{"title":"An autopsy case of anti-MDA5 antibody-positive amyopathic dermatomyositis with an initial manifestation of panniculitis on the left upper arm.","authors":"Takafumi Onose, Toshiki Kido, Ikuma Okada, Miho Yamazaki, Hiroyuki Hounoki, Teruhiko Makino, Naotaka Yamauchi, Takashi Matsushita, Kazuyuki Tobe, Koichiro Shinoda","doi":"10.1093/mrcr/rxad038","DOIUrl":"10.1093/mrcr/rxad038","url":null,"abstract":"<p><p>A 53-year-old man was presented with refractory panniculitis on the left upper arm that had persisted for 10 months. The patient was diagnosed with lupus profundus, wherein oral glucocorticoid therapy was initiated. Four months prior, ulceration was observed in the same area. Dapson was administered instead, scarring the ulcer but enlarging the panniculitis. Five weeks earlier, he developed a fever, productive cough, and dyspnoea. Three weeks earlier, a skin rash was observed on the forehead, left auricle posterior to the neck, and extensor aspect of the left elbow. Chest computed tomography showed pneumonia in the right lung, after which the patient's dyspnoea worsened. The patient was admitted and diagnosed with anti-MDA5 antibody-positive amyopathic dermatomyositis (ADM) based on skin findings, hyperferritinaemia, and rapidly progressive diffuse lung shadows. Glucocorticoid pulse therapy, intravenous cyclophosphamide, and tacrolimus were initiated, and later, plasma exchange therapy was combined. However, his condition worsened and required management with extracorporeal membrane oxygenation. The patient expired on day 28 after hospitalisation. An autopsy revealed hyalinising to fibrotic stages of diffuse alveolar damage. Strong expression of myxovirus resistance protein A was observed in three skin biopsy specimens from the time of initial onset, consistent with ADM. Anti-MDA5 antibody-positive ADM not only manifests typical cutaneous symptoms, but also rarely occurs with localised panniculitis, such as in the present case. In patients with panniculitis of unknown aetiology, the possibility of initial symptoms of ADM should be included in the differential diagnosis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"86-90"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10025345","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 PR3-ANCA-positive interstitial pneumonia with a moderate dose of glucocorticoid and rituximab.","authors":"Misako Higashida-Konishi, Mitsuhiro Akiyama, Satoshi Hama, Tatsuhiro Oshige, Keisuke Izumi, Hisaji Oshima, Yutaka Okano","doi":"10.1093/mrcr/rxad024","DOIUrl":"10.1093/mrcr/rxad024","url":null,"abstract":"<p><p>Antineutrophil cytoplasmic antibody (ANCA)-positive interstitial pneumonia (IP) is reported as IP that is ANCA-positive and does not involve organ damage associated with vasculitis other than the lungs. While the combination of glucocorticoid and rituximab is effective in ANCA-associated vasculitis, the treatment strategy for ANCA-positive IP has not been established. Here, we report the first case of successful treatment of proteinase 3 (PR3)-ANCA-positive IP with a moderate dose of glucocorticoid and rituximab. The patient was an 80-year-old male who presented with subacute dry cough and dyspnoea. Blood tests revealed elevated levels of C-reactive protein, Krebs von den Lungen 6 (KL-6), and PR3-ANCA. Chest computed tomography (CT) showed interstitial shadows and infiltrates around honeycomb cysts. 18F-fluorodeoxyglucose (FDG) positron emission tomography CT revealed an uptake of FDG in the IP area. After starting treatment with a moderate dose of prednisolone and rituximab, the patient's clinical symptoms disappeared, C-reactive protein and KL-6 turned to be normal, and infiltrates around the cysts of honeycomb lungs disappeared. Prednisolone was gradually decreased to 2 mg, and no relapse or adverse events were observed during the course of treatment. Our case suggests that early treatment with a moderate dose of glucocorticoid and rituximab is effective for PR3-ANCA-positive IP.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":" ","pages":"137-140"},"PeriodicalIF":0.0,"publicationDate":"2023-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9888637","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 ureteral stenosis due to ureteritis probably associated with rheumatoid arthritis.","authors":"Norihiro Nagamura, Tomoyuki Sugitani","doi":"10.1093/mrcr/rxac071","DOIUrl":"https://doi.org/10.1093/mrcr/rxac071","url":null,"abstract":"<p><p>Ureteritis associated with the immunological disorder is rarely reported, and most cases in this category are small vessel vasculitis and immunoglobulin G4-related disease. Rheumatoid arthritis (RA)-associated ureteritis is uncommon, and underlying aetiology is unclear. We present a patient with ureteritis who had a medical history of RA and was successfully treated with steroids and immunosuppressant. A 49-year-old woman who had been treated for RA and atopic dermatitis suffered from gross haematuria for 5 successive days. Contrast-enhanced computed tomography (CT) showed right-dominant upper urinary tract dilatation with enhanced thickened wall. The haematuria continued accompanied with intermittent right back and lower abdominal pain, and the following CT image taken after 3 months presented the progression to bilateral hydronephrosis. Ureteral stents were placed, and antibiotic therapy was