[New knowledge on the diagnosis and prognosis of interstitial lung disease in scleroderma]

According to their clinical manifestation 101 patients with scleroderma were separated in morphea (n = 17) and progressive systemic sclerosis (PSS) (n = 84). PSS was divided into Type I (Acrosclerosis, n = 19), Type II (proximal ascending scleroderma, n = 61), and Type III (trunk scleroderma, n = 4). Lung function testing was done, including vital capacity, total capacity, FEV1.0, airway resistance, CO-transfer factor, lung compliance, blood gases at rest and during exercise. Patients suffering from Type-II or -III PSS, especially when displaying signs of inflammation in blood and serum (Form A) carry a much higher risk of developing severe life-limiting lung involvement compared to patients with morphea or Type-I PSS, or those without signs of inflammation (Form B). Lung function indicates the severity of functional involvement and inables to differentiate from obstructive bronchopulmonary disease. Bronchoalveolar lavage (BAL) showed a normal cell pattern in all Type-I patients (n = 5). In contrast, only six out of 33 Type-II patients had a normal BAL. PSS patients showing signs of inflammation in blood and serum (Form A) had, compared to those without inflammation (Form B), significantly elevated numbers of inflammatory cells in BAL. These results show that in patients with a high incidence of pulmonary manifestation in PSS (Type II, III, Form A) an inflammatory cell pattern in BAL was found significantly more frequently than in those patients without this risk factor (Type I, Form B). In the course of disease lung function of patients without inflammatory activity in BAL remained unchanged irrespective of whether there was an immunosuppressive treatment or not.(ABSTRACT TRUNCATED AT 250 WORDS)