Modern rheumatology case reports最新文献_第4页

A Case of Vesiculobullous Dermatomyositis with Anti-NXP-2 Antibody without Malignancy. 一例无恶性肿瘤的抗 NXP-2 抗体膀胱脓肿性皮肌炎病例

IF 0.9

Modern rheumatology case reports Pub Date : 2024-08-01 DOI: 10.1093/mrcr/rxae037

Ryota Naito, Ryosuke Hiwa, Ryuta Inaba, Kosaku Murakami, Akihiko Kitoh, Yo Kaku, Teruasa Murata, Yuki Ichimura, Naoko Okiyama, Ichizo Nishino, Mirei Shirakashi, Hideo Onizawa, Hideaki Tsuji, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Akira Onishi, Masao Tanaka, Hajime Yoshifuji, Akio Morinobu

{"title":"A Case of Vesiculobullous Dermatomyositis with Anti-NXP-2 Antibody without Malignancy.","authors":"Ryota Naito, Ryosuke Hiwa, Ryuta Inaba, Kosaku Murakami, Akihiko Kitoh, Yo Kaku, Teruasa Murata, Yuki Ichimura, Naoko Okiyama, Ichizo Nishino, Mirei Shirakashi, Hideo Onizawa, Hideaki Tsuji, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Akira Onishi, Masao Tanaka, Hajime Yoshifuji, Akio Morinobu","doi":"10.1093/mrcr/rxae037","DOIUrl":"https://doi.org/10.1093/mrcr/rxae037","url":null,"abstract":"Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for anti-nuclear matrix protein 2 (NXP-2) antibody, one of the DM-specific autoantibodies. Serological and histopathological findings did not support autoimmune blistering disease. Physical and histological findings suggested that the severe edema in combination with the interface dermatitis of DM contributed to blister formation. Although a systemic examination was performed, no evidence of malignancy was found. Following initiation of immunosuppressive therapy, the patient showed significant improvement in both skin lesions and myositis. This case represents the first report of anti-NXP-2-positive VD without malignancy or autoimmune blistering disease. Subcutaneous edema, a characteristic feature of anti-NXP-2-positive DM, could be related to the formation of VD.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141861955","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

Coexistence of anti-KS and anti-TIF1-γ antibodies in clinically amyopathic dermatomyositis presenting with rapid progression of interstitial lung disease. 抗-KS和抗-TIF1-γ抗体同时存在于间质性肺病快速进展的临床淀粉样变性皮肌炎中。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-08-01 DOI: 10.1093/mrcr/rxae033

Yuichiro Ota, Toshiki Ohisa, Akira Ishii, Mai Sugiyama, Yasushi Kondo, Ayumi Nishikawa, Noriko Sasaki, Chiho Yamada, Shinji Sato

{"title":"Coexistence of anti-KS and anti-TIF1-γ antibodies in clinically amyopathic dermatomyositis presenting with rapid progression of interstitial lung disease.","authors":"Yuichiro Ota, Toshiki Ohisa, Akira Ishii, Mai Sugiyama, Yasushi Kondo, Ayumi Nishikawa, Noriko Sasaki, Chiho Yamada, Shinji Sato","doi":"10.1093/mrcr/rxae033","DOIUrl":"https://doi.org/10.1093/mrcr/rxae033","url":null,"abstract":"Polymyositis/Dermatomyositis (PM/DM) is an idiopathic inflammatory myopathy (IIM) manifesting mainly as symmetrical proximal muscle weakness and/or typical cutaneous features due to autoimmune mechanisms. Clinically amyopathic dermatomyositis (CADM) is a subset of DM that exhibits only the typical cutaneous features without any clinical muscle symptoms. Several autoantibodies have been found specifically in patients with PM/DM, including CADM patients. Anti-KS antibody is one of a group of anti-aminoacyl transfer RNA (ARS) antibodies that are mainly associated with fever, Raynaud's phenomenon, polyarthritis, and interstitial lung disease (ILD), whereas anti-TIF1-γ antibody is frequently found in DM patients with malignancy. Here, we report a CADM patient having both anti-KS antibody and anti-TIF1-γ antibody. This patient developed an acute exacerbation of ILD and was successfully treated with high dose corticosteroid pulse therapy together with immunosuppressive agents. Although earlier experience had indicated that the seminal characteristic of anti-KS-positive ILD was slowly developing disease onset with little or no progression over the clinical course, the present patient suffered rapidly progressive disease.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141861956","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

