Characteristics of Connective Tissue Diseases Associated Interstitial Lung Diseases - A Single Centre Study in Sri Lanka.

Abstract

Introduction: Interstitial Lung Diseases are the major cause of mortality in Connective Tissue Diseases.

Objectives: Our aim is to describe patients with Interstitial Lung Disease associated with Connective Tissue Diseases (CTD-ILD) in Chest Clinic -Colombo 08.

Methods: We conducted a descriptive cross-sectional study at Central Chest Clinic, Colombo, Sri Lanka and data of all patients attending the clinic during were analysed.

Results: The sample consisted of 83 consecutive patients diagnosed with CTD-ILD. The age ranged from 24 to 72 years with mean (SD) age of 55.6 (10.6) years. The majority was female patients (n=70, 88.6%). The majority 53.0% (n=44) had RA and SSc was the second commonest CTD-ILD (n=22, 26.5%). NSIP 51(61.9%) was the most frequently observed HRCT pattern in our cohort whereas UIP was the second commonest. RA-ILD subgroup demonstrated female (n=37, 86.0%) preponderance. NSIP (n=24, 55.8%) was the commonly observed HRCT pattern fallowed by UIP (n=15, 34.9%) pattern. Among RA-ILD patients, 28 (65.1%) had positive Rheumatoid factor. Majority of (n=20, 90.9%) SSc-ILD were females. Sixteen (72.7%) of them had NSIP pattern in HRCT analysis fallowed by UIP (n=6, 27.3%).

Conclusion: RA-ILD was the most frequent type of CTD-ILD followed by SSc. More importantly; the present study revealed the predominant NSIP pattern and clear female preponderance in RA-ILD compared to global data. We recommend prospective multicenter studies to be carried out and prospective disease registries to be established to explore the epidemiological, clinical, radiological and prognostic characteristics of CTD‑ ILD in Sri Lanka.