Modern rheumatology case reports最新文献

Parallel course of calcinosis and cancer in a patient with anti-NXP2-positive dermatomyositis: A case report. 抗nxp2阳性皮肌炎患者钙质沉着症和癌症的平行病程：1例报告。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag016

Christopher A Mecoli, Akila N Viswanathan, Antony Rosen, Livia Casciola-Rosen

{"title":"Parallel course of calcinosis and cancer in a patient with anti-NXP2-positive dermatomyositis: A case report.","authors":"Christopher A Mecoli, Akila N Viswanathan, Antony Rosen, Livia Casciola-Rosen","doi":"10.1093/mrcr/rxag016","DOIUrl":"10.1093/mrcr/rxag016","url":null,"abstract":"Background: In patients with dermatomyositis (DM) and anti-nuclear matrix protein 2 autoantibodies, associations with both cancer and calcinosis are well established. However, the relationship between the progression of calcinosis and the development of cancer remains unknown. Herein, we describe a unique case that prompts hypotheses regarding shared immunologic mechanisms underlying these clinical complications.Case presentation: We present the case of a 46-year-old woman diagnosed with biopsy-proven anti-nuclear matrix protein 2-positive DM manifesting as proximal muscle weakness, myalgias, pruritic rash, and elevations in creatine phosphokinase. Five years into her disease course, the patient developed an abrupt, coincident severe calcinosis and stage IIB squamous cell carcinoma of the cervix. She required aggressive immunosuppression, immunomodulation, and cancer therapy, which ultimately resulted in the remission of her cancer and improvement of her DM, with her calcinosis achieving a quiescent state. Her case describes a parallel course of calcinosis and cancer, and it highlights the challenges of managing refractory DM with malignancy.Conclusions: Our case demonstrates that calcinosis and cancer can develop concurrently and follow a parallel course over time, suggesting a possible mechanistic link between the two complications. This report also highlights that communication between rheumatology, medical oncology, and radiation oncology is pivotal for optimally treating these complex patients.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13002130/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147367803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A case of AL amyloidosis-associated arthritis initially misdiagnosed as rheumatoid arthritis. AL淀粉样变性相关关节炎最初误诊为类风湿性关节炎1例。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag020

Yukina Tanimoto, Hirotaka Yamada, Shintaro Yasui, Michio Mugitani, Keiji Kurata, Keisuke Nishimura, Jun Saegusa

{"title":"A case of AL amyloidosis-associated arthritis initially misdiagnosed as rheumatoid arthritis.","authors":"Yukina Tanimoto, Hirotaka Yamada, Shintaro Yasui, Michio Mugitani, Keiji Kurata, Keisuke Nishimura, Jun Saegusa","doi":"10.1093/mrcr/rxag020","DOIUrl":"10.1093/mrcr/rxag020","url":null,"abstract":"Excessive production of immunoglobulin light chains can result in amyloid light-chain (AL) amyloidosis, which manifests as arthritis in ~3.7% of cases. Myeloma-associated amyloid arthropathy, a musculoskeletal manifestation of AL amyloidosis, often mimics rheumatoid arthritis, making an accurate diagnosis challenging. Musculoskeletal ultrasonography has emerged as a valuable tool in the differential diagnosis of arthralgia; however, the specific sonographic features of AL amyloidosis-related arthritis remain poorly characterised. We present a case of AL amyloidosis-associated arthritis initially misdiagnosed and treated as seronegative rheumatoid arthritis. A comprehensive diagnostic workup - including musculoskeletal ultrasonography, magnetic resonance imaging, ultrasound-guided biopsy of the left shoulder joint, and systemic evaluations such as serum and urine protein electrophoresis and bone marrow examination - led to a definitive diagnosis of AL amyloidosis secondary to multiple myeloma. This case highlights the need to consider AL amyloidosis in the differential diagnosis of atypical arthritis, particularly in seronegative patients with a poor response to rheumatoid arthritis therapies, the involvement of nonclassical joints, or systemic manifestations such as proteinuria or peripheral neuropathy. Although not diagnostic on its own, musculoskeletal ultrasonography may reveal atypical synovial changes suggestive of amyloid deposition and assist in guiding targeted biopsies, which remain essential for a definitive diagnosis. Notably, this case demonstrated positive power Doppler signals, in contrast to previous reports, suggesting a broader spectrum of ultrasonographic findings in amyloid arthropathy than previously recognised.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147367780","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}