Löfgren syndrome, characteristics of Japanese cases: a case and a review of the literature. 洛夫格伦综合征，日本病例的特征：一个病例和文献综述。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-31 DOI: 10.1093/mrcr/rxae034

Hirokazu Taguchi, Shuji Sumitomo, Hideki Oka, Shigeo Hara, Koichiro Ohmura

{"title":"Löfgren syndrome, characteristics of Japanese cases: a case and a review of the literature.","authors":"Hirokazu Taguchi, Shuji Sumitomo, Hideki Oka, Shigeo Hara, Koichiro Ohmura","doi":"10.1093/mrcr/rxae034","DOIUrl":"https://doi.org/10.1093/mrcr/rxae034","url":null,"abstract":"Löfgren syndrome (LS) is a sarcoidosis subtype characterized by an acute disease course, bilateral hilar lymphadenopathy, erythema nodosum, and ankle arthritis. LS in Caucasians appears to be self-limiting; however, our patients required glucocorticoid (GC) treatment. Here, we present a case of LS and review the literature to identify the characteristics of the Japanese patients with LS. Sixty-six-year-old woman was referred to our hospital; she initially presented with an acute onset of low-grade fever and ankle arthritis, followed by erythema nodosum. Skin biopsy revealed a non-caseating granuloma, and chest computed tomography scan displayed bilateral hilar lymphadenopathy; she was diagnosed with LS. Her arthralgia ameliorated spontaneously, but erythema persisted, necessitating GC treatment. Literature review revealed that the Japanese LS patients showed more fever, were more frequently treated with GC and more patients seemed to relapse, which may be explained by the absence of human leukocyte antigen-DR isotype 3, a good prognostic allele in Caucasians. Japanese LS may cause severe symptoms after development because of the differences in human leukocyte antigen from foreign countries. For early diagnosis, it is important to evaluate erythema nodosum and bilateral hilar lymphadenopathy in patients with polyarthritis involving ankle arthralgia.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141857475","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

Pediatric SAPHO syndrome with pleural effusion: case report of a unique finding in a rare disease. 伴有胸腔积液的小儿 SAPHO 综合征：罕见疾病中一个独特发现的病例报告。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-31 DOI: 10.1093/mrcr/rxae038

Christopher Kruger, Christine Wang, Andrew Grim

{"title":"Pediatric SAPHO syndrome with pleural effusion: case report of a unique finding in a rare disease.","authors":"Christopher Kruger, Christine Wang, Andrew Grim","doi":"10.1093/mrcr/rxae038","DOIUrl":"https://doi.org/10.1093/mrcr/rxae038","url":null,"abstract":"Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disease characterized by bone inflammation and skin manifestations including acne, palmoplantar pustulosis, psoriasis, or hidradenitis suppurativa. SAPHO syndrome is considered on the same spectrum as chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis (CNO/CRMO), the former often being the nomenclature in adults and the latter in children. The diagnosis is made on patterns of clinical manifestations and is a diagnosis of exclusion. While skin and bone manifestations are commonly described with SAPHO syndrome, pleural involvement is rare, and few cases have been described in the literature, especially in pediatric patients. Herein we present a 14-year-old female with a past medical history of hidradenitis supprtiva, eczema, psoriasis, and a prior episode of culture-negative osteomyelitis who presented to the emergency room with chief complaints of right sided pain with inspiration and back pain. Exam revealed palmoplantar pustulosis, hidradenitis supprativa, psoriasis, and tenderness of vertebrae. Imaging showed a right sided pleural effusion and multiple sites of osteitis. Laboratory evaluation revealed elevated inflammatory markers, an exudative pleural effusion with neutrophilic predominance, and no evidence of malignancy, infection, or immunodeficiency. The patient was diagnosed with SAPHO syndrome and treated with naproxen, methotrexate, and golimumab with significant improvement including resolution of the pleural effusion. Pediatric SAPHO syndrome is a rare disease that classically causes osteitis and skin manifestations. This case highlights that pleural effusion can be a rare manifestation of pediatric SAPHO syndrome. Patients with suspected SAPHO syndrome with respiratory symptoms should be evaluated for pleural effusion.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141857476","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

