{"title":"Successful treatment of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapidly progressive interstitial lung disease complicated by bilateral breast cancer following the additional tofacitinib: A case report.","authors":"Takashi Yamane, Midori Kitayama","doi":"10.1093/mrcr/rxae060","DOIUrl":"10.1093/mrcr/rxae060","url":null,"abstract":"<p><p>Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody-positive dermatomyositis (MDA5-DM) often causes rapidly progressive interstitial lung disease (RP-ILD). Although cancer complications in MDA5-DM are less frequently reported compared to other forms of DM, they do occur. For MDA5-DM patients with cancer, particularly in paraneoplastic settings, the primary treatment strategy often targets the malignancy first. However, surgery, chemotherapy, and radiotherapy carry significant risks of exacerbating ILD, especially in patients with respiratory failure. Despite improved prognosis with initial immunosuppressive combination therapy, some cases of MDA5-DM with RP-ILD remain refractory to treatment. Recent studies have shown the potential benefit of Janus kinase (JAK) inhibitors for refractory cases, though their impact on cancer progression remains a concern. We report a 48-year-old woman with MDA5-DM, RP-ILD, and bilateral breast cancer. Due to her respiratory condition, radical surgery and chemotherapy were initially not possible. Endocrine therapy and immunosuppressive therapy were administered, but the disease remained refractory. Tofacitinib, combined with plasma exchange, improved her ILD, allowing for bilateral mastectomy. One year later, MDA5 antibody titers became negative, and glucocorticoids were discontinued after two years. Three years later, neither MDA5-DM nor breast cancer has recurred. This is the first report of MDA5-DM complicated by breast cancer, and the first use of JAK inhibitors in such a case. Effective treatment requires close collaboration with oncologists to balance the risks and benefits of therapy in cases of MDA5-DM with RP-ILD and cancer.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"93-99"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335402","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":"Granulomatosis with polyangiitis with lacrimal gland enlargement and pancreatic swelling: A case report and a literature review.","authors":"Tetsuya Kawajiri, Shigeru Iwata, Katsunori Tanaka, Takeru Sonoda, Mizuki Nishikawa, Ryuta Iwamoto, Yuichi Takahashi, Fumiyoshi Kojima, Takao Fujii","doi":"10.1093/mrcr/rxae066","DOIUrl":"10.1093/mrcr/rxae066","url":null,"abstract":"<p><p>A 62-year-old man had bilateral eyelid swelling for 4 months. Two months before admission, he developed fatigue and lost 5 kg of body weight. Further examination revealed elevated serum C-reactive protein, normal angiotensin-converting enzyme, elevated proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA), and normal immunoglobulin (Ig)G4 concentration. Chest X-ray and computed tomography showed no enlarged hilar lymph nodes, but positron emission tomography-computed tomography showed fluorodeoxyglucose accumulation in both lacrimal glands, in lung nodules, and in the pancreas. Tissue biopsies of the lacrimal glands and pulmonary nodules showed granuloma with giant cells, but no IgG4-positive cells or fibrosis. Pancreatic tissue showed no findings of autoimmune pancreatitis. In the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis, the total score was 10 points. Final comprehensive diagnosis was granulomatosis with polyangiitis, based on the negative results of differential diseases, such as IgG4-related diseases and sarcoidosis. Prednisolone 60 mg/day was started on Day 8, and rituximab 500 mg/body/week on Day 12. After beginning treatment, general malaise and lacrimal gland enlargement were resolved, PR3-ANCA and C-reactive protein became negative, and the nodular shadow in the lungs disappeared. This is the first report of granulomatosis with polyangiitis presenting both lacrimal gland and pancreatic lesions.