Maria João Cadório, João Oliveira, João Gama, Cátia Duarte
{"title":"Kikuchi-Fujimoto Disease and primary Sjögren's Syndrome coexisting: a case-based literature review.","authors":"Maria João Cadório, João Oliveira, João Gama, Cátia Duarte","doi":"10.1093/mrcr/rxae058","DOIUrl":"https://doi.org/10.1093/mrcr/rxae058","url":null,"abstract":"<p><p>Kikuchi-Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotizing lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterized by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with NSAIDs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with Systemic Lupus Erythematosus, and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren's Syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren Syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142305106","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":"Tense blisters and hemorrhagic bullae as the first manifestation of eosinophilic granulomatosis with polyangiitis.","authors":"Hajime Inokuchi, Mitsuhiro Akiyama, Hiroto Horikawa, Yasushi Kondo, Shuntaro Saito, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko","doi":"10.1093/mrcr/rxae056","DOIUrl":"https://doi.org/10.1093/mrcr/rxae056","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis (EGPA) poses a significant diagnostic challenge due to its varied clinical presentation. Here, we present a case of a 59-year-old female with a history of asthma and sinusitis, who manifested with an extremely rare presentation of drastic tense blisters and hemorrhagic bullae alongside purpuric lesions and peripheral neuropathy. Examinations revealed eosinophilia, positive anti-neutrophil cytoplasmic antibody, and characteristic pathological findings with small vessel vasculitis in the purpura. Treatment with glucocorticoids and cyclophosphamide led to rapid improvement in peripheral eosinophilia, skin manifestations and motor neuron deficits. Although rare, our case underscores that bullous skin lesions should be recognized as a potential cutaneous hallmark of EGPA to aid timely diagnosis, since prompt treatment initiation is crucial given the potential irreversible organ damage and poor prognosis of EGPA.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142305107","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 eosinophilic granulomatosis with polyangiitis preceded by allergic bullous lesions.","authors":"Eiko Hasegawa, Momoko Iijima, Akinari Sekine, Noriko Inoue, Kiho Tanaka, Tatsuya Suwabe, Masayuki Yamanouchi, Yuki Oba, Ayako Kume, Kei Kono, Keiichi Kinowaki, Kenichi Ohashi, Yutaka Yamaguchi, Yasuhiro Suyama, Takehiko Wada, Naoki Sawa, Kotono Takahashi, Shigeharu Ueki, Yoshifumi Ubara","doi":"10.1093/mrcr/rxae046","DOIUrl":"https://doi.org/10.1093/mrcr/rxae046","url":null,"abstract":"<p><p>A 62-year-old man with a history of diabetes mellitus was hospitalised with numbness of lower limbs, bullous lesions of the whole body, kidney dysfunction, presence of eosinophils, and elevated antineutrophil cytoplasmic antibodies to myeloperoxidase and anti-bullous pemphigoid 180 antibodies and was diagnosed with mononeuritis multiplex. Kidney and muscle biopsies showed vasculitis with fibrinoid necrosis, whereas skin biopsies showed only blister formation between the epidermis and dermis; a high eosinophilic infiltrate was present in all three tissues. These findings led to a diagnosis of eosinophilic granulomatosis with polyangiitis combined with allergic bullous lesions. Immunohistological examination indicated cytolytic eosinophils and extracellular traps, suggesting the presence of eosinophil extracellular trap cell death (eosinophil ETosis) in diseased tissue.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142305104","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":"Elderly-onset inflammatory myopathy associated with Sjögren's syndrome following SARS-CoV-2 vaccination.","authors":"Yuta Komori, Satomi Kobayashi, Keiko Hatano, Yuko Saito, Tomio Arai, Kanae Kubo","doi":"10.1093/mrcr/rxae055","DOIUrl":"https://doi.org/10.1093/mrcr/rxae055","url":null,"abstract":"<p><p>As vaccination against SARS-CoV-2 has progressed, various autoimmune diseases, including inflammatory myopathies, have been reported to develop after vaccination. Sjögren's syndrome (SS) sometimes presents as extra-glandular manifestations including inflammatory myopathy. In this report, we describe a case of inflammatory myopathy associated with SS that occurred in an atypically elderly patient after receiving the first dose of the SARS-CoV-2 mRNA vaccine (BNT162b2). The inflammatory myopathy was pathologically classified into non-specific myositis and characterised by predominant infiltration of the B cell lineage in this case. Combined treatment with glucocorticoid, intravenous immunoglobulin, and immunosuppressant resulted in an improvement in swallowing function and muscle strength. While we recognise the efficacy and safety of SARS-CoV-2 vaccines, we also emphasise the importance of recognising that individuals with an immunogenetic predisposition such as positivity of anti SS-A antibody may show disease activity including inflammatory myopathy following vaccination in SS, even at an atypically old age.