Praveen K Ramani, Florin Grigorian, Heidi Lightle, Saumya V Joshi
{"title":"Paediatric Cogan Syndrome masquerading as IgA vasculitis.","authors":"Praveen K Ramani, Florin Grigorian, Heidi Lightle, Saumya V Joshi","doi":"10.1093/mrcr/rxad060","DOIUrl":"10.1093/mrcr/rxad060","url":null,"abstract":"<p><p>Paediatric Cogan Syndrome is a rare and underrecognised autoimmune vasculitis characterised by ocular inflammation and sensorineural hearing loss. Its etiopathogenesis, diagnosis, and management are not well defined. We report a 12-year-old girl who initially presented with symptoms of IgA vasculitis formerly called Henoch Schoenlein Purpura (HSP) and eventually developed anterior uveitis and bilateral sensorineural hearing loss leading to the diagnosis of atypical Cogan Syndrome. The workup for infectious etiologies and other systemic rheumatologic disorders was negative. The management was multidisciplinary involving Rheumatology, Ophthalmology, Otorhinolaryngology, and Audiology. The anterior uveitis responded well to systemic glucocorticoids and Methotrexate, but the hearing loss was grossly progressive warranting a cochlear implant. We are not aware of Paediatric Cogan Syndrome being reported as a mimicker of IgA vasculitis previously in the literature. It is an important finding as IgA vasculitis is prevalent in the paediatric age group and new-onset ocular or vestibular symptoms after IgA vasculitis should alert the clinician to the possibility of Cogan Syndrome. In the absence of well-defined diagnostic criteria, it is crucial to recognise the clinical symptoms of Paediatric Cogan Syndrome for early diagnosis and treatment since the delay in diagnosis can lead to permanent disability.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71416413","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 dupilumab combination therapy for exacerbation of atopic dermatitis in a patient with eosinophilic granulomatosis with polyangiitis treated with mepolizumab.","authors":"Yosuke Iwadate, Yoshiyuki Arinuma, Yu Matsueda, Tomoki Tanaka, Tatuhiko Wada, Sumiaki Tanaka, Kenji Oku, Kunihiro Yamaoka","doi":"10.1093/mrcr/rxad059","DOIUrl":"10.1093/mrcr/rxad059","url":null,"abstract":"<p><p>We report a 60-year-old male with eosinophilic granulomatosis with polyangiitis (EGPA) complicated with atopic dermatitis (AD). The patient was initially treated with prednisolone, cyclosporine A, and mepolizumab (MEPO). Due to worsening skin symptoms after prednisolone tapering, dupilumab (DUP) was added as an adjunctive therapy for AD confirmed by skin biopsy. The combination therapy of MEPO and DUP resulted in rapid improvement of skin symptoms, suggesting it may be an effective therapeutic option for patients with EGPA and AD. This case report emphasises the importance of a multidisciplinary approach in treating complex diseases such as EGPA and AD.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41174765","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 atypical IgG4-related disease presenting hypereosinophilia, polyneuropathy, and liver dysfunction.","authors":"Hiroki Mukoyama, Kosaku Murakami, Hideo Onizawa, Mirei Shirakashi, Ryosuke Hiwa, Hideaki Tsuji, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Akio Morinobu","doi":"10.1093/mrcr/rxad053","DOIUrl":"10.1093/mrcr/rxad053","url":null,"abstract":"<p><p>Ig (immunoglobulin) G4-related disease (Ig4-RD) affects several organs, including salivary glands, lacrimal glands, pancreas, biliary ducts, and retroperitoneum. A 72-year-old woman was examined for hypereosinophilia, high levels of IgG4, polyneuropathy, liver dysfunction, enlargement of lymph nodes and lacrimal glands, and beaded dilation of the bile ducts. We diagnosed Ig4-RD based on biopsies of the lymph nodes, liver, and submandibular gland. The symptoms of the patient improved after glucocorticoid treatment. This was a novel and atypical case of Ig4-RD that was difficult to differentiate from other diseases, including eosinophilic granulomatosis with polyangiitis, idiopathic hypereosinophilic syndrome, and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes syndrome. This case report highlights the importance of biopsies in differentiating Ig4-RD.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41176014","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":"Total knee arthroplasty for neuropathic arthropathy in a patient with leprosy.","authors":"Sakumo Kii, Motoki Sonohata, Masaaki Mawatari","doi":"10.1093/mrcr/rxad057","DOIUrl":"10.1093/mrcr/rxad057","url":null,"abstract":"<p><p>Patients with leprosy are known to tend to develop neuropathic arthropathy, known as Charcot joint. There are no case reports of total knee arthroplasty (TKA) in patients with leprosy with polyarticular neuropathic arthropathy, and the results are unknown. In this study, we report a case of TKA in a patient with leprosy with polyarticular neuropathic arthropathy and discuss its outcomes and indications. Right TKA using the NexGen Legacy Constrained Condylar Knee implant was performed in a 62-year-old man with neuropathic arthropathy in multiple joints with clinical symptoms, particularly in the right knee. Seven years post-operation, the