Pulmonary manifestations in Egyptian patients with systemic sclerosis

Abstract Aim of the work To study the occurrence of interstitial lung disease (ILD) and pulmonary hypertension (PH) in a cohort of Egyptian systemic sclerosis (SSc) patients and their relation to clinical variables. Patients and methods Thirty SSc patients underwent pulmonary function tests (PFTs), plain chest X-ray and chest high-resolution computed tomography to assess parenchymal abnormality and maximum fibrosis score (Fibmax). Transthoracic echocardiography to screen for evidence of pH. Nailfold capillary microscopy examination for recognizing nailfold capillary abnormalities and staging, skin thickness assessment by modified Rodnanu0027s skin score (MRSS). Results The mean age of the patients was 40.97 ± 12.63 years; 22 females and 8 males and disease duration was 9.65 ± 8.18 years. 17(56.7%) patients had diffuse cutaneous systemic sclerosis (dcSSc) and 13(43.3%) localized cutaneous (lcSSc). All patients showed restriction in the PFTs. ILD was present in 83% and PH in 17%; ground-glass opacity in 83.3%, septal thickening in 56.7%, honeycombing in 43.3%, bronchiolectasis in 23.3% and consolidations in 20% of the patients. ILD was significantly more in dcSSc than in lcSSc (p = 0.025). PH was present in 29.4% of the dcSSc patients but in none of the lcSSc patients. MRSS was significantly higher in patients with pulmonary affection than those without (p = 0.016) and in patients with ILD and PH than those with ILD alone. A significant correlation was found between the Fibmax and MRSS (r = 0.87, p  11 years, forced vital capacity (FVC)  20. Conclusion ILD is a frequent finding and PH is common in SSc patients especially the dcSSc subtypes. Disease duration, decline in FVC and increased skin thickness are associated with an increased risk of ILD.