Bittmann I, Holl-Ulrich K
Pathologisches Institut, Diakoniekrankenhaus Rotenburg, Rotenburg.
Z Rheumatol. 2009 Oct;68(8):639, 641-6, 648-9. doi: 10.1007/s00393-009-0481-z.
Rheumatic diseases or collagen vascular diseases represent a heterogeneous group of immunologically mediated inflammatory disorders. The respiratory system is often affected,the causes being manifold: infection, medication toxicity and specific manifestations of immunological processes due to the underlying disease. The lung can be involved in all its components. Due to their extremely broad differential diagnosis, pulmonary vasculitic syndromes still constitute a major challenge for the pathologist. Pulmonary involvement is frequent in primary systemic vasculitis (PSV) associated with anti-neutrophil-cytoplasmic antibodies (ANCA); other PSV only rarely affect the lungs. Histomorphologically, small vessel vasculitis with neutrophil alveolitis and diffuse alveolar hemorrhage, as well as extravascular intraparenchymal or peribronchial granulomas, can point to PSV. A single biopsy is often insufficient to identify all diagnostic criteria. Therefore, the selection of suitable biopsy material and correlation with clinical, serological and radiological parameters is indispensable. Almost all forms of interstitial lung disease may be present in collagen vascular disease; however, several parallel morphological types, rather than one in isolation, are frequently found.
风湿性疾病或胶原血管疾病是一组由免疫介导的异质性炎症性疾病。呼吸系统常受影响，其原因多种多样：感染、药物毒性以及基础疾病导致的免疫过程的特定表现。肺的各个组成部分都可能受累。由于其鉴别诊断范围极其广泛，肺血管炎综合征对病理学家来说仍然是一项重大挑战。与抗中性粒细胞胞浆抗体（ANCA）相关的原发性系统性血管炎（PSV）中肺部受累很常见；其他PSV很少累及肺部。组织形态学上，伴有中性粒细胞肺泡炎和弥漫性肺泡出血的小血管炎，以及血管外实质内或支气管周围肉芽肿，可能提示PSV。单次活检往往不足以确定所有诊断标准。因此，选择合适的活检材料并与临床、血清学和放射学参数相关联是必不可少的。几乎所有形式的间质性肺疾病都可能出现在胶原血管疾病中；然而，经常发现的是几种平行的形态学类型，而不是单一孤立的类型。
