Sarcoidosis: a long term follow up study

Background and aim of work: The study investigated the occurrence, time and mode of presentation, clinical features, course of pulmonary disease and p rognosis of all patients with sarcoidosis looked after by one physician in a district general hospital during the period 1965-1996. The hospital cover ed a catchment population of 150,000. Methods: A detailed review of 212 pat ient notes was carried out with the aid of a purposely designed structured data collection form. Postal questionnaires were sent to those patients not currently attending to determine survival/state of health. Death certifica tes were analysed to determine the cause of death. Results: A diagnosis of sarcoidosis was based on clinical grounds in 63 of the 212 cases, with hist ological proof confirming sarcoidosis in 149 cases. There was a slightly hi gher incidence in females than males, with four familial instances document ed. There was pulmonary involvement in 192 cases classified in the usual wa y at presentation and the course of these patients was studied. Patients wi th Stage 1 and 2 disease had resolution rates in excess of 80%, and Stage 3 50%. For the remainder, two patterns emerged: one group with persistent in filtration or fibrosis but little disability or disease progression, and an other with advancing disease refractory to steroid therapy with a bad progn osis. Conclusions: The good prognosis of patients with Stage I disease was confirmed. There were fewer patients presenting with Stage 2 and 3 disease and their prognosis was better than in other published studies. Overall, th e numbers of patients progressing from one stage to another was small. Alth ough there was a small group of patients with steroid-refractory, progressi ve, fibrosis with a bad prognosis, the mortality rate from sarcoidosis in t his study was small.