Modern rheumatology case reports最新文献

Benralizumab for eosinophilic granulomatosis with polyangiitis and subclinical cardiac involvement: a fertility-preserving case report. Benralizumab用于嗜酸性肉芽肿病合并多血管炎和亚临床心脏累及：一个保留生育能力的病例报告。

IF 0.9

Modern rheumatology case reports Pub Date : 2026-08-31 DOI: 10.1093/mrcr/rxag086

Takashi Yamane, Ayaka Inoue, Mari Nishio

{"title":"Benralizumab for eosinophilic granulomatosis with polyangiitis and subclinical cardiac involvement: a fertility-preserving case report.","authors":"Takashi Yamane, Ayaka Inoue, Mari Nishio","doi":"10.1093/mrcr/rxag086","DOIUrl":"https://doi.org/10.1093/mrcr/rxag086","url":null,"abstract":"Cardiac involvement, which may remain clinically silent, is a major determinant of prognosis in eosinophilic granulomatosis with polyangiitis (EGPA), and remission induction generally requires glucocorticoids combined with cyclophosphamide or rituximab. In contrast, treatment selection may be challenging in women wishing to preserve fertility because cyclophosphamide carries a risk of gonadal toxicity. We report a 40-year-old woman with a history of asthma, eosinophilic chronic rhinosinusitis, and eosinophilic otitis media who developed postpartum-onset EGPA and presented with neurological and cutaneous manifestations. Elevated cardiac troponin I levels and newly developed electrocardiographic abnormalities suggested subclinical myocardial injury despite the absence of cardiac symptoms. Intravenous methylprednisolone pulse therapy was initiated immediately. Because cardiac troponin I levels showed no early decline after glucocorticoid initiation, and glucocorticoid monotherapy was considered potentially insufficient to control eosinophil-mediated myocardial injury, additional eosinophil-targeted therapy was selected after shared decision-making that balanced fertility preservation with the need for prompt disease control. Benralizumab was chosen because of its potent eosinophil-depleting effect. Peripheral eosinophils rapidly decreased to zero, troponin I normalized by Day 26, and glucocorticoids were successfully discontinued within 5 months. To our knowledge, this is the first report of benralizumab used as part of a fertility-preserving remission-induction strategy to avoid cyclophosphamide in a woman with EGPA and cardiac involvement. This approach may represent an individualized therapeutic option for carefully selected patients in whom fertility preservation is an important treatment consideration.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148868514","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

Early-Onset Systemic Lupus Erythematosus with Discoid Rash and Chondritis. 早发系统性红斑狼疮伴盘状皮疹和软骨炎。

IF 0.9

Modern rheumatology case reports Pub Date : 2026-08-29 DOI: 10.1093/mrcr/rxag087

Renaissance Majee, Hannah Bozell, Barbara Ostrov

{"title":"Early-Onset Systemic Lupus Erythematosus with Discoid Rash and Chondritis.","authors":"Renaissance Majee, Hannah Bozell, Barbara Ostrov","doi":"10.1093/mrcr/rxag087","DOIUrl":"https://doi.org/10.1093/mrcr/rxag087","url":null,"abstract":"Juvenile Systemic Lupus Erythematosus (jSLE) is a rare disorder that presents with greater severity compared to adult Systemic Lupus Erythematosus. It typically manifests in adolescent females with constitutional symptoms, multisystem involvement, and a malar rash. In this case, a 9-year-old African American pre-pubertal girl presented with a progressive facial and scalp rash, weight loss, night sweats without fever, and abdominal pain following recent penicillin exposure. On laboratory results and clinical examination, leucopenia, auricular chondritis, palatal ulceration, and marked facial rash were found on physical exam and initial lab testing, alongside a negative infectious disease workup. Autoimmune workup demonstrated an elevated ANA and anti-dsDNA with low complement levels. Therefore, she met diagnostic criteria for jSLE. She improved rapidly with corticosteroids. Over the following year, she subsequently developed lupus nephritis. This case illustrates an atypical presentation of jSLE with early-onset prior to puberty and uncommon features, including chondritis, discoid lesions, and isolated mucocutaneous symptoms, with no associated joint pain or organ damage at initial presentation. Atypical jSLE presentations require high clinical suspicion to prevent delayed diagnosis and progression to complications such as lupus nephritis.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148858051","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

