Pulmonary manifestations in connective tissue disorders: Hospital-based study at a Tertiary Care Hospital.

ABSTRACT

Background. The pleuro-pulmonary manifestations frequently occur in patients with the connective tissue disorders (CTDs), and limited data are available on this topic from India. Methods. Between January 2002 and December 2006, 195 patients with various CTDs having respiratory symptoms were evaluated for respiratory system involvement. Results. Interstitial lung disease (ILD) was the commonest (38.5%) presentation of CTDs. It was observed in nearly threefourth of the cases with scleroderma followed by rheumatoid arthritis (RA) cases (44.5%). Pulmonary arterial hypertension was observed in 53.8% and 60% of cases with scleroderma and mixed connective tissue disorder (MCTD), respectively. In RA and systemic lupus erythematosus (SLE), pulmonary hypertension was a rare presentation. Pulmonary function tests were abnormal in all the patients with MCTD, 89.9% patients with RA, 84.6% patients with scleroderma and nearly one-third patients with SLE. Restrictive defect was the most common abnormality [RA (88.7%), scleroderma (72.7%), SLE (66%)]. High resolution computed tomography revealed reticulonodular lesions (n=78); pleural effusion (n=15, mainly in patients with RA and SLE); honeycomb appearance (n=14; mainly in patients with RA and scleroderma); and bronchiectasis (n=9, mainly in patients with RA). Conclusions. Pulmonary manifestations are common in patients with CTDs, ILD being the most common pulmonary involvement. All patients with CTDs should be systematically evaluated and monitored for pulmonary involvement.