Department of Pneumonology, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
Respiration. 2012;83(1):5-12. doi: 10.1159/000335128. Epub 2012 Jan 11.
Over the past 10 years, the widespread clinical applicability of semi-invasive and noninvasive diagnostic tools including medical thoracoscopy and ultrasonography has expanded the occurrence of pleural effusions to include several rare diseases such as granulomatous, connective tissue and autoimmune disorders including sarcoidosis, granulomatosis with polyangiitis (Wegener's), systemic sclerosis, lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, amyloidosis, Langerhans cell histiocytosis, and others. The purpose of this review is to summarize the current state of the knowledge regarding pathogenetic mechanisms of pleural involvement in rare disease entities and to highlight the need for more efforts to understand the underlying mechanisms for a more effective therapy.
在过去的 10 年中，包括胸腔镜和超声检查在内的半侵袭性和非侵袭性诊断工具的广泛临床应用，使胸腔积液的发生扩展到包括几种罕见疾病，如肉芽肿性、结缔组织和自身免疫性疾病，包括结节病、肉芽肿性多血管炎（韦格纳氏）、系统性硬化症、红斑狼疮、类风湿关节炎、干燥综合征、淀粉样变性、朗格汉斯细胞组织细胞增生症等。本文的目的是总结目前对罕见疾病实体胸膜受累的发病机制的认识，并强调需要进一步努力了解潜在机制，以实现更有效的治疗。