引用次数: 0

Anti-SRP antibody-positive polymyositis complicated by neuromyelitis optica spectrum disorder: a case report and literature review. 抗srp抗体阳性多发性肌炎合并视神经脊髓炎1例并文献复习。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxaf082

Eisuke Tanaka, Mai Kawazoe, Shotaro Masuoka, Yudai Aikawa, Takashi Tanaka, Keiko Koshiba, Zento Yamada, Risa Wakiya, Eri Watanabe, Sei Muraoka, Takahiko Sugihara, Toshihiro Nanki

{"title":"Anti-SRP antibody-positive polymyositis complicated by neuromyelitis optica spectrum disorder: a case report and literature review.","authors":"Eisuke Tanaka, Mai Kawazoe, Shotaro Masuoka, Yudai Aikawa, Takashi Tanaka, Keiko Koshiba, Zento Yamada, Risa Wakiya, Eri Watanabe, Sei Muraoka, Takahiko Sugihara, Toshihiro Nanki","doi":"10.1093/mrcr/rxaf082","DOIUrl":"10.1093/mrcr/rxaf082","url":null,"abstract":"Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory demyelinating disease primarily affecting the optic nerves and spinal cord. Polymyositis (PM) is an idiopathic inflammatory myopathy characterized by proximal muscle weakness. The anti-signal recognition particle (SRP) antibody is a myositis-specific autoantibody. We herein present a case of anti-SRP antibody-positive PM that developed 6 years after the onset of NMOSD. A 52-year-old woman was diagnosed with NMOSD 6 years previously based on left visual disturbance, a high signal intensity and contrast enhancement of the optic nerve on short τ inversion recovery (STIR)-magnetic resonance imaging (MRI), and anti-aquaporin 4 antibody positivity. She received methylprednisolone pulse therapy followed by oral prednisolone (PSL) at a starting dose of 40 mg daily. Three years later, due to recurrent numbness in the left lower limb and difficulty in reducing the PSL dose to ≤10 mg/day, satralizumab was initiated. At 52 years old, she developed myalgia and muscle weakness in both thighs. PM was diagnosed based on an elevated serum creatine kinase level, anti-SRP antibody positivity, high signal intensities in the right triceps and bilateral adductor muscles on STIR-MRI, and muscle biopsy findings. Treatment with high-dose PSL and tacrolimus markedly attenuated her symptoms. Satralizumab was continued for NMOSD stabilisation. Previous studies reported the coexistence of NMOSD and autoimmune diseases; however, NMOSD with PM/DM is rare. We described a case of NMOSD with anti-SRP antibody-positive PM and provided a literature review.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145758944","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}

引用次数: 0

Critical limb Ischaemia in granulomatosis with polyangiitis: a rare but severe complication. 肉芽肿病伴多血管炎的严重肢体缺血：罕见但严重的并发症。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxaf084

Anushka Aggarwal, Rohini Handa, Rakesh Mahajan, Shradha Engels

{"title":"Critical limb Ischaemia in granulomatosis with polyangiitis: a rare but severe complication.","authors":"Anushka Aggarwal, Rohini Handa, Rakesh Mahajan, Shradha Engels","doi":"10.1093/mrcr/rxaf084","DOIUrl":"10.1093/mrcr/rxaf084","url":null,"abstract":"Granulomatosis with polyangiitis (GPA) is a small-to-medium vessel vasculitis usually presenting with upper airway, pulmonary, and renal involvement. Critical limb ischaemia (CLI) and arterial thrombosis are rare but severe complications of GPA, often resulting in poor prognosis and limb loss. We describe a 34-year-old woman presenting with right upper limb CLI on a background of GPA, manifesting as fever, purpura with upper airway, pulmonary, and renal involvement. Investigations confirmed proteinase-3 anti-neutrophil cytoplasmic antibody positivity, elevated inflammatory markers, proteinuria with active urinary sediment, and imaging revealed pulmonary infiltrates with sinus involvement. She received IV steroids and rituximab but developed acute limb-threatening ischaemia due to brachial artery thrombosis. Immediate thrombectomy with thrombolysis restored blood flow and prevented amputation. Available literature highlights the extreme rarity of CLI in GPA (<1%), with most reported cases resulting in limb loss despite immunosuppression. Prompt diagnosis using Doppler/angiogram and urgent surgical intervention, in conjunction with immunosuppression, is critical for limb salvage. This case underscores the importance of early recognition and combined surgical-medical management in GPA presenting with arterial thrombosis and CLI, which can successfully preserve limb function and improve outcomes.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145795759","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}

