the dose was increased to 16 mg/week. In October of the same year, anaemia was observed and MTX was reduced, but anaemia progressed. Blood tests showed pancytopenia, and he was referred to Rheumatology on suspicion of drug-induced pancytopenia. Pancytopenia improved with discontinuation of MTX and administration of folic acid. His platelet count was markedly increased to 1,400,000/μl at one point, decreased to 400,000/μl, and then gradually increased to 700,000-1,000,000/μl. Despite taking an antiplatelet drug, he developed cerebral infarction in June 2019. The JAK2V617F mutation was noted, and he was diagnosed with essential thrombocythemia. Hydroxycarbamide was started, but the effect was insufficient. Baricitinib, a JAK1/2 inhibitor indicated for rheumatoid arthritis, was started in August with the expectation that it would also be effective for essential thrombocythemia. The platelet count decreased to ∼400,000-600,000 cells/μl, and a decrease in the C-reactive protein level and the improvement of arthritis were noted. We report this case because it is considered to be a valuable case, suggesting that baricitinib may be effective for essential thrombocythemia.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"1-4"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9545338","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":"Simultaneous hybrid closed-wedge supramalleolar osteotomy and ankle arthrodesis for post-traumatic varus ankle arthritis with periarticular deformity: A case report.","authors":"Hayato Kobayashi, Yoji Shido","doi":"10.1093/mrcr/rxad033","DOIUrl":"10.1093/mrcr/rxad033","url":null,"abstract":"<p><p>The treatment of end-stage ankle arthritis associated with extra-articular tibial deformity, especially for ankles deformed by previous traumatic injuries and/or surgical treatment, is extremely challenging but rewarding. Only one previous report describes simultaneous tibial deformity correction and ankle arthrodesis for tibial malalignment and ipsilateral ankle arthritis. Here, we describe a unique case of post-traumatic ankle osteoarthritis concomitant with extra-articular varus deformity in a 77-year-old female. We have combined medial opening-wedge supramalleolar osteotomy (SMO) and lateral closed-wedge SMO in this case, which we termed 'hybrid' closed-wedge SMO, to overcome the limitations of traditional closed-wedge SMO. The patient was successfully treated with simultaneous hybrid closed-wedge SMO and ankle arthrodesis using a single lateral locking plate. To our knowledge, this is the first report describing the successful introduction of a hybrid closed-wedge osteotomy procedure in the distal tibia. Three years after surgery, the patient could walk without assistance and swim normally. The patient experienced no discomfort or pain in the operated ankle and was satisfied with the results. Radiographs confirmed that the pre-existing ankle joint line was parallel to the ground and almost invisible. The hind foot alignment was slightly valgus. No progression of the subtalar joint arthritis was detected. The simultaneous hybrid closed-wedge SMO and ankle arthrodesis was technically difficult but effective. This technique preserves leg length and subtalar joint motion. Additionally, a single lateral incision minimises the risk of impaired blood supply. The one-stage surgical treatment reduces the recovery time, duration of hospitalisation, and surgical costs. Rigid locking fixation with careful postoperative weight-bearing is required for uneventful bone healing.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"37-42"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9601339","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}
Ayako Makiyama, Yoshiyuki Abe, Hoshiko Furusawa, Masahiro Kogami, Taiki Ando, Kurisu Tada, Mitsuho Onimaru, Akihiro Ishizu, Ken Yamaji, Naoto Tamura