American Knee Society Score-knee and -function, which represent knee function and activities of daily living on a scale of 100 points, were significantly improved compared with preoperative values, from 30 to 99 points and 0 to 60 points, respectively. Indications for arthroplasty for neuropathic arthropathy should be carefully considered in each individual case. In this case, the patient had neuropathic arthropathy in multiple joints; however, TKA was performed because recovery of function in the right knee was expected to significantly improve the patient's activities of daily living, and a good mid-term clinical outcome was achieved. Therefore, indications for arthroplasty should be considered in patients with systemic neuropathic arthropathy such as leprosy, and with accurate assessment and appropriate implant selection, good long-term outcomes may be expected.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49686817","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":"Overlapping systemic lupus erythematosus, seropositive erosive rheumatoid arthritis, and ANCA-associated vasculitis: A case report and literature review.","authors":"Emma Reesor, Sankalp Virendrakumar Bhavsar","doi":"10.1093/mrcr/rxad056","DOIUrl":"10.1093/mrcr/rxad056","url":null,"abstract":"<p><p>The overlap of rheumatoid arthritis and systemic lupus erythematosus is well described, with a syndrome known as 'rhupus'. ANCA-associated vasculitis, however, is uncommonly associated with other autoimmune conditions. Here, we present a case of lupus, rheumatoid arthritis, and ANCA-associated vasculitis, with significant time elapsed between presentations, resulting in an accumulation of the three diagnoses over the course of 35 years. The patient was diagnosed with lupus at age 45, presenting with inflammatory polyarthritis, thrombocytopenia, leukopenia, positive ANA, positive anti-DNA, and hypocomplementemia. She was maintained on hydroxychloroquine therapy with minimal disease activity. Approximately 20 years later, she had a flare of polyarthritis with bilateral wrist erosive arthropathy and a positive rheumatoid factor, diagnosed as rheumatoid arthritis. Anti-TNFα therapy was initiated, and she was stable for a further 10 years. At age 79, she developed ANCA-associated vasculitis AAV with pulmonary and renal manifestations, treated with rituximab induction therapy and steroids. She recovered and her MPO-ANCA titre normalised. One year later, off the anti-TNFα, she again experienced an acute kidney injury with a repeat rise in MPO-ANCA. She was re-induced and maintained on steroids and rituximab. This novel case highlights the range of possible overlap syndromes, as well as how multiple autoimmune diagnoses can evolve over decades in previously stable disease. There is growing work around polyautoimmunity with ANCA-associated vasculitis and other systemic autoimmune diseases, which has potential to identify common aetiologies and pathogenesis. Knowledge of these overlap syndromes can help to recognise and manage these conditions in a timely manner.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41177708","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":"Arthritis after vaccination against SARS-CoV-2 infection: Reactive arthritis or post-vaccination arthritis?","authors":"Chokan Baimukhamedov, Galina Veklenko, Gaziza Smagulova","doi":"10.1093/mrcr/rxad058","DOIUrl":"10.1093/mrcr/rxad058","url":null,"abstract":"","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41242744","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":"Nontuberculous mycobacteriosis oligoarthritis of the right hand misdiagnosed as rheumatoid arthritis: A case report.","authors":"Yuji Joyo, Sanshiro Yasuma, Takuya Usami, Yusuke Hattori, Yohei Noda, Sakurako Kato, Ryohei Kondo, Soichiro Watanabe, Yuko Waguri-Nagaya","doi":"10.1093/mrcr/rxad052","DOIUrl":"10.1093/mrcr/rxad052","url":null,"abstract":"<p><p>Infection with Mycobacterium marinum has several different clinical presentations. Most commonly, it appears as a solitary papulonodular lesion on an extremity. A rare presentation of osteoarticular M. marinum involving multiple small joints and tenosynovitis of the hand, which was misdiagnosed as rheumatoid arthritis, is reported. The patient was initially treated for seronegative rheumatoid arthritis but failed to respond to methotrexate. Magnetic resonance imaging showed arthritis and tenosynovitis. Subsequently, synovial biopsy led to histological and microbiological diagnosis. Antimycobacterial treatment should be started promptly in such cases. The combined use of rifampicin, ethambutol, and clarithromycin appears to be effective, and debridement is indicated in patients with deep-seated infections.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41172234","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":"Correction to: A case of atypical IgG4-related disease presenting hypereosinophilia, polyneuropathy, and liver dysfunction.","authors":"","doi":"10.1093/mrcr/rxad066","DOIUrl":"10.1093/mrcr/rxad066","url":null,"abstract":"","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138471453","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}