Peshin R, O'Neill L, Browne P, O'Connell P, Kearns G
Department of Rheumatology, Beaumont Hospital, Dublin, Republic of Ireland.
Clin Rheumatol. 2009 Jun;28 Suppl 1:S31-4. doi: 10.1007/s10067-009-1098-y. Epub 2009 Feb 5.
Interstitial lung disease is an important cause of mortality and morbidity in patients with systemic sclerosis (SSc). There are currently no recommended guidelines for management of these patients. This is probably due to the rarity of this condition, as well as clinical trials with only a small number of cases. There are published case report and case series along with the two main trials, viz. Scleroderma Lung Study and the Fibrosing Alveolitis Study, but again, there is no consensus on treatment protocols. In this report, we present a case of aggressive interstitial lung disease in a patient with SSc, which improved dramatically on treatment with intra-venous cyclophosphamide and high dose prednisolone therapy.
间质性肺病是系统性硬化症(SSc)患者死亡和发病的重要原因。目前尚无针对这些患者管理的推荐指南。这可能是由于这种疾病的罕见性,以及临床试验病例数量较少。除了两项主要试验,即硬皮病肺部研究和纤维化肺泡炎研究外,还有已发表的病例报告和病例系列,但同样,在治疗方案上没有达成共识。在本报告中,我们介绍了一例SSc患者的侵袭性间质性肺病病例,该病例在接受静脉注射环磷酰胺和高剂量泼尼松龙治疗后显著改善。