{"title":"Polyarteritis nodosa diagnosed in a young male after COVID-19 vaccine: A case report.","authors":"Ayako Makiyama, Yoshiyuki Abe, Hoshiko Furusawa, Masahiro Kogami, Taiki Ando, Kurisu Tada, Mitsuho Onimaru, Akihiro Ishizu, Ken Yamaji, Naoto Tamura","doi":"10.1093/mrcr/rxad037","DOIUrl":"10.1093/mrcr/rxad037","url":null,"abstract":"<p><p>In response to the coronavirus disease 2019 pandemic, the coronavirus disease 2019 vaccine was rapidly developed and the effectiveness of the vaccine has been established. However, various adverse effects have been reported, including the development of autoimmune diseases. We report a case of new-onset polyarteritis nodosa in a 32-year-old male following the coronavirus disease 2019 vaccination. The patient developed limb pain, fever, pulmonary embolism, multiple subcutaneous nodules, and haematomas. Skin biopsy revealed necrotising inflammation accompanied by fibrinoid necrosis and high inflammatory cell infiltration in the walls of medium to small arteries. The symptoms resolved following corticosteroid treatment. Although it is difficult to prove a relationship between the vaccine and polyarteritis nodosa, similar cases have been reported and further reports and analyses are therefore necessary.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"125-132"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9630744","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":"Thrombotic thrombocytopenic purpura that developed 3 years after systemic lupus erythematosus had remitted with rituximab therapy.","authors":"Reina Tsuda, Toshiki Kido, Ikuma Okada, Aoi Kobiyama, Masatoshi Kawataka, Miho Yamazaki, Ryoko Asano, Hiroyuki Hounoki, Koichiro Shinoda, Kazuyuki Tobe","doi":"10.1093/mrcr/rxad032","DOIUrl":"10.1093/mrcr/rxad032","url":null,"abstract":"<p><p>Patients with systemic lupus erythematosus (SLE) occasionally develop thrombotic thrombocytopenic purpura (TTP), which can be fatal. Here, we report a case of TTP developing 3 years after SLE remitted with rituximab (RTX) therapy. A 50-year-old woman was treated with RTX for marked immune thrombocytopenic purpura and autoimmune haemolytic anaemia due to SLE relapse. After induction of remission, she was treated with prednisolone alone without maintenance therapy with RTX. Approximately 3 years later, she was readmitted with marked thrombocytopenia and severe renal dysfunction. On admission, she was diagnosed with TTP for the first time based on severe reduction in a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity and detection of ADAMTS13 inhibitors. CD19+ B cells in the patient's serum increased to 34%, suggesting that B cells had reactivated once the effect of RTX had subsided. The patient was successfully treated with plasmapheresis, glucocorticoid pulse therapy, and RTX. There are no previous reports of newly diagnosed TTP with ADAMTS13 inhibitor production after having achieved remission of SLE with RTX. Therefore, our report also discusses the potential mechanisms of production of new autoantibodies after B-cell depletion therapy.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"57-62"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9673006","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 of refractory enteritis and arthritis with combination of tumour necrosis factor and interleukin-6 inhibition in patients with ulcerative colitis.","authors":"Sho Ishigaki, Yasushi Kondo, Yuichiro Ota, Po-Sung Chu, Hironari Hanaoka, Tsutomu Takeuchi, Yuko Kaneko","doi":"10.1093/mrcr/rxad031","DOIUrl":"10.1093/mrcr/rxad031","url":null,"abstract":"<p><p>An 18 year-old man with autoimmune hepatitis-primary sclerosing cholangitis-overlap syndrome and ulcerative colitis was admitted due to relapsed enteritis and polyarthritis after cessation of infliximab. Colonoscopy and articular ultrasonography revealed large ulcers in the colon with crypt abscess in the specimens and active enthesitis and synovitis, respectively. His intestinitis was improved with golimumab but arthritis was persistent. Golimumab was switched to secukinumab, which was effective for arthritis. However, colitis was flared resulting in total colorectal resection. One month after colectomy, polyarthritis was relapsed. Tocilizumab ameliorated arthritis but enteritis emerged again, and switching tocilizumab to adalimumab improved enteritis but arthritis exacerbated. Finally, we restarted tocilizumab for arthritis with continued adalimumab for enteritis. The dual cytokine blocking strategy, tumour necrosis factor-α and interleukin-6 inhibition, subsided both of his refractory enteritis and arthritis and maintained remission for more than 3 years without any serious adverse event. Our case suggests that enteritis and arthritis in inflammatory bowel disease may be different in pathophysiology and raises the possible usefulness of simultaneous inhibition of two inflammatory cytokines in such cases.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"33-36"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9973808","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}