Commeau P, Grollier G, Foucaúlt J P, Potier J C
Poumon Coeur. 1983 Jan-Feb;39(1):31-5.
Whereas pulmonary fibrosis is a very frequent manifestation of scleroderma, pulmonary hypertension of the precapillary type is a much rarer occurrence in this affection. Two cases of scleroderma with subsequent development of a pulmonary hypertension are reported. Various hypotheses have been advanced to explain the origin of this disorder during the course of scleroderma. Rather than a direct consequence of the sclerodermic pulmonary fibrosis it probably arises secondary to the specific, diffuse, primary pulmonary vascular lesions.
虽然肺纤维化是硬皮病非常常见的表现,但毛细血管前型肺动脉高压在这种疾病中则较为罕见。本文报告了两例硬皮病继发肺动脉高压的病例。关于硬皮病病程中这种病症的起源,已经提出了各种假说。它可能并非硬皮病肺纤维化的直接后果,而是继发于特定的、弥漫性的原发性肺血管病变。