引用次数: 0

Bilateral orbital masses caused by IgG4-expressing MALT lymphoma in the right side and IgG4-related ophthalmic disease in the left side: A case report. 右侧表达igg4的MALT淋巴瘤及左侧igg4相关眼病所致双侧眼眶肿块1例

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag006

Yasuto Araki, Yuko Mishima, Taketo Yamada, Yuji Akiyama, Toshihide Mimura

{"title":"Bilateral orbital masses caused by IgG4-expressing MALT lymphoma in the right side and IgG4-related ophthalmic disease in the left side: A case report.","authors":"Yasuto Araki, Yuko Mishima, Taketo Yamada, Yuji Akiyama, Toshihide Mimura","doi":"10.1093/mrcr/rxag006","DOIUrl":"10.1093/mrcr/rxag006","url":null,"abstract":"A 60-year-old Japanese man developed a protruding right eye. He underwent a magnetic resonance imaging scan, which revealed a right orbital mass. The serum immunoglobulin G4 (IgG4) level was elevated, and IgG4+ plasma cells were observed in biopsy specimens of the mass. The result of biopsy and Southern blot analysis revealed that the mass was caused by IgG4+ extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. He received a total of 36 Gy of radiation therapy, and the mass disappeared. Five years later, he developed a protruding left eye. The magnetic resonance imaging scan at that time revealed a left orbital mass. Biopsy revealed findings of IgG4-related disease in the left orbital mass, but no findings of mucosa-associated lymphoid tissue lymphoma. He has been followed up without glucocorticoid treatment. Here, we report a patient who developed IgG4+ MALT lymphoma in the right orbital mass and IgG4-related ophthalmic disease in the left orbital mass. Because the treatment strategy for IgG4-related ophthalmic disease and malignant lymphoma is completely different, we emphasise the need for biopsy.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146088691","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}

引用次数: 0

Therapeutic evolution and outcomes in EGPA complicated by diffuse alveolar haemorrhage: Case-based review. EGPA合并弥漫性肺泡出血的治疗进展和结果：基于病例的回顾。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag028

Yang Liu, Xing Zhao, Jinfang Gao, Ke Xu

{"title":"Therapeutic evolution and outcomes in EGPA complicated by diffuse alveolar haemorrhage: Case-based review.","authors":"Yang Liu, Xing Zhao, Jinfang Gao, Ke Xu","doi":"10.1093/mrcr/rxag028","DOIUrl":"10.1093/mrcr/rxag028","url":null,"abstract":"Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic vasculitis characterised by asthma, eosinophilia, and multi-organ involvement. Diffuse alveolar haemorrhage (DAH) is an uncommon, but life-threatening pulmonary complication in EGPA. We report on a 49-year-old, previously healthy woman who presented initially with asthma-like symptoms and later developed a fever, haemoptysis, cutaneous purpura, and periorbital oedema. Laboratory evaluation revealed marked eosinophilia, anaemia, elevated inflammatory markers, and a strongly positive MPO-ANCA. Bronchoalveolar lavage fluid was haemorrhagic and contained hemosiderin-laden macrophages, indicating DAH. Broad-spectrum antibiotics were empirically initiated but discontinued after metagenomic next-generation sequencing (mNGS) of the bronchoalveolar lavage fluid excluded infection. A bone marrow biopsy showed eosinophilic hyperplasia without clonal mutations. A diagnosis of MPO-ANCA positive EGPA with DAH was established. The patient received pulse methylprednisolone, prednisone, intravenous immunoglobulin, mepolizumab, and rituximab. Clinical symptoms improved rapidly, and radiological signs of alveolar haemorrhage had nearly resolved within days. Our case illustrates that the integration of rituximab and mepolizumab with corticosteroids can achieve rapid remission and steroid sparing in EGPA-DAH. While evidence remains limited to case reports and small series, targeted biologics may fundamentally improve outcomes in this high-risk subset. Prospective studies are warranted to define optimal treatment strategies.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147517929","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}

引用次数: 0

Small vessel vasculitis diagnosed with conchotome muscle biopsy: A case series. 经肌活检诊断的小血管炎：一个病例系列。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag029