Planned pregnancy after remission of anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease: a case report with more than 4 years of postpartum follow-up. 抗mda5抗体阳性皮肌炎相关间质性肺病缓解后计划妊娠：产后随访4年以上1例

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Modern rheumatology case reports Pub Date : 2026-08-28 DOI: 10.1093/mrcr/rxag085

Daisuke Kobayashi, Sayuri Takamura, Shunsuke Sakai, Yoko Wada, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Kuroda, Suguru Yamamoto

{"title":"Planned pregnancy after remission of anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease: a case report with more than 4 years of postpartum follow-up.","authors":"Daisuke Kobayashi, Sayuri Takamura, Shunsuke Sakai, Yoko Wada, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Kuroda, Suguru Yamamoto","doi":"10.1093/mrcr/rxag085","DOIUrl":"https://doi.org/10.1093/mrcr/rxag085","url":null,"abstract":"Pregnancy after anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis-associated interstitial lung disease is rarely described, and preconception management remains uncertain. A 29-year-old woman presented with fever, arthralgia, cough, characteristic Gottron's and inverse Gottron's papules, normal muscle enzyme levels, mild interstitial lung abnormalities on high-resolution computed tomography, and positivity for anti-melanoma differentiation-associated gene 5 antibody. Because of the potential risk of rapid pulmonary deterioration, she received prednisolone, a calcineurin inhibitor, and intravenous cyclophosphamide, followed by maintenance immunosuppression. Mycophenolate mofetil was discontinued as part of preconception planning 24 months after disease onset. After two biochemical pregnancies that ended before ultrasonographic confirmation of a gestational sac, a subsequent pregnancy was confirmed approximately 36 months after disease onset while she was receiving prednisolone 10 mg/day and tacrolimus. Pregnancy was not complicated by relapse of dermatomyositis or interstitial lung disease, hypertensive disorder, gestational diabetes, fetal growth restriction, or non-reassuring fetal status. She delivered a healthy female infant at 38 weeks and 3 days of gestation by vaginal delivery. At more than 4 years postpartum, she remained relapse-free, and the antibody remained negative. This case suggests that planned pregnancy may be feasible in carefully selected patients after sustained preconception disease control, discontinuation of teratogenic drugs, and continuation of pregnancy-compatible immunosuppression under multidisciplinary surveillance; serial anti-MDA5 antibody measurements may provide adjunctive information when interpreted alongside clinical, pulmonary function, and imaging assessments.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148852243","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

Polymyositis with Scleroderma-like Features and an Anti-PM-Scl75 Antibody Detected by Line Blot but Not by Protein Array or Immunoprecipitation: A Case Report and Literature Review. 具有硬皮病样特征的多发性肌炎和抗pm - scl75抗体，行印迹检测而非蛋白质阵列或免疫沉淀：1例报告和文献复习。

IF 0.9

Modern rheumatology case reports Pub Date : 2026-08-26 DOI: 10.1093/mrcr/rxag083

Kota Hyakuna, Shigeru Iwata, Kodai Ueno, Taro Nishikawa, Ryo Matsumiya, Ran Nakashima, Kanako Sagan, Fumiyoshi Kojima, Kohei Hori, Jyunko Taruya, Shoko Yorozu, Takao Fujii