introduced for obstructive pyelonephritis. Ureterocystoscopy and following biopsy from the upper ureteral tract showed a chronic inflammatory change in the histopathology, and we finally considered the stenosing ureteritis to be caused by immune-mediated mechanism related to RA. After starting steroid therapy with methotrexate, therapeutic response was obtained to remove the stents. In the cases of ureteritis or ureteral stenosis of unknown aetiology with a medical history of immunological disorders, we should consider the underlying immune-activated state and try to test contrast-enhanced CT and histological examination before performing a surgical procedure. After excluding the common causes of ureteritis or ureteral stenosis, these tests would support the appropriate diagnosis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"335-339"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9668294","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 home-based low-intensity resistance exercise programme with supervision for secondary sarcopenia in a patient with established rheumatoid arthritis: A case report.","authors":"Norikazu Hishikawa, Shogo Toyama, Noriyoshi Shimahara, Kazuaki Taira, Takashi Kawasaki, Suzuyo Ohashi, Koshiro Sawada, Yasuo Mikami","doi":"10.1093/mrcr/rxad018","DOIUrl":"https://doi.org/10.1093/mrcr/rxad018","url":null,"abstract":"<p><p>Patients with established rheumatoid arthritis (RA) are at risk of developing secondary sarcopenia. Both pharmacological and exercise interventions are essential for treatment. However, for such patients, exercise loads may lead to the progression of joint destruction and worsening of pain. The purpose of this report was to assess the feasibility of a home-based, low-intensity resistance exercise programme. The case was a 70-year-old female patient with sarcopenia secondary to RA. She was diagnosed with RA at 57 years of age and began medication, maintaining remission of disease activity. However, she did not exercise habitually and was relatively inactive in her daily life. Furthermore, she had kinesiophobia associated with her RA-related symptoms. We suggested implementing a home-based low-intensity resistance exercise programme >3 months, with the goal of exercising the lower extremities daily. The patient was asked to self-monitor using an exercise diary and attend monthly outpatient visits for repeat counselling and feedback. As a result, she was able to perform the exercise programme safely with high compliance, improving her kinesiophobia as well as physical inactivity, although her skeletal muscle mass did not change. Regular exercise and increased physical activity may help prevent the onset of sarcopenia. These results demonstrate the feasibility of implementing our exercise programme and support its potential for preventing the progression of sarcopenia. While questions remain in terms of treating sarcopenia, we believe that our findings will lead to the establishment of an exercise programme for patients with sarcopenia secondary to established rheumatoid arthritis.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"354-358"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10026494","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}
Takeshi Suga, Yukiko Hidaka, Maisa Hori, Hiroshi Yamasaki, Daisuke Wakasugi, Satoshi Yamasaki, Rin Yamaguchi, Hiroaki Ida, Munetoshi Nakashima
{"title":"Successful treatment of a patient with Takayasu's arteritis complicated with Crohn's disease with ustekinumab: A case report.","authors":"Takeshi Suga, Yukiko Hidaka, Maisa Hori, Hiroshi Yamasaki, Daisuke Wakasugi, Satoshi Yamasaki, Rin Yamaguchi, Hiroaki Ida, Munetoshi Nakashima","doi":"10.1093/mrcr/rxac090","DOIUrl":"https://doi.org/10.1093/mrcr/rxac090","url":null,"abstract":"<p><p>A 17-year-old woman was referred to our department with fever, general malaise, and weight loss. She was diagnosed with Takayasu arteritis (TAK) and Crohn's disease (CD) following positron emission tomography-computed tomography (PET-CT) and colonoscopy, respectively. Serological human leukocyte antigen (HLA) typing revealed HLA-B52 positivity. Initial treatment with prednisolone (PSL) (0.5 mg/kg) was insufficient; therefore, ustekinumab and 5-aminosalicylic acid were added. This treatment achieved PSL-free remission for both diseases, as confirmed by PET-CT and colonoscopy. Although treatment guidelines for TAK and CD have been previously established, treatment of patients with TAK with coexisting CD is controversial. Our case suggests that ustekinumab has the ability to achieve TAK remission in addition to its therapeutic effect on CD.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"448-452"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9661935","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":"Anti-glomerular basement membrane diseases and thrombotic microangiopathy treated with rituximab.","authors":"Nanase Honda, Rihiro Shigehara, Kazunori Furuhashi, Yoshiki Nagai, Naoto Yokogawa","doi":"10.1093/mrcr/rxac091","DOIUrl":"https://doi.org/10.1093/mrcr/rxac091","url":null,"abstract":"<p><p>A 68-year-old male patient presented with a 2-week history of malaise and anuria. Renal replacement therapy with haemodialysis was begun for acute kidney injury. His anti-glomerular basement membrane (anti-GBM) antibody titre was 3060 U/ml. Based on this finding, anti-GBM disease was diagnosed. Plasmapheresis and high-dose glucocorticoid therapy were begun, but his haemolytic anaemia and thrombocytopenia progressed. A disintegrin and metalloprotease with thrombospondin type 1 motif, 13 (ADAMTS-13) activity decreased to 33%, but no inhibitor was detected. Secondary thrombotic microangiopathy was suspected, and rituximab therapy was begun. The addition of rituximab is thought to have further reduced the anti-GBM antibodies, prevented recurrence, stabilised the platelet count, and facilitated the patient's withdrawal from plasmapheresis and glucocorticoid therapy. Rituximab may be a viable therapeutic option for anti-GBM diseases complicated with thrombotic microangiopathy.