A case of Buerger's disease with vasculopathy and skin fibrosis requiring differential diagnosis from systemic sclerosis. 一例布格氏病伴有血管病变和皮肤纤维化，需要与系统性硬化症进行鉴别诊断。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-31 DOI: 10.1093/mrcr/rxae036

Masashi Funada, Shingo Nakayamada, Shunsuke Fukuyo, Satoshi Kubo, Aya Nawata, Yuya Fujita, Yoshiya Tanaka

{"title":"A case of Buerger's disease with vasculopathy and skin fibrosis requiring differential diagnosis from systemic sclerosis.","authors":"Masashi Funada, Shingo Nakayamada, Shunsuke Fukuyo, Satoshi Kubo, Aya Nawata, Yuya Fujita, Yoshiya Tanaka","doi":"10.1093/mrcr/rxae036","DOIUrl":"https://doi.org/10.1093/mrcr/rxae036","url":null,"abstract":"Buerger's disease is characterized by peripheral ischemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger's disease in a 51-year-old male who presented with findings resembling systemic sclerosis. The patient exhibited Raynaud's phenomenon in year X-3, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year X. Diagnostic testing showed positive microvasculopathy on nailfold videocapillaroscopy (NVC) and positive fibrosis on skin biopsy. Although the patient fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for systemic sclerosis, several findings in this case were atypical for systemic sclerosis, including left-right asymmetry in finger involvement, nail loss, and negative autoantibody tests. Contrast-enhanced computed tomography showed poor perfusion of the right ulnar artery, and a heavy smoking history was established in the patient case. Therefore, based on Shionoya's criteria, he was diagnosed with a case of Buerger's disease confined to the upper extremity. Smoking cessation and vasodilator therapy resulted in the prompt resolution of ischemic symptoms, skin hardening, and ulcerations. Furthermore, NVC abnormalities improved, and ulnar artery occlusion showed reperfusion on repeat testing. The present case suggests that hypoxemia-driven microvasculopathy may contribute to vascular occlusion and skin fibrosis observed in this atypical presentation.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141857474","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

A case of cryopyrin-associated periodic syndrome due to somatic mosaic mutation complicated with recurrent circinate erythematous psoriasis. 体细胞镶嵌突变引起的冷冻素相关周期综合征并发复发性环状红斑性银屑病1例。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-08 DOI: 10.1093/mrcr/rxad067