{"title":"Polymyositis with Scleroderma-like Features and an Anti-PM-Scl75 Antibody Detected by Line Blot but Not by Protein Array or Immunoprecipitation: A Case Report and Literature Review.","authors":"Kota Hyakuna, Shigeru Iwata, Kodai Ueno, Taro Nishikawa, Ryo Matsumiya, Ran Nakashima, Kanako Sagan, Fumiyoshi Kojima, Kohei Hori, Jyunko Taruya, Shoko Yorozu, Takao Fujii","doi":"10.1093/mrcr/rxag083","DOIUrl":"https://doi.org/10.1093/mrcr/rxag083","url":null,"abstract":"Anti-PM-Scl antibodies are associated with systemic sclerosis-myositis overlap syndrome. However, anti-PM-Scl75/100 reactivity detected by line blot assays is frequently not confirmed by protein array or immunoprecipitation, indicating that such results should be interpreted with caution in Japan. A 72-year-old woman developed limb fatigue and Raynaud's phenomenon, followed by muscle weakness, elevated serum creatine kinase levels, and gangrenous changes in several fingers. Magnetic resonance imaging and muscle biopsy findings were consistent with myositis. She tested positive for antinuclear antibodies with a speckled pattern. Line blot testing showed strong anti-PM-Scl75 reactivity, and fingertip ulcers raised suspicion of systemic sclerosis-spectrum manifestations. Treatment with prednisolone (25 mg/day) and high-dose intravenous immunoglobulin improved the muscle enzyme elevation and digital lesions. However, anti-PM-Scl75 antibodies were not detected by either protein array or immunoprecipitation. The final clinical diagnosis was polymyositis with systemic sclerosis-spectrum features. We therefore reviewed previously reported cases in which anti-PM-Scl antibodies were detected by line blot but were not confirmed by other assays. This case demonstrates that myositis with systemic sclerosis-like clinical manifestations may occur even when anti-PM-Scl75 reactivity is detected only by line blot assay. Although autoantibody testing may aid in the diagnosis of myositis, the results should be interpreted in light of the analytical characteristics of each assay. In particular, anti-PM-Scl75/100 reactivity detected by line blot should be interpreted with caution when the antinuclear antibody staining pattern is not nucleolar.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148820803","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

Adult Hypophosphatasia with a Single Heterozygous c.572A>G p.Glu191Gly Mutation in the ALPL gene: Case Report. 成人低磷酸化伴ALPL基因单杂合c.572A>G . glu191gly突变：病例报告

IF 0.9

Modern rheumatology case reports Pub Date : 2026-08-26 DOI: 10.1093/mrcr/rxag082

Kota Hyakuna, Shigeru Iwata, Taro Nishikawa, Kodai Ueno, Yuta Shimomura, Takeru Sonoda, Takashi Kato, Ryo Matsumiya, Takao Fujii

{"title":"Adult Hypophosphatasia with a Single Heterozygous c.572A>G p.Glu191Gly Mutation in the ALPL gene: Case Report.","authors":"Kota Hyakuna, Shigeru Iwata, Taro Nishikawa, Kodai Ueno, Yuta Shimomura, Takeru Sonoda, Takashi Kato, Ryo Matsumiya, Takao Fujii","doi":"10.1093/mrcr/rxag082","DOIUrl":"https://doi.org/10.1093/mrcr/rxag082","url":null,"abstract":"Adult-onset hypophosphatasia manifests after age 18, and is characterized by reduced tissue-nonspecific alkaline phosphatase (ALP) activity. This can lead to fractures, pseudo-fractures, osteomalacia, decreased bone density (bone loss), muscle weakness, myalgia, arthralgia, headache, dental symptoms (loss of permanent teeth, periodontal disease), and pseudogout. Over 480 distinct genetic mutations have been identified to date, with eight cases reported for the c.572A>G (p.Glu191Gly) mutation. However, all reported cases involved compound heterozygous mutations, and there have been no previous reports of cases of a single heterozygous mutation. We report the case of a 40-year-old woman with persistent hypophosphatasia, elevated urinary phosphoethanolamine levels suggesting adult-onset hypophosphatasia, and a heterozygous missense mutation (c.572A>G p.Glu191Gly) in the ALPL gene according to genetic testing. Treatment with subcutaneous injections of aspartate phosphatase alpha was initiated, which was followed by significant improvement of the pain, enhanced range of motion in both upper limbs, and improved gait. This patient's case differed from previously reported cases in that it involved a heterozygous c.572A>G (p.Glu191Gly) mutation alone in adult-onset hypophosphatasia. Adult-onset hypophosphatasia is an extremely rare disorder. However, its symptoms resemble those of systemic rheumatic diseases such as polymyalgia rheumatica and fibromyalgia, and rheumatologists may encounter this condition in routine clinical practice. Importantly, it can be detected based on low serum ALP levels.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148820782","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

Autoimmune Hepatitis in Two Patients with Behçet's Disease: A Case Report. 自身免疫性肝炎合并behaperet病2例报告

