Birnbaum Julius, Danoff Sonye, Askin Frederic B, Stone John H
Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Arthritis Rheum. 2007 Jun;56(6):2065-71. doi: 10.1002/art.22633.
Microscopic polyangiitis (MPA) may present with a syndrome that resembles idiopathic pulmonary fibrosis (IPF). We describe an MPA patient with the clinical presentation of a "pulmonary-muscle" syndrome in which interstitial lung disease antedated the onset of myopathy. Identification of vasculitis on muscle biopsy was instrumental in recognizing clinical, radiographic, and histopathologic features that were more characteristic of MPA than of IPF. Institution of glucocorticoid and cyclophosphamide therapy led to the induction of a complete remission. The histologic findings in this case implicate subclinical episodes of alveolar hemorrhage as the mechanism of interstitial lung disease in MPA.
显微镜下多血管炎（MPA）可能表现出与特发性肺纤维化（IPF）相似的综合征。我们描述了一名MPA患者，其临床表现为“肺-肌肉”综合征，其中间质性肺疾病早于肌病发作。肌肉活检发现血管炎有助于识别那些更符合MPA而非IPF特征的临床、影像学和组织病理学特征。糖皮质激素和环磷酰胺治疗促使病情完全缓解。该病例的组织学发现提示肺泡出血的亚临床发作是MPA中间质性肺疾病的发病机制。