Natsuka Umezawa, Hirokazu Sasaki, Shimpei Oka, Tomoko Niwano, Shinsuke Yasuda

{"title":"Small vessel vasculitis diagnosed with conchotome muscle biopsy: A case series.","authors":"Natsuka Umezawa, Hirokazu Sasaki, Shimpei Oka, Tomoko Niwano, Shinsuke Yasuda","doi":"10.1093/mrcr/rxag029","DOIUrl":"10.1093/mrcr/rxag029","url":null,"abstract":"A percutaneous conchotome muscle biopsy, which is a semi-open technique, provides significant diagnostic yield, especially for inflammatory myopathies. In patients with small vessel vasculitis, muscle involvement could sometimes be the sole or most prominent organ involvement. While open muscle biopsy has been reported to be useful for the diagnosis of vasculitis, the diagnostic utility with conchotome muscle biopsy for vasculitis remains unclear. Here, we report four cases in which a conchotome muscle biopsy made a significant contribution to their diagnosis of small vessel vasculitis. All cases presented with myalgia and weakness at the onset of their diseases, and with an elevation of myeloperoxidase antineutrophil cytoplasmic antibodies. Pathological examination of muscle tissues revealed significant inflammatory infiltration with fibrinoid necrosis in perimysial arterioles, measuring 50-100 mm in diameter, in all of four cases. Disruption of the elastic plates of the arterioles was found with Elastica van Gieson staining in three cases. Low levels of patients' pain, evaluated with a visual analogue scale (0-100), ranging from 0 to 30, and no occurrence of adverse events in our cases indicated the safety of this technique. All patients were treated successfully with glucocorticoids, with or without immunosuppressants. The conchotome muscle biopsy could yield the pathological features of small vessel vasculitis with minimal invasiveness.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147517922","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}

引用次数: 0

Expanding the role of avacopan beyond renal involvement: Clinical insights from two cases. 扩大阿伐柯潘的作用，使其不再受累于肾脏：两个病例的临床观察。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag022

Andreia Rita Henriques, Nuno Oliveira, Pedro Castro, Helena Pinto, Emanuel Ferreira, Helena Sá

{"title":"Expanding the role of avacopan beyond renal involvement: Clinical insights from two cases.","authors":"Andreia Rita Henriques, Nuno Oliveira, Pedro Castro, Helena Pinto, Emanuel Ferreira, Helena Sá","doi":"10.1093/mrcr/rxag022","DOIUrl":"10.1093/mrcr/rxag022","url":null,"abstract":"Granulomatosis with polyangiitis is a necrotising small vessel vasculitis characterised by granulomatous inflammation that can involve the eyes, ear-nose-throat region, and lungs, often refractory to standard immunosuppressive therapies such as glucocorticoids, rituximab, and cyclophosphamide. The complex immunopathogenesis of granulomatous lesions, involving neutrophil swarming, macrophage activation, T-cell responses, and complement activation through the C5a receptor, contributes to treatment challenges. Avacopan, an oral C5a receptor antagonist, has demonstrated efficacy in antineutrophil cytoplasmic antibody-associated vasculitis, particularly in kidney involvement, with evidence of glucocorticoid-sparing benefits from the ADVOCATE trial. However, data on its role in granulomatous manifestations remain limited. We report two cases of severe, refractory granulomatosis with polyangiitis with ocular, ear-nose-throat, and pulmonary granulomatous disease unresponsive to standard therapies but achieving complete clinical and immunological remission with avacopan. Both patients successfully discontinued glucocorticoids and showed marked clinical improvement and radiological resolution of lesions. These cases, supported by recent trial sub-analyses, suggest that avacopan may be an effective therapeutic option for granulomatous granulomatosis with polyangiitis manifestations, enabling glucocorticoid reduction and improved disease control.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147380253","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}

引用次数: 0

Anti-signal recognition particle antibody-positive immune-mediated necrotising myopathy with inclusion body myositis-like features in a patient with human immunodeficiency virus and syphilis infection. 抗信号识别颗粒抗体阳性免疫介导坏死性肌病伴包涵体肌炎样特征的一例人类免疫缺陷病毒和梅毒感染患者

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag025

Natsumi Nagao, Yu Funakubo Asanuma, Fumito Inayoshi, Keita Okamoto, Nobuhito Okumura, Yukiko Iwasaki, Toshihide Mimura, Ichizo Nishino, Yuji Akiyama