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"155-162"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515545","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":"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 HF with reduced ejection fraction 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":" ","pages":"1-5"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","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":"Mid-term outcomes of distal tibial oblique osteotomy in patients with rheumatoid arthritis: A report of three cases.","authors":"Takaaki Noguchi, Makoto Hirao, Kosuke Ebina, Yuki Etani, Gensuke Okamura, Manabu Sakata, Taihei Miura, Seiji Okada, Jun Hashimoto","doi":"10.1093/mrcr/rxae068","DOIUrl":"10.1093/mrcr/rxae068","url":null,"abstract":"<p><p>Although distal tibial oblique osteotomy (DTOO) has usually been indicated for noninflammatory diseases such as osteoarthritis, we encountered three patients with rheumatoid arthritis (RA) who underwent DTOO. We evaluated radiographic parameters and clinical scores, including self-reported scores for the foot and ankle [self-administered foot evaluation questionnaire (SAFE-Q)] and the Japanese Society for Surgery of the Foot (JSSF) ankle/hindfoot scale. In all three cases, radiographic improvements were achieved along with improvements in SAFE-Q and JSSF scores after mid-term follow-up. Supramalleolar osteotomy including DTOO could be indicated even for inflammatory diseases such as RA if erosive changes are not evident in the ankle joint.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"15-19"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515546","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":"Persistent liver injury following avacopan discontinuation in antineutrophil cytoplasmic antibody-associated vasculitis: A rare case of liver biopsy in the chronic phase of liver injury.","authors":"Hiroyuki Yamaguchi, Nayu Fujii, Hideki Shimizu","doi":"10.1093/mrcr/rxae071","DOIUrl":"10.1093/mrcr/rxae071","url":null,"abstract":"<p><p>Drug-induced liver injury is a major concern associated with the use of avacopan, a new therapeutic agent for antineutrophil cytoplasmic antibody-associated vasculitis. Here, we report a rare instance of liver biopsy performed for persistent liver injury following discontinuation of avacopan in an elderly woman with microscopic polyangiitis. The patient developed cholestatic liver injury after 2 months of avacopan therapy, which led to drug cessation. Despite the discontinuation of avacopan followed by supportive care, the liver enzyme levels remained elevated, necessitating liver biopsy. Histological examination revealed residual inflammation and focal necrosis around the portal vein, indicating ongoing liver injury despite avacopan withdrawal. This case report highlights the hepatotoxic potential of avacopan, the importance of vigilant liver function monitoring, and the value of liver biopsy in the chronic phase of avacopan-induced liver injury. Further research is required to elucidate avacopan hepatotoxicity mechanisms and identify potential risk factors for drug-induced liver injury in patients with antineutrophil cytoplasmic antibody-associated vasculitis.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"163-167"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142645345","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":"Unique presentation of acute neuro-Behçet's disease involving a cytotoxic oedema core surrounded by vasogenic oedema.","authors":"Shohei Yamashita, Daiki Fujimori, Shigemoto Igari, Yusuke Yamamoto, Takahiro Mizuuchi, Hiroaki Mori, Haeru Hayashi, Koichiro Tahara, Tetsuji Sawada","doi":"10.1093/mrcr/rxae032","DOIUrl":"10.1093/mrcr/rxae032","url":null,"abstract":"<p><p>A 53-year-old woman with recurrent stomatitis, genital ulcers, and folliculitis was admitted to Tokyo Medical University Hospital after experiencing visual disturbances for the past 2 weeks and a nonthrobbing headache for the past 3 days. She had also developed numbness in her left extremities. An ophthalmological examination revealed inflammatory changes in the eye. Cerebrospinal fluid analysis showed increased cell counts, protein, and interleukin-6 levels. Brain magnetic resonance imaging revealed multiple high signal intensities on T2-weighted/fluid-attenuated inversion recovery images of the pons and occipital and parietal lobes. The T2-weighted/fluid-attenuated inversion recovery high-signal-intensity lesion in the pons was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient mapping, suggesting cytotoxic oedema. Another high-signal-intensity lesion on T2-weighted/fluid-attenuated inversion recovery was isointense to hyperintense on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, indicating vasogenic