IF 0.9

Modern rheumatology case reports Pub Date : 2026-08-25 DOI: 10.1093/mrcr/rxag062

Majid Alikhani, Seyed Mohammad Tavangar, Mina Nikvarz, Somayeh Soroureddin

{"title":"Autoimmune Hepatitis in Two Patients with Behçet's Disease: A Case Report.","authors":"Majid Alikhani, Seyed Mohammad Tavangar, Mina Nikvarz, Somayeh Soroureddin","doi":"10.1093/mrcr/rxag062","DOIUrl":"https://doi.org/10.1093/mrcr/rxag062","url":null,"abstract":"Background: Behçet's disease (BD) is a multisystem inflammatory disorder with variable manifestations. Although hepatic involvement is rare, abnormal liver enzymes may occasionally be observed. We describe two patients with BD who developed autoimmune hepatitis (AIH), confirmed by liver biopsy.Case presentation: We report two Iranian patients-one male and one female-diagnosed with BD, who later developed persistently elevated liver enzymes. Both had elevated IgG levels, negative viral hepatitis markers, and liver biopsy findings compatible with autoimmune hepatitis (AIH). Due to limited retrospective serologic data, the diagnosis was established based on multidisciplinary clinicopathological assessment and response to immunosuppressive therapy. Sonography showed fatty liver in both cases. Treatment with azathioprine and prednisolone led to normalization of liver enzymes over a long-term follow-up.Conclusions: Although uncommon, AIH can co-occur with BD and should be considered in patients with persistently elevated transaminases. Liver biopsy is crucial for definitive diagnosis. Early immunosuppressive therapy can prevent disease progression.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148820756","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

Chronic Nonbacterial Osteomyelitis/Chronic Recurrent Multifocal Osteomyelitis - A Case Report and Literature Review. 慢性非细菌性骨髓炎/慢性复发性多灶性骨髓炎1例报告并文献复习。

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Modern rheumatology case reports Pub Date : 2026-08-20 DOI: 10.1093/mrcr/rxag081

Christophe Persad, Niamh Donaldson, Simona Huica

引用次数: 0

Persistent parvovirus B19 infection in an older patient with rheumatoid arthritis receiving immunosuppressive therapy: A case report and literature review. 接受免疫抑制治疗的老年类风湿关节炎患者持续性细小病毒B19感染：1例报告并文献复习

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Modern rheumatology case reports Pub Date : 2026-07-17 DOI: 10.1093/mrcr/rxag057

Masako Oyama, Yusuke Yoshida, Tetsumi Yoshida, Hiroki Kobayashi, Yuji Shigemasa, Takahisa Usagawa, Makoto Fujii, Michinori Ishitoku, Tomohiro Sugimoto, Shintaro Hirata

{"title":"Persistent parvovirus B19 infection in an older patient with rheumatoid arthritis receiving immunosuppressive therapy: A case report and literature review.","authors":"Masako Oyama, Yusuke Yoshida, Tetsumi Yoshida, Hiroki Kobayashi, Yuji Shigemasa, Takahisa Usagawa, Makoto Fujii, Michinori Ishitoku, Tomohiro Sugimoto, Shintaro Hirata","doi":"10.1093/mrcr/rxag057","DOIUrl":"https://doi.org/10.1093/mrcr/rxag057","url":null,"abstract":"An 84-year-old man with rheumatoid arthritis undergoing treatment with methotrexate presented with fever, general fatigue, and appetite loss. He had a 3-month history of unexplained normocytic anaemia with reticulocytopenia. Bone marrow examination revealed erythroid hypoplasia with giant pro-erythroblasts, leading to a diagnosis of parvovirus B19 infection. In this case, intravenous immunoglobulin therapy improved the patient's anaemia and systemic symptoms. Six months after the treatment, parvovirus B19 DNA remained detectable using polymerase chain reaction; however, the viral load had markedly decreased, indicating a favourable virological response. Twenty cases of parvovirus B19 infection in older adults (aged ≥65 years) were identified in the literature. Immunocompromised older patients frequently develop persistent parvovirus B19 infections that require treatment with intravenous immunoglobulin therapy, whereas persistent infections are not observed in immunocompetent older individuals. Therefore, this case suggests that clinicians should be aware of persistent parvovirus B19 infection in older immunosuppressed patients who develop unexplained anaemia with reticulocytopenia. Moreover, intravenous immunoglobulin therapy may be considered as an effective therapeutic option for persistent parvovirus B19 infection in immunocompromised patients.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148474980","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-PM/Scl antibody-positive myositis with muscle pathology resembling anti-synthetase syndrome. 抗pm /Scl抗体阳性肌炎，肌肉病理类似抗合成酶综合征。

