A LONGITUDINAL-STUDY OF PULMONARY-FUNCTION IN DANISH PATIENTS WITH SYSTEMIC-SCLEROSIS

Objective: To determine the types, prevalence and development of respi ratory abnormalities in patients with systemic sclerosis (SSc), and to correlate the results with clinical and serological findings. Methods : 176 patients with SSc observed longitudinally were retrospectively i ncluded in the study. The change per year of vital capacity (VC), forc ed expiratory volume in one second/vital capacity (FEV1/VC), diffusing capacity (DLco) and diffusing constant (Kco) of carbon monoxide from the first till the latest pulmonary function test were correlated to c linical and serological findings, including anti-centromere, anti-Scl- 70, and antinucleolar antibodies. Results: An isolated reduction of DL co was seen in 47% and a restrictive ventilatory pattern in 25% of the patients. Restrictive ventilatory pattern correlated to pulmonary fib rosis, dyspnoea, a low prevalence (13%) of anticentromere antibodies a nd a high prevalence of anti-Scl-70 antibodies (36%). Progression of D Lco reduction was related to long disease duration, presence of antice ntromere antibodies and absence of treatment with penicillamine. Concl usion: Pulmonary involvement is common in patients with SSc. The occur rence of different serological abnormalities in patients with restrict ive disease and in patients with progressive isolated reduction of DLc o, suggests that the two types of pulmonary damage may have different pathogeneses rather than being different stages in the progression of pulmonary damage.