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142305105","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":"Acute Heart Failure with Reduced Ejection Fraction Following Ozoralizumab in A Patient with Rheumatoid Arthritis: A Case Report.","authors":"Takaaki Ito, Yuji Miyoshi","doi":"10.1093/mrcr/rxae053","DOIUrl":"https://doi.org/10.1093/mrcr/rxae053","url":null,"abstract":"<p><p>Ozoralizumab (OZR), a novel next-generation tumor necrosis factor (TNF) inhibitor with variable heavy-chain domains of heavy-chain-only antibodies, named Nanobody®, was approved in September 2022 as the sixth TNF inhibitor in Japan. Other previous TNF inhibitors have been associated with various adverse drug reactions (ADRs), including heart failure (HF). The real-world data on these rare but clinically significant ADRs associated with OZR is lacking. Herein, we report a case of an 81-year-old female patient with rheumatoid arthritis who was insufficiently responsive to previous TNF inhibitors and developed HF with reduced ejection fraction (HFrEF) after the first OZR administration. Her condition improved after OZR discontinuation, suggesting that OZR may have precipitated the HFrEF despite tolerance with previous TNF inhibitors. Further studies are warranted to elucidate the mechanism and incidence of OZR-associated HF.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147239","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 Japanese case of VEXAS syndrome after COVID-19 vaccination: Comparison with previously reported cases.","authors":"Yui Miyagi, Hiroshi Kobayashi, Yoshihiro Umebayashi, Akira Okimura, Munehide Nakatsugawa, Ayaka Maeda, Yohei Kirino, Akiko Aoki","doi":"10.1093/mrcr/rxae054","DOIUrl":"https://doi.org/10.1093/mrcr/rxae054","url":null,"abstract":"<p><p>VEXAS syndrome is a novel adult-onset autoinflammatory disorder caused by variants in the UBA1 gene. Here, we report a Japanese case of VEXAS syndrome in which symptoms began one day after the second booster dose of a coronavirus disease 2019 (COVID-19) messenger ribonucleic acid vaccine, and a UBA1 variant was subsequently confirmed. Combined with the three cases reported thus far, this suggests that the COVID-19 vaccine may be one of the triggers for development of VEXAS syndrome in Asian populations. Since COVID-19 vaccines have been reported to be associated with various autoinflammatory and autoimmune diseases, it is important to continue to pay close attention to the relationship between COVID-19 vaccines and VEXAS syndrome.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147238","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":"Induced membrane technique and intramedullary beaming for the extensive bone defect of the talonavicular joint: A case report and literature review.","authors":"Yuki Ohue, Kosho Togei, Hiroaki Shima, Yoshihiro Hirai, Ken Tanaka, Masashi Neo","doi":"10.1093/mrcr/rxae048","DOIUrl":"https://doi.org/10.1093/mrcr/rxae048","url":null,"abstract":"<p><p>Cases involving both the induced membrane technique (IMT) and intramedullary beaming (IB) are generally rare. Here, we report such a case in an 83-year-old man who presented with left midfoot pain. Pyogenic arthritis was suspected based on clinical findings, and curettage was performed, revealing an extensive bone defect. The patient was clinically diagnosed with seronegative rheumatoid arthritis (RA). Therefore, the patient underwent both IMT and IB for the extensive bone defect in the talus and navicular regions caused by seronegative RA. The patient exhibited satisfactory short-term outcomes.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134959","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":"Reply to the Letter from Suzuki K et al.","authors":"Koto Hattori, Shigeru Tanaka, Hiroshi Nakajima","doi":"10.1093/mrcr/rxae052","DOIUrl":"https://doi.org/10.1093/mrcr/rxae052","url":null,"abstract":"","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142116535","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":"Epstein-Barr Virus-Encoded RNA-Positive Lymphocytes in Bone Marrow and Lymph Nodes in an Autopsy Case of TAFRO Syndrome.","authors":"Nao Tsugita, Ippei Miyagawa, Shingo Nakayamada, Aya Nawata, Shumpei Kosaka, Masanobu Ueno, Yoshiya Tanaka","doi":"10.1093/mrcr/rxae049","DOIUrl":"https://doi.org/10.1093/mrcr/rxae049","url":null,"abstract":"<p><p>A 55-year-old man was admitted to the hospital with vomiting, diarrhoea, and chest pain. Upon examination, he exhibited signs of increased inflammatory response, acute kidney injury, and thrombocytopenia, leading to a diagnosis of TAFRO syndrome, which was supported by the clinical evidence of generalized lymphadenopathy, pleural effusion, and hepatosplenomegaly. Despite receiving intensive multimodal immunosuppressive therapy, including glucocorticoid pulse therapy (methylprednisolone 1,000 mg/day), tocilizumab, and cyclosporine in the intensive care unit, the patient showed minimal response and succumbed to the disease on the seventh day of hospitalization. Histopathological analysis of the lymph nodes revealed idiopathic multicentric Castleman disease (iMCD)-like features, and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization identified multiple EBER-positive cells. These findings highlight the elusive pathogenic mechanism of TAFRO syndrome and the potential resistance of some patients to standard treatments such as tocilizumab. The presence of EBER-positive cells in lymph nodes or bone marrow may serve as an indicator of disease severity and treatment resistance. Therefore, histopathological detection of EBER-positive cells may help predict responsiveness to conventional treatments, disease severity, and prognosis in patients with TAFRO syndrome.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142057718","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}