oedema. The vasogenic oedema in the left occipital lobe contained a small core that was hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient, suggesting cytotoxic oedema. The patient was diagnosed with acute neuro-Behçet's disease and responded well to high-dose glucocorticoid and colchicine treatment. The present report emphasises that patients with acute neuro-Behçet's disease may present with cytotoxic oedema in the pons and cerebral spheres. Further reports of similar cases would contribute to a better understanding of the role of cytotoxic oedema in the pathophysiology of neuro-Behçet's disease and help elucidate the mechanisms underlying a unique presentation characterised by a central cytotoxic oedema core within vasogenic oedema.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"188-192"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141319400","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":"Anaphylaxis and prolonged allergic reactions caused by intra-articular injection of diclofenac etalhyaluronate in knee osteoarthritis: A case report.","authors":"Tomohito Takeshige, Ryo Koyama, Yuta Hotchi, Kenji Kawai, Makiko Kohmaru, Takashi Akimoto, Junko Watanabe, Toshifumi Yae, Kenji Kido, Manabu Sugita, Kazuhisa Takahashi","doi":"10.1093/mrcr/rxae067","DOIUrl":"10.1093/mrcr/rxae067","url":null,"abstract":"<p><p>Osteoarthritis (OA) is a common condition. The treatment of knee OA aims to improve the quality of life and clinical symptoms, mainly knee pain. To develop a new treatment option, diclofenac etalhyaluronate (ONO-5704/SI-613; trade name: JOYCLU) was developed. Its anti-inflammatory and pain-relieving effects have been demonstrated. Conversely, adverse events have also been reported. Here, we report the case of a 71-year-old female who presented with anaphylaxis and prolonged allergic reactions after an intra-articular injection of JOYCLU. A basophil activation test (BAT) was performed to determine the cause of the anaphylaxis. BAT showed no positive findings. As the incidence of knee OA increases, the number of cases of JOYCLU use is expected to grow; therefore, careful administration is required. In this case, the cause was unknown by BAT. Thus, further development of appropriate testing methods is necessary. Intra-articular allergic reactions in knee OA may be modified and worsened besides normal allergic reactions; therefore, caution is required.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"53-56"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515544","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}
Ilaria Girolami, Reinhard Walter Kluge, Lorenz Gallmetzer, Cecilia Albi, Paola Sorgi, Christian Dejaco, Martina Tauber, Esther Hanspeter, Peter Matzneller
{"title":"Multicentric reticulohistiocytosis with oral and laryngeal involvement in association with autoimmune/inflammatory syndrome induced by adjuvants: Expanding the spectrum of two uncommon entities.","authors":"Ilaria Girolami, Reinhard Walter Kluge, Lorenz Gallmetzer, Cecilia Albi, Paola Sorgi, Christian Dejaco, Martina Tauber, Esther Hanspeter, Peter Matzneller","doi":"10.1093/mrcr/rxae040","DOIUrl":"10.1093/mrcr/rxae040","url":null,"abstract":"<p><p>Multicentric reticulohistiocytosis (MRH) is the most frequent entity in the group of reticulohistiocytoses. It is usually accompanied by symmetrical erosive polyarthritis and is frequently associated with cancer and autoimmune disorders. Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) is an inflammatory syndrome triggered by adjuvants such as those contained in vaccines or by silicone implants. Here, we report the case of a 71-year-old woman with a history of breast cancer treated with surgery and subsequent prosthesis who developed a systemic hyperinflammatory syndrome including seronegative symmetric polyarthritis, multiple skin lesions, and two large nodular lesions in the oral cavity and larynx. The clinical picture was consistent with a clinical diagnosis of ASIA, with breast implant rupture and/or vaccination against severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) as possible triggers. Histopathology of skin, oral nodules, and laryngeal nodules revealed cutaneous/mucous and submucosal infiltration of large epithelioid mononuclear or binucleated cells with fine granular ground glass-like cytoplasm and round to kidney-shaped nuclei with prominent nucleoli, without atypical features or relevant pleomorphism, accompanied by sparse giant cells and lymphocytes. These cells stained positive for CD68 and CD45 and negative for S100, CD1a, and markers of epithelial or neural/melanocytic differentiation, altogether consistent with a diagnosis of reticulohistiocytosis. Clinicopathological correlation allowed the final diagnosis of MRH. To our knowledge, this is the first report of a co-occurrence of MRH with ASIA, and this is relevant to broaden the spectrum of both these rare diseases.