PULMONARY MANIFESTATIONS IN A COHORT OF EGYPTIAN SYSTEMIC SCLEROSIS PATIENTS

Abstract

SUMMARY AND CONCLUSION S ystemic sclerosis is a systemic autoimmune disease that is characterised by endothelial dysfunction resulting in a small-vessel vasculopathy, fibroblast dysfunction with resultant excessive collagen production and fibrosis, and immunological abnormalities. Skin involvement is a nearly universal feature of SSc. It is characterized by variable extent and severity of skin thickening and hardening. The gold standard or approved method for assessing and measuring the dermal skin thickness is called the Modified Rodnan Skin Score (MRSS). Nail-fold Capillary Microscopy is a noninvasive procedure of significant value for the assessment of microvascular abnormalities in SSc. The typical findings demonstrate dilated capillary loops, giant capillaries, hemorrhages, avascular areas and structural derangement. Recent evidence indicates a predictive value of Nail-fold Capillary Microscopy for future severe organ involvement. The two most common types of direct pulmonary involvement are ILD and PH and all of the known pulmonary manifestations reported have been described in each of the subsets of disease. The aim of the study was to study the occurrence of the pulmonary manifestations especially ILD and PH in a cohort of Egyptian patients with SSc and their relation to the extent of skin affection. The present study included 30 SSc patients who fulfilled the 2013 ACR/EULAR Classification Criteria for SSc (Van Den Hoogen et al., 2013). Patients with other connective tissue diseases, those with PH or ILD of other causes, those with pulmonary malignancies, and those who are smokers were excluded from the study. Twenty-two patients were females (73.3%) and 8 were males (26.7%), their age ranged from 22 to 65 years, with mean SD 40.97 ± 12.63 years and mean duration of disease 9.65 ± 8.18 years. All patients were subjected to detailed medical history taking together with full clinical examination including rheumatological examination. Skin fold thickness was scored for all patients by MRSS (Clements et al., 1995). Laboratory investigations including CBC, ESR, CRP, renal function tests, liver function tests, PFTs, Plain x-ray chest, Transthoracic Echocardiography (ECHO), HRCT of the chest and Nail Fold Capillary Microscopic Examination were done. Our study included 30 SSc patients 22 females (73.3%) and 8 males (26.7%), their age ranged from 22 to 65 years, with mean SD 40.97 ± 12.63 years and mean duration of disease 9.65 ± 8.18 years. All our studied patients (100%) showed some degree of restriction in the pulmonary function tests; since FEV-1% level was below normal level with a mean of 66.91 ± 16.13, FVC% was below normal with a mean of 60.23 ± 12.02, FEV-1/FVC% was above normal with a mean of 93.29 ± 11.44. In our study, 25 patients (83%) showed evidence of ILD by the HRCT of the chest including the 5 (17%) patients with evidence of PH in HRCT in the form of dilated pulmonary artery and in the echocardiography with RVSP>35 mmHg, TR and dilated RV.