Division of Rheumatology, Department of Medicine, Faculty of Health Sciences, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa.
Division of Pulmonology, Department of Medicine, Faculty of Health Sciences, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa.
Rheumatol Int. 2018 Apr;38(4):657-662. doi: 10.1007/s00296-017-3893-0. Epub 2017 Nov 28.
To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate-severe lung disease. Univariate predictors of ILD included: disease duration (OR 1.08, 95% CI 1.01-1.15); cough (OR 2.93, 95% CI 1.37-6.29); dyspnoea (OR 2.44, 95% CI 1.23-4.87); bibasal crackles (OR 7.58, 95% CI 3.31-17.37); diffuse cutaneous SSc (dcSSc) (OR 4.55, 95% CI 2.10-9.86) and a speckled anti-nuclear antibody (ANA) pattern (OR 2.47, 95% CI 1.25-4.90). Conversely, limited cutaneous disease (OR 0.22, 95% CI 0.09-0.50) and anti-centromere antibody (ACA) (OR 0.12, 95% CI 0.02-0.97) were protective. Independent predictors of ILD on multivariate analysis were bibasal crackles (OR 9.43, 95% CI 3.25-27.39), disease duration (OR 1.19, 95% CI 1.09-1.30) and speckled ANA (OR 2.95, 95% CI 1.22-7.15). Almost all (86.4%) patients received immunosuppressive treatment and the leading cause of death was related to ILD itself (44.4%). In this cohort of predominantly Black South Africans, SSc ILD was common and carried a poor prognosis. ILD occurred mainly, but not exclusively, in patients with dcSSc, especially those with a speckled ANA pattern. Conversely, the presence of ACA was protective against ILD.
调查南非系统性硬化症（SSc）患者中特发性肺纤维化（ILD）的频率、严重程度和预测因素。回顾性记录分析在三级结缔组织疾病诊所就诊的 SSc 患者。ILD 患者的定义为临床发现、影像学和肺功能检查相结合，与无 ILD 的患者相比，在人口统计学、临床特征和自身抗体方面进行比较。在纳入的 151 名患者中，大多数（86.8%）为黑人，40%有 ILD，其中 39%有中重度肺病。ILD 的单变量预测因素包括：疾病持续时间（OR 1.08，95%CI 1.01-1.15）；咳嗽（OR 2.93，95%CI 1.37-6.29）；呼吸困难（OR 2.44，95%CI 1.23-4.87）；双基底爆裂声（OR 7.58，95%CI 3.31-17.37）；弥漫性皮肤 SSc（dcSSc）（OR 4.55，95%CI 2.10-9.86）和斑点状抗核抗体（ANA）模式（OR 2.47，95%CI 1.25-4.90）。相反，局限性皮肤疾病（OR 0.22，95%CI 0.09-0.50）和抗着丝点抗体（ACA）（OR 0.12，95%CI 0.02-0.97）具有保护作用。多变量分析中 ILD 的独立预测因素为双基底爆裂声（OR 9.43，95%CI 3.25-27.39）、疾病持续时间（OR 1.19，95%CI 1.09-1.30）和斑点状 ANA（OR 2.95，95%CI 1.22-7.15）。几乎所有（86.4%）患者都接受了免疫抑制治疗，主要死亡原因与 ILD 本身有关（44.4%）。在这组主要为黑人的南非患者中，SSc ILD 很常见，预后不良。ILD 主要发生在，但不仅限于，dcSSc 患者中，尤其是具有斑点状 ANA 模式的患者。相反，ACA 的存在可预防 ILD。
