复发性多软骨炎和临床淀粉性皮肌炎共存:一种罕见的自身免疫性疾病关联。

Rafael A Ríos-Rivera, Luis M Vilá
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引用次数: 0

摘要

复发性多软骨炎(RPC)是一种罕见的自身免疫性全身性疾病,其特征是软骨组织的反复炎症。它可以单独发生,也可以与其他自身免疫性疾病、血管炎或血液系统疾病联合发生。然而,RPC与皮肌炎的关联是非常罕见的。在此,我们报告了一例38岁男性并发RPC和临床淀粉样病变皮肌炎(CADM)的病例,其表现为耳廓软骨炎、鼻软骨炎、多关节炎、gottron丘疹、指尖丘疹,皮肤活检符合皮肌炎,抗黑色素瘤分化相关基因5 (MDA5)抗体阳性。皮质类固醇可消除RPC特征,但CADM表现对皮质类固醇、环磷酰胺、硫唑嘌呤和羟氯喹具有耐药性。随后用利妥昔单抗治疗可有效控制CADM的表现。该病例强调了认识到CADM是与RPC相关的自身免疫性疾病的一部分的重要性,并保持高度的认识,以启动有效的治疗,以避免与这些疾病相关的长期并发症。
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Coexistent Relapsing Polychondritis and Clinically Amyopathic Dermatomyositis: A Rare Association of Autoimmune Disorders.

Relapsing polychondritis (RPC) is an uncommon autoimmune systemic disease characterized by recurrent inflammation of the cartilage tissue. It can occur alone or in association with other autoimmune diseases, vasculitis, or hematologic disorders. However, the association of RPC with dermatomyositis is extremely rare. Herein, we present a case of a 38-year-old man who developed concurrent RPC and clinically amyopathic dermatomyositis (CADM) manifested by auricular chondritis, nasal chondritis, polyarthritis, gottron papules, fingertip papules, skin biopsy consistent with dermatomyositis, and positive antimelanoma differentiation-associated gene 5 (MDA5) antibodies. RPC features resolved with corticosteroids, but CADM manifestations were resistant to corticosteroids, cyclophosphamide, azathioprine, and hydroxychloroquine. Subsequent therapy with rituximab was effective to control CADM manifestations. This case highlights the importance of recognizing CADM as part of the autoimmune diseases linked with RPC and maintaining a high level of awareness to initiate effective therapy to avoid the long-term complications associated with these conditions.

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自引率
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发文量
35
审稿时长
12 weeks
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