IF 0.9

Modern rheumatology case reports Pub Date : 2026-07-12 DOI: 10.1093/mrcr/rxag065

Kosuke Naito, Mayu Yagita-Sakamaki, Atsuko Nishikawa, Toshio Tanaka

{"title":"Anti-PM/Scl antibody-positive myositis with muscle pathology resembling anti-synthetase syndrome.","authors":"Kosuke Naito, Mayu Yagita-Sakamaki, Atsuko Nishikawa, Toshio Tanaka","doi":"10.1093/mrcr/rxag065","DOIUrl":"https://doi.org/10.1093/mrcr/rxag065","url":null,"abstract":"Anti-PM/Scl antibodies were originally thought to be autoantibodies associated with overlap syndromes involving idiopathic inflammatory myopathies (IIM) and systemic sclerosis (SSc), but some patients with these antibodies present with clinical features similar to those of anti-synthetase syndrome (ASyS). Herein, we report the case of a 63-year-old Japanese woman with anti-PM/Scl antibody-positive, but anti- aminoacyl tRNA synthetases (anti-ARS) antibody-negative myositis with interstitial lung disease (ILD) and dermatomyositis-like skin manifestations. Her muscle pathological findings resembled those observed in ASyS, including perifascicular fibre necrosis and increased alkaline phosphatase (ALP) activity in the perimysium. Owing to the potential risk of SSc overlap and scleroderma renal crisis (SRC), calcineurin inhibitor (CNI) treatment was avoided, and mycophenolate mofetil (MMF) was administered in addition to glucocorticoids. This approach enabled rapid tapering of glucocorticoids, resulting in a favourable clinical course. To our knowledge, this report provides novel insights into the muscle pathology of anti-PM/Scl antibody-positive myositis, highlighting pathological similarities with ASyS. Our findings also suggest that MMF may be a reasonable immunosuppressive therapy, especially when there is a risk of SRC.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148427883","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

Infliximab for laryngopharyngeal involvement in Behçet's syndrome. 英夫利昔单抗治疗behet综合征的咽喉受累。

IF 0.9

Modern rheumatology case reports Pub Date : 2026-07-10 DOI: 10.1093/mrcr/rxag068

Miyu Wakatsuki, Hiroyuki Yamashita, Hiroshi Kaneko

{"title":"Infliximab for laryngopharyngeal involvement in Behçet's syndrome.","authors":"Miyu Wakatsuki, Hiroyuki Yamashita, Hiroshi Kaneko","doi":"10.1093/mrcr/rxag068","DOIUrl":"https://doi.org/10.1093/mrcr/rxag068","url":null,"abstract":"Laryngopharyngeal involvement in Behçet's syndrome is rare but clinically significant because it may cause severe odynophagia, dysphagia, and airway compromise. Optimal treatment has not been established, and irreversible structural sequelae, including laryngopharyngeal stenosis and ulcer scarring, may occur. We describe two men with Behçet's syndrome and symptomatic laryngopharyngeal involvement who showed rapid improvement after infliximab administration following an insufficient response to corticosteroids. In case 1, a 25-year-old man was classified as having suspected Behçet's syndrome based on recurrent oral ulcers, pharyngeal ulceration, arthritis, folliculitis-like skin lesions, and previous episodes of abdominal pain. He developed severe pharyngeal pain and dysphagia due to ulcerative lesions extending from the posterior pharyngeal wall to the esophageal inlet. Intravenous prednisolone resulted in limited endoscopic and symptomatic improvement, whereas infliximab promptly relieved pain and restored oral intake, with no recurrence over 14 years of follow-up. In case 2, a 29-year-old man presented with recurrent fever, oral aphthae, genital ulcers, arthritis, erythema nodosum-like lesions, and pharyngeal and laryngeal mucosal lesions associated with dysphagia. His symptoms and laryngoscopic abnormalities improved promptly after infliximab administration following an inadequate response to prednisolone. These cases underscore the importance of considering early anti-tumor necrosis factor-α therapy in steroid-refractory or relapsing laryngopharyngeal Behçet's syndrome, because progression to irreversible structural complications, including stenosis and adhesion, may necessitate surgical intervention if inflammation is not controlled before permanent damage occurs.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148427681","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