Taiki Ando, Yoshiyuki Abe, Ken Yamaji, Ryuta Nishikomori, Naoto Tamura

{"title":"A case of cryopyrin-associated periodic syndrome due to somatic mosaic mutation complicated with recurrent circinate erythematous psoriasis.","authors":"Taiki Ando, Yoshiyuki Abe, Ken Yamaji, Ryuta Nishikomori, Naoto Tamura","doi":"10.1093/mrcr/rxad067","DOIUrl":"10.1093/mrcr/rxad067","url":null,"abstract":"Cryopyrin-associated periotic syndrome (CAPS) is a rare autoinflammatory disease caused by genetic variants in innate immunity genes. Autoinflammatory diseases, including CAPS, mediate proinflammatory cytokines such as interleukin (IL)-1 and IL-18 and result in severe systemic inflammation. A gain-of-function mutation in the NLR family pyrin domain-containing 3 (NLRP3) gene, which encodes the protein cryopyrin, was identified to be responsible for CAPS in 2001, and since then several additional pathogenic mutations have been found. Moreover, other phenotypes have been identified based on severity and symptomatology, including familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease/chronic neurologic cutaneous articular syndrome. Prompt diagnosis of CAPS remains challenging, however, due to unspecific, extensive clinical signs, and delayed diagnosis and treatment targeting IL-1 lead to multiorgan damage. Another factor complicating diagnosis is the existence of somatic mosaic mutations in the NLRP3 gene in some cases, resulting in symptoms and clinical courses that are atypical. The frequency of somatic mosaic mutations in CAPS was estimated to be 19% in a systematic review. Psoriasis is a chronic inflammatory skin disease that affects ∼3% of the global population. Although no reports have shown complication between CAPS and psoriasis, these diseases have several similarities and potential relationships, for instance activation of T helper 17 cells in the dermis and increased NLRP3 gene expression in psoriatic skin compared with normal skin. Here, we report a case of CAPS due to a somatic mosaic mutation with recurrent circinate erythematous psoriasis.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138465246","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

Neurofascialvascular training for the treatment of Raynaud's phenomenon: A case report. 治疗雷诺现象的神经-筋膜-血管训练：病例报告。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-08 DOI: 10.1093/mrcr/rxae026

Paolo Bertacchini

{"title":"Neurofascialvascular training for the treatment of Raynaud's phenomenon: A case report.","authors":"Paolo Bertacchini","doi":"10.1093/mrcr/rxae026","DOIUrl":"10.1093/mrcr/rxae026","url":null,"abstract":"Primary Raynaud's phenomenon (PRP) is characterised by episodic, reversible, and disabling vasospasms of the peripheral arteries. In the most severe cases, it can lead to ulceration of the fingers and toes. Neuro fascial VascularTraining (NFVT) is a novel therapeutic approach for treating PRP. NFVT aims to enhance peripheral circulation and stimulate the autonomic nervous system (ANS) by engaging multiple physiological mechanisms simultaneously. This integrated approach works to reduce vasospasms and alleviate associated symptoms through neurodynamic and myofascial interventions. A 54-year-old woman, who has experienced pain and hypoesthesia in her hands for 9 years, received a diagnosis of PRP without systemic sclerosis in 2014. The patient reported daily colour changes in her fingers, along with pain and a temporary decrease in tactile sensitivity. The patient engaged in ten 30-minute exercise sessions, and the clinical outcomes were assessed based on several parameters. These included the frequency and duration of vasospastic attacks, evaluated using the Raynaud Condition Score, as well as pain and tingling, measured through the daily Numeric Rating Scale. The Composite Autonomic Symptom Score (COMPASS 31) was utilised to assess dysautonomia, while the frequency of medication use and the Disabilities of the Arm, Shoulder, and Hand questionnaire were also considered. The results indicated a significant improvement in symptoms. NFVT improved symptoms and motor dysfunction in a patient with Raynaud's syndrome, demonstrating how NFVT can increase peripheral blood flow, stimulate the ANS, and improve symptoms in PRP.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140900759","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

Avacopan's potential to decrease MPO-ANCA titres concurrent with ameliorated activity in ANCA-associated vasculitis. Avacopan 可降低 MPO-ANCA 滴度，同时改善 ANCA 相关性血管炎的活动。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-08 DOI: 10.1093/mrcr/rxae016

Tomoki Taniguchi, Ryosuke Hiwa, Mikihito Shoji, Eriho Yamaguchi, Mirei Shirakashi, Hideo Onizawa, Hideaki Tsuji, Koji Kitagori, Ran Nakashima, Shuji Akizuki, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Akio Morinobu