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"422-425"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9668286","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":"Non-bacterial vertebral osteitis as the first manifestation of pustulotic arthro-osteitis.","authors":"Yoshitaka Ueda, Naoto Yokogawa, Kota Shimada","doi":"10.1093/mrcr/rxad016","DOIUrl":"https://doi.org/10.1093/mrcr/rxad016","url":null,"abstract":"<p><p>Pustulotic arthro-osteitis (PAO) is an osteoarticular comorbidity of palmoplantar pustulosis, a chronic, recurrent, inflammatory skin disease presenting with erythema, scales, and pustules on the palms and soles. Palmoplantar pustulosis is one of the most common skin diseases in Japan and is accompanied by PAO in 10-30% of patients. PAO often involves anterior chest wall lesions, but vertebral involvement is uncommon. The present report describes a case of PAO in which the initial manifestation was only non-bacterial vertebral osteitis, with palmoplantar pustulosis developing 8 months after its onset. A patient with vertebral osteitis of unknown aetiology should be followed up and examined periodically for skin problems, which may provide a clue to the presence of PAO.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"488-490"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9668817","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":"Two cases of systemic lupus erythematosus after administration of severe acute respiratory syndrome coronavirus 2 vaccine.","authors":"Mayu Sakai, Ken Takao, Masami Mizuno, Hironori Ando, Yui Kawashima, Takehiro Kato, Saki Kubota, Tokuyuki Hirose, Takuo Hirota, Yukio Horikawa, Daisuke Yabe","doi":"10.1093/mrcr/rxad008","DOIUrl":"https://doi.org/10.1093/mrcr/rxad008","url":null,"abstract":"<p><p>Although there is a great demand for increased coronavirus disease 2019vaccination worldwide, rare side effects of the vaccines in susceptible individuals are attracting attention. We recently treated two patients who developed systemic lupus erythematosus after administration of a severe acute respiratory syndrome coronavirus 2 vaccine from Pfizer-BioNTech or Moderna. While causal relationships between vaccination and adverse events are difficult to discern due to both confounding and masking factors, our findings suggest that attention to possible adjuvant-related autoimmune diseases in certain individuals receiving severe acute respiratory syndrome coronavirus 2 vaccines is appropriate.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"378-382"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9724090","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}
Jack Pepys, Robin J Borchert, Narmathy Thambirajah, Cyrus Daruwalla, Dimitrios Apostolopoulos, Dominic G O'Donovan, Timothy Ham, Charlotte Brierley
{"title":"A rare presentation of rapidly progressing myopathy in an adolescent.","authors":"Jack Pepys, Robin J Borchert, Narmathy Thambirajah, Cyrus Daruwalla, Dimitrios Apostolopoulos, Dominic G O'Donovan, Timothy Ham, Charlotte Brierley","doi":"10.1093/mrcr/rxac097","DOIUrl":"https://doi.org/10.1093/mrcr/rxac097","url":null,"abstract":"<p><p>We present a case of severe juvenile dermatomyositis with limited response to steroids in an adolescent who developed symptoms within hours after receiving Pfizer BNT162b2 coronavirus disease 2019 vaccine. The patient presented with severe weakness of proximal muscles, dyspnoea, and tachycardia. His muscle enzymes were raised, and he was diagnosed with severe juvenile dermatomyositis following magnetic resonance imaging and muscle biopsy. His management was challenging, requiring multidisciplinary input, and difficult decisions with regard to the appropriate immunomodulatory treatments. The patient had to undergo escalating immunosuppressive treatments before he began to recover clinically and biochemically. To our knowledge, this is the first case in an adolescent although a few cases of similar presentations following coronavirus disease 2019 vaccination have been reported in adults. Elucidating the potential relationship of the vaccine with this severe myopathy in an adolescent is important for global vaccination policies, but avoiding the conflation of association with causation is also crucial in the context of the pandemic.</p>","PeriodicalId":18677,"journal":{"name":"Modern Rheumatology Case Reports","volume":"7 2","pages":"410-415"},"PeriodicalIF":0.0,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10025962","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, Ryo Sasaki, Akira Ishiwata, Shuji Hatakeyama, Masami Matsumura, 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}