pleuropulmonary involvement in connective tissue disorders in a tertiary care hospital in africa

1. Abstract 1.1 Background: Pleuropulmonary manifestations are very common and associated with high mortality in patients with Connective Tissue Disorders (CTDs). Their frequency and patterns are variable depending on the type of CTD. Limited data is available in these patients in Sub-Saharan Africa. 1.2 Aim: To study the spectrum of pleuropulmonary manifestations of CTDs in a tertiary hospital in Douala, Cameroon. 1.3 Methods: This was a cross sectional hospital-based study, including CTD patients recruited in the Douala general hospital Rheumatology and Chest Medicine clinics. CTD was defined according to the American College of Rheumatology and European League against Rheumatism (ACR/EULAR) criteria. Between January and August 2016, all consenting adult patients with various CTDs were assessed for pleuropulmonary involvement using a clinical examination, Chest X-Ray (CXR), Pulmonary Function Tests (PFT) and High Resonance Computed Tomography (HRCT). 1.4 Results: We included 54 CTD patients, 29 had Rheumatoid Arthritis (RA), 16 Systemic Lupus Erythematosus (SLE), 7 scleroderma (SSc) and 2 Mixed Connective Tissue Disease (MCTD). Pulmonary clinical signs and symptoms were present in 18 patients (33.3%) with dyspnea (27.8%) and cough (13.0%) being more predominant. Chest HRCT revealed mostly Interstitial Lung Disease (ILD) patterns in 16 patients (29.6%) with honeycombing lesions occurring in 9.3% of CTD patients, (10.3% of RA patients and 28.6% of SSc patients) and Ground glass lesions occurring in 5.6% of CTD patients [1/29 RA (3.4%) and 2/7 scleroderma patients (28.6%)].  PFTs abnormalities were seen in 51.9% of CTD patients (28/54) and restrictive defect was the most common abnormality as seen in 41.4% of RA patients,71.4% of scleroderma patients, 56.2% of SLE patients and 50.0% of MCTD patients. Pulmonary hypertension was a rare finding as seen only in 1 RA patient (3.4%). Seven out of 16 (43.8%) CTD patients with radiographic lesions and 15 out of 28 (53.6%) CTD patients with abnormal PFTs were asymptomatic. There was a significant association between pleuropulmonary involvement and methotrexate use, (p=0.046), and corticosteroid use (p=0.033). 1.5 Conclusion: One third of CTDs patients have clinical involvement. Half of the asymptomatic patients have radiographic and/or PFTs abnormalities. CTDs should be systematically screened for pulmonary involvement. 2. Keywords:  Africa; Connective Tissue Disorders; Pleuropulmonary