{"title":"Avacopan's potential to decrease MPO-ANCA titres concurrent with ameliorated activity in ANCA-associated vasculitis.","authors":"Tomoki Taniguchi, Ryosuke Hiwa, Mikihito Shoji, Eriho Yamaguchi, Mirei Shirakashi, Hideo Onizawa, Hideaki Tsuji, Koji Kitagori, Ran Nakashima, Shuji Akizuki, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Akio Morinobu","doi":"10.1093/mrcr/rxae016","DOIUrl":"10.1093/mrcr/rxae016","url":null,"abstract":"Avacopan, an orally administered C5a receptor antagonist, is effective in microscopic polyangiitis via the inhibition of neutrophil priming induced by C5a. However, the exact effect of avacopan on the production of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is yet to be clearly established. This report presents a microscopic polyangiitis patient without major organ damage where high levels of MPO-ANCA persisted with high-dose steroid therapy and azathioprine, but the addition of avacopan led to a reduction in MPO-ANCA titres. The present case implies that avacopan-mediated inhibition of C5a may lead to a reduction in MPO-ANCA levels, thereby potentially ameliorating the pathophysiology of ANCA-associated vasculitis. Nevertheless, the impact of avacopan on MPO-ANCA production cannot be asserted solely based on this report; therefore, further examination is necessary through subgroup analysis using data from larger-scale studies.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140873989","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

Painful snapping of the middle finger caused by hyperplasia of the ulnar lateral band: A case report. 尺侧带增生导致的中指折断疼痛：病例报告。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-08 DOI: 10.1093/mrcr/rxad073

Anna Matsuo, Taku Suzuki, Hiroo Kimura, Noboru Matsumura, Takuji Iwamoto, Masaya Nakamura

引用次数: 0

Hypothermia, bradycardia, and hypotension during glucocorticoid or cyclosporine A therapy in a boy with Kikuchi-Fujimoto disease. 一名患有菊池-藤本氏病的男孩在接受糖皮质激素或环孢素 A 治疗期间出现低体温、心动过缓和低血压。

IF 0.9

Modern rheumatology case reports Pub Date : 2024-07-08 DOI: 10.1093/mrcr/rxae023

Yasuyoshi Hiramatsu, Kazuki Takahashi, Masaki Shimomura, Kota Taniguchi, Yuka Okura, Mitsuru Nawate, Yutaka Takahashi, Ichiro Kobayashi

{"title":"Hypothermia, bradycardia, and hypotension during glucocorticoid or cyclosporine A therapy in a boy with Kikuchi-Fujimoto disease.","authors":"Yasuyoshi Hiramatsu, Kazuki Takahashi, Masaki Shimomura, Kota Taniguchi, Yuka Okura, Mitsuru Nawate, Yutaka Takahashi, Ichiro Kobayashi","doi":"10.1093/mrcr/rxae023","DOIUrl":"10.1093/mrcr/rxae023","url":null,"abstract":"Kikuchi-Fujimoto disease (KFD) is an inflammatory disease of unknown aetiology characterised by fever and cervical lymphadenopathy. Although KFD is a self-limiting disease, patients with severe or long-lasting course require glucocorticoid therapy. We presently report a 17-year-old boy with KFD who had seven relapses since the onset at 4 years old. He suffered from hypothermia, bradycardia, and hypotension during the treatment with prednisolone or methylprednisolone. All of his vital signs recovered after cessation of the drug in addition to fluid replacement and warming. Thus, glucocorticoid was effective but could not be continued because of the adverse event. Although hypothermia developed during the treatment with 5 mg/kg/day of cyclosporine A (CsA) at his second relapse, he was successfully treated with lower-dose CsA (3 mg/kg/day). Thereafter, he had five relapses of KFD until the age of 12 years and was treated by 1.3-2.5 mg/kg/day of CsA. Hypothermia accompanied by bradycardia and hypotension developed soon after concomitant administration of ibuprofen at his fifth and sixth relapses even during low-dose CsA therapy. Conclusively, glucocorticoid, standard dose of CsA, or concomitant use of non-steroidal anti-inflammatory drugs may cause hypothermia, bradycardia, and hypotension and needs special attention. Low-dose CsA could be a choice for such cases with KFD.","PeriodicalId":94146,"journal":{"name":"Modern rheumatology case reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141082923","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