Grubben M J, Kerstens P J, Wiersma J M, Boerbooms A M, Festen J
Department of Internal Medicine, University Hospital, Nijmegen, Netherlands.
Neth J Med. 1993 Dec;43(5-6):269-76.
Pleuro-pulmonary involvement is frequently encountered in connective tissue disease. The pathological changes due to connective tissue disease are multifold. They include pleural, interstitial and nodular manifestations as well as airway lesions and vascular changes. In clinical decision making it is important to differentiate between effects of the underlying connective tissue disease, complications due to treatment, such as opportunistic infections, toxic and idiosyncratic drug reactions, and unrelated primary pulmonary diseases. We describe 2 patients with a connective tissue disease and pleuro-pulmonary complications. The diagnostic procedures are discussed. The result of the open lung biopsy was consistent with the diagnosis of rheumatic disease and also Sjögren's disease in the first patient and excluded infection and vasculitis in the second patient. Whenever histological investigation is needed to establish and/or exclude a diagnosis of pulmonary involvement in connective tissue disease, the open lung biopsy remains the "gold standard". We therefore propose a flow-chart for use in the clinical approach to the patient with interstitial lung disease of unknown origin.
结缔组织病常伴有胸膜肺受累。结缔组织病所致的病理改变多种多样，包括胸膜、间质及结节性表现，以及气道病变和血管改变。在临床决策中，区分潜在结缔组织病的影响、治疗引起的并发症（如机会性感染、毒性和特异质药物反应）以及无关的原发性肺部疾病非常重要。我们描述了2例患有结缔组织病并伴有胸膜肺并发症的患者，并讨论了诊断程序。首例患者的开胸肺活检结果与风湿性疾病及干燥综合征的诊断相符，而第二例患者的活检结果排除了感染和血管炎。每当需要组织学检查来确立和/或排除结缔组织病肺部受累的诊断时，开胸肺活检仍是“金标准”。因此，我们提出了一个流程图，用于指导对不明原因间质性肺病患者的临床处理。
