Sarcoidosis: a long-term follow up study

Abstract

Background and aim of work: The study investigated the occurrence, time and mode of presentation, clinical features, course of pulmonary disease and prognosis of all patients with sarcoidosis looked after by one physician in a district general hospital during the period 1965-1996. The hospital covered a catchment population of 150,000.

Methods: A detailed review of 212 patient 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 certificates 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 histological proof confirming sarcoidosis in 149 cases. There was a slightly higher incidence in females than males, with four familial instances documented. There was pulmonary involvement in 192 cases classified in the usual way at presentation and the course of these patients was studied. Patients with 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 infiltration or fibrosis but little disability or disease progression, and another with advancing disease refractory to steroid therapy with a bad prognosis.

Conclusions: The good prognosis of patients with Stage 1 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, the numbers of patients progressing from one stage to another was small. Although there was a small group of patients with steroid-refractory, progressive, fibrosis with a bad prognosis, the mortality rate from sarcoidosis in this study was small.