Interstitial lung disease in patients with connective tissue disease: Subtypes, clinical features and comorbidities in the Western Cape, South Africa

BACKGROUND: Interstitial lung disease (ILD) is highly prevalent in patients with connective tissue disease (CTD) and is poorly characterised in South Africa. OBJECTIVES: To describe the clinical, serological and radiological features of CTD-ILD and their associations in patients attending a tertiary referral hospital. METHODS: A cross-sectional study collating clinical, serological and radiological features of CTD-ILD in patients attending rheumatology and respiratory outpatient clinics in a tertiary referral hospital. RESULTS: Of 124 CTD-ILD patients, 37 (29.8%) had rheumatoid arthritis (RA), 32 (25.8%) systemic sclerosis (SSc) and 55 (44.4%) other autoimmune connective tissue diseases (OCTD). Most patients were female (86.3%), of mixed racial ancestry (75.0%), and the median age was 55 years. Nonspecific interstitial pneumonia (NSIP) was the most common ILD pattern (63.7%), followed by usual interstitial pneumonia (UIP) (26.6%). Overall, 60.5% were current or past smokers, 33.1% had previous pulmonary tuberculosis infection, and 75.6% had gastro-oesophageal reflux disease. Patients with RA were older, had similar frequencies of NSIP and UIP, and had significantly better pulmonary function tests than the SSc and OCTD groups. Within three years of CTD diagnosis, two-thirds of the SSc and OCTD patients and almost half of the RA patients had developed ILD. Clinical features, chest X-rays and pulmonary function tests correlated poorly with high-resolution computerised tomography (HRCT). No case of acute pneumonitis was documented in CTD-ILD patients treated with methotrexate (MTX). CONCLUSION: We suggest routine HRCT in all newly diagnosed CTD patients, particularly those with SSc and OCTD, where more than two-thirds of the patients had developed ILD within three years of their CTD. The use of MTX was not associated with the development of acute pneumonitis in patients with ILD. KEY POINTS: Clinical features, chest X-rays and pulmonary function tests correlated poorly with high-resolution computerised tomography (HRCT). Smoking, environmental toxins, gastro-oesophogeal reflux and previous pulmonary tuberculosis infection were significant comorbidities in CTD-ILD patients. Early screening of ILD with HRCT is recommended, particularly in SSc. Use of MTX before and after ILD diagnosis was not associated with acute pneumonitis.