Dutcher Jordan S, Bui Albert, Ibe Tochukwu A, Umadat Goyal, Harper Eugene P, Middlebrooks Erik H, Mohseni Michael M, Phillips Michael B
Mayo Clinic Alix School of Medicine, Jacksonville, Florida.
Department of Internal Medicine, Mayo Clinic, Jacksonville, Florida.
Proc (Bayl Univ Med Cent). 2021 Jan 14;34(3):384-386. doi: 10.1080/08998280.2020.1871177.
ANCA-associated vasculitis is a multiorgan autoimmune inflammatory disease that has a heterogeneous clinical presentation. Our case report provides additional evidence supporting the association between granulomatosis with polyangiitis and myositis. In our patient with proximal muscle weakness and pain, a normal creatine kinase and lack of antibodies to muscular fiber units ruled out primary myositis. Distinct magnetic resonance imaging of the brain within the deep gray matter in addition to positive serologies were consistent with a diagnosis of granulomatosis with polyangiitis. ANCA-associated vasculitis, specifically granulomatosis with polyangiitis, may be overlooked if musculoskeletal manifestations are the presenting symptoms. Prompt and aggressive treatment prevented this patient from experiencing multiorgan failure.
抗中性粒细胞胞浆抗体相关性血管炎是一种多器官自身免疫性炎症性疾病，临床表现具有异质性。我们的病例报告提供了更多证据，支持肉芽肿性多血管炎与肌炎之间的关联。在我们这位出现近端肌无力和疼痛的患者中，肌酸激酶正常且缺乏针对肌纤维单位的抗体，排除了原发性肌炎。除血清学阳性外，脑深部灰质的独特磁共振成像结果与肉芽肿性多血管炎的诊断相符。如果肌肉骨骼表现为首发症状，抗中性粒细胞胞浆抗体相关性血管炎，特别是肉芽肿性多血管炎，可能会被忽视。及时且积极的治疗使该患者避免了多器官功能衰竭。
