Suresh Sumatha Channapatna, Hasan Abida, Zonnoor Seyedeh Leila, Anziska Yaacov, Christopher-Stine Lisa, Tanji Kurenai, Kabani Naureen
Department of Internal Medicine, SUNY Downstate Health Sciences, Brooklyn, NY, USA 11226.
Department of Rheumatology, SUNY Downstate Health Sciences, Brooklyn, New York, USA 11226.
Neuromuscul Disord. 2023 Jul;33(7):570-574. doi: 10.1016/j.nmd.2023.04.004. Epub 2023 Apr 7.
IgG4-Related Disease (IgG4-RD)is a chronic fibroinflammatory disease typically characterized by inflammation or tumefaction of the organs involved. Skeletal muscle is not one of the typical organs involved in IgG4-RD. Isolated myositis related to IgG4-RD without common organ involvement such as lacrimal or salivary glands or retroperitoneal fibrosis is a controversial and debatable entity. Here we report a case of inflammatory myopathy in an elderly woman with several atypical clinical, lab, and histopathological findings suggestive of IgG4-related myositis. Two such case reports of IgG4-related myositis were reported in the literature review. This is a third case report of elevated IgG4 positive plasma cell infiltration in muscle with severe endomysial fibrosis and unusual myositis features (Figs. 1 and 2). This case-based review opens a possibility of a novel presentation of IgG4-RD and new pathogenesis in myositis.
IgG4相关性疾病（IgG4-RD）是一种慢性纤维炎症性疾病，其典型特征为受累器官的炎症或肿大。骨骼肌并非IgG4-RD的典型受累器官。与IgG4-RD相关的孤立性肌炎，且无泪腺或唾液腺、腹膜后纤维化等常见器官受累情况，是一个存在争议的实体。在此，我们报告一例老年女性的炎性肌病，该病例具有一些非典型的临床、实验室和组织病理学表现，提示为IgG4相关性肌炎。在文献综述中报道了两例此类IgG4相关性肌炎的病例报告。这是第三例关于肌肉中IgG4阳性浆细胞浸润增加、伴有严重肌内膜纤维化和不寻常肌炎特征的病例报告（图1和图2）。基于该病例的综述为IgG4-RD的新表现形式及肌炎的新发病机制提供了一种可能性。
