Source: Annual Congress 2002 - Interstitial lung disease
Disease area: Interstitial lung diseases

Abstract

Background
Sjogren's syndrome is the second commonest autoimmune disease, with primary Sjogren's (pSS) in a significant minority. The reported high incidence of pulmonary and lymphomatous disease led us to evaluate 10 patients attending respiratory and/or rheumatology clinics with a diagnosis of probable or definite pSS,(Fox's Criteria).
Results
i)9 patients were female. Mean age 61
ii)4 had respiratory symptoms with 5 showing varied CT changes typical of the condition - all 5 had fibrosis, 1 extensive lymphadenopathy and 2 concomitant brochciectatic features.
iii)8 had full pulmonary function assessment performed. 5 had evidence of restriction only,(mean FEV1/FVC 103%), with 4 of those having a significantly reduced transfer coefficient,(KCO mean 56).
Conclusions
Previous reports cite 75% of pSS patients develop pulmonary manifestations, even though most were asymptomatic (Constanopoulos SH et al;Chest 88:226-9). Our findings support this, with 80% having pulmonary involvement despite the majority being asymptomatic. CT scanning was useful in the diagnosis and differentiation of the pulmonary subtypes. We recommend serial CT scanning and pulmonary physiology assessment in the management of this condition, although one study suggests pulmonary involvement occurs early and most patients do not develop progressive lung disease (Davidon BK et al;Ann Rheum Dis 2000 Sep;59(9):709-12).

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Citations should be made in the following way:
S. A. Wright, R. P. Convery, N. Liggett (Craigavon, United Kingdom). Interstitial lung disease in Sjogren's syndrome. Eur Respir J 2002; 20: Suppl. 38, 3676

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