Novak Srdan
Odjel za reumatologiju i klinikku imunologiju, Kresimirova 42, 51000 Rijeka.
Reumatizam. 2010;57(2):105-8.
Intersitial lung disease is a frequent complication of systemic sclerosis that often has a poor pognosis and together with pulmonary arterial hypertension are the most common cause of death in scleroderma patients. For detection and evaluation of interstitial lung disease, high-resolution CT and pulmorary functional tests are pivotal. The decision about whether to start treatment is often the most difficult challenge. Patients with short duration of systemic disease with recent deterioration in DCO are the candidates for immunosupressive therapy. Best current initial treatment is intravenous monthly cyclophosphamide together with low-dose oral glucocorticoids although azathioprine and mycophenolate mofetil are also widely used.
间质性肺病是系统性硬化症的常见并发症，通常预后较差，与肺动脉高压一起是硬皮病患者最常见的死亡原因。对于间质性肺病的检测和评估，高分辨率CT和肺功能测试至关重要。决定是否开始治疗往往是最困难的挑战。系统性疾病病程短且近期一氧化碳弥散量（DCO）恶化的患者是免疫抑制治疗的候选者。目前最佳的初始治疗是每月静脉注射环磷酰胺联合低剂量口服糖皮质激素，尽管硫唑嘌呤和霉酚酸酯也被广泛使用。