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"200-206"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141891405","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":"Isolated central nervous system lymphomatoid granulomatosis in an older adult patient with systematic lupus erythematosus: A case report.","authors":"Takayuki Kambe, Makoto Yamaguchi, Takayuki Katsuno, Hirokazu Sugiyama, Keisuke Kamiya, Kentaro Imai, Hiroshi Kinashi, Shogo Banno, Yasuhiko Ito, Takuji Ishimoto","doi":"10.1093/mrcr/rxae062","DOIUrl":"10.1093/mrcr/rxae062","url":null,"abstract":"<p><p>Lymphomatoid granulomatosis (LYG) is a rare, T-cell-rich Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative systemic disease. Only a few cases of LYG have been described in patients with autoimmune disorders, with only one case described in a patient with systemic lupus erythematosus (SLE). However, no cases of isolated central nervous system (CNS)-LYG have been reported in patients with autoimmune diseases. Since isolated CNS involvement is rare, its clinicopathological features remain incompletely understood. Herein, we report about an 85-year-old Japanese woman who was diagnosed with SLE 26 years ago and was stable and maintained on prednisone (5 mg/day) for 20 years. Twenty-six days before admission, she developed cognitive dysfunction. Brain magnetic resonance imaging (MRI) revealed multiple nodular lesions with ring-like enhancements seen on gadolinium-enhanced T1-weighted imaging. A brain biopsy was performed on the right frontal lobe lesion. Pathological findings revealed T-cell infiltration surrounding the blood vessels with fibrin deposition, a few multinucleated cells, and large atypical cells with prominent nucleoli. Large atypical cells positive for CD20 and EBV-encoded RNA were seen at a density of >100 cells per high-power field. Based on laboratory testing, imaging, and pathology findings, the patient was diagnosed with Grade III LYG. Treatment with tirabrutinib (480 mg once daily) was started. The patient achieved clinical response to treatment, as evidenced by improved mental status. In patients with SLE who present with multiple nodular or ring-like lesions on brain MRI, a brain biopsy with histological diagnosis is crucial for the exclusion of CNS-LYG.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"68-74"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142407345","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":"Arthroscopic treatment of septic arthritis of the ankle joint caused by streptococcus in patients with chronic inflammatory diseases: Two case reports.","authors":"Gensuke Okamura, Makoto Hirao, Takaaki Noguchi, Yuki Etani, Kosuke Ebina, Taihei Miura, Hideki Tsuboi, Atsushi Goshima, Seiji Okada, Jun Hashimoto","doi":"10.1093/mrcr/rxae041","DOIUrl":"10.1093/mrcr/rxae041","url":null,"abstract":"<p><p>This report describes the arthroscopic treatment of septic arthritis of the ankle joint in two patients with inflammatory diseases, including rheumatoid arthritis and nail psoriasis. We treated both the ankle joints with antibiotic administration and urgent arthroscopic synovectomy and irrigation, although the procedure was performed several days (4 and 6 days) after the time at which the infection would have occurred. Fortunately, no recurrence has been seen for >18 and 20 months, respectively, after surgery, without antibiotic administration. Although septic arthritis of the ankle joint accounts for a small proportion of joint arthritis cases, diagnosis as early as possible is important. Our experience suggests that arthroscopic synovectomy and irrigation are effective for septic ankle arthritis even in chronic inflammatory disease cases.</p>","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":"31-34"},"PeriodicalIF":0.9,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141891403","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}