{"title":"Anti-signal recognition particle antibody-positive immune-mediated necrotising myopathy with inclusion body myositis-like features in a patient with human immunodeficiency virus and syphilis infection.","authors":"Natsumi Nagao, Yu Funakubo Asanuma, Fumito Inayoshi, Keita Okamoto, Nobuhito Okumura, Yukiko Iwasaki, Toshihide Mimura, Ichizo Nishino, Yuji Akiyama","doi":"10.1093/mrcr/rxag025","DOIUrl":"10.1093/mrcr/rxag025","url":null,"abstract":"Inclusion body myositis (IBM) and immune-mediated necrotising myopathy (IMNM) are inflammatory myopathies characterized by specific clinical and pathological features. IBM is associated with human immunodeficiency virus (HIV) infection, whereas IMNM is extremely rare in individuals positive for HIV. To date, no reports have described the coexistence of IMNM and IBM-like pathological features in patients positive for HIV. Herein, we report a rare case of a 40-year-old man with HIV-associated subacute progressive myopathy presenting with simultaneous proximal and distal muscle weakness, dysphagia, and markedly elevated creatine kinase levels. Serological testing revealed strong positivity for anti-signal recognition particle antibodies. Muscle biopsy revealed coexisting pathological features suggesting both IMNM and IBM. The patient showed insufficient response to antiretroviral therapy and glucocorticoids; however, intravenous immunoglobulin therapy resulted in a marked clinical response. This case highlights the possible coexistence of IMNM and IBM-like pathology in the context of HIV infection and provides insights into the pathophysiology of HIV-associated myopathy. Furthermore, this report suggests the potential effectiveness of intravenous immunoglobulin treatment in such cases.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147505777","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}

引用次数: 0

Possible involvement of eosinophil Etosis in hepatic injury associated with hypereosinophilic syndrome. 嗜酸性粒细胞代谢可能参与嗜酸性粒细胞增多综合征相关肝损伤。

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Modern rheumatology case reports Pub Date : 2026-01-06 DOI: 10.1093/mrcr/rxag015

Yuka Yoshiki, Haruka Miki, Keinosuke Hizuka, Toshiki Sugita, Mizuki Yagishita, Taihei Nishiyama, Masaru Shimizu, Ayako Ohyama, Saori Abe, Ayako Kitada, Hiromitsu Asashima, Yuya Kondo, Hiroto Tsuboi, Shigeharu Ueki, Isao Matsumoto

{"title":"Possible involvement of eosinophil Etosis in hepatic injury associated with hypereosinophilic syndrome.","authors":"Yuka Yoshiki, Haruka Miki, Keinosuke Hizuka, Toshiki Sugita, Mizuki Yagishita, Taihei Nishiyama, Masaru Shimizu, Ayako Ohyama, Saori Abe, Ayako Kitada, Hiromitsu Asashima, Yuya Kondo, Hiroto Tsuboi, Shigeharu Ueki, Isao Matsumoto","doi":"10.1093/mrcr/rxag015","DOIUrl":"10.1093/mrcr/rxag015","url":null,"abstract":"Hypereosinophilic syndrome (HES) is a rare disorder characterised by persistent eosinophilia and multi-organ damage. While hepatic involvement is an uncommon complication, the underlying pathological mechanism driving tissue injury remains poorly understood, as conventional evidence of eosinophil degranulation is often difficult to ascertain in collagen-rich organs like the liver. We report a case of HES-associated liver injury that implicates eosinophil ETosis (EETosis)-an active cytolytic cell death-in the pathogenic process. A patient presented with marked eosinophilia and elevated liver enzymes. Liver biopsy revealed dense eosinophilic infiltration. Critically, double immunostaining for galectin-10 and major basic protein yielded the direct pathological evidence of EETosis within the liver tissue of an HES patient. Furthermore, the patient's peripheral blood eosinophils exhibited significant spontaneous ETosis in vitro, suggesting an inherent predisposition to tissue damage. This case demonstrates that eosinophil ETosis is a key pathogenic mechanism in HES-related hepatic injury. Our findings underscore the utility of combined galectin-10 and major basic protein staining for the pathological diagnosis of eosinophil-mediated damage and suggest that spontaneous ETosis may serve as a novel biomarker for identifying HES patients at high risk of organ involvement, providing a rationale for targeting eosinophil ETosis in therapeutic strategies.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147483068","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}

引用次数: 0