Extended experience in the long-term corticosteroid treatment of pulmonary sarcoidosis

A 15-yr experience with symptomatic pulmonary parenchymal sarcoidosis treated with corticosteroids has included 250 patients. Within the past 5 yr 192 patients have been actively studied and these are reported in detail. Varied clinical observations are described. Serial measurements of vital capacity and steady-state carbon monoxide diffusing capacity were performed. All of these patients were sufficiently symptomatic and incapacitated to require treatment. Ninety-one percent were Black, 72% were female, and 72% were in the third and fourth decades. In response to treatment there was overall clinical improvement and an increase in vital capacity in 150 (83%) of 180 patients, although only a temporary increase in 12 of these patients. By contrast, only 82 (46%) showed an increase in carbon monoxide steady-state diffusing capacity and only temporarily in 19 patients. Changes in vital capacity correlate well with changes in symptoms and the radiographic appearance and are of great assistance in assessing the clinical course and need for continued treatment. The diffusing capacity is more difficult to interpret. Continued treatment was required in 74 patients (48%) because of recurrent clinical relapses as treatment was withdrawn. Such relapses were observed in 70% of the patients. Improvement and discontinuation of treatment was achieved in 66 patients (42%). In the 44 in whom there was no observed relapse, 34 were treated for less than 2.5 yr. In the total group, longer periods of treatment, 2.5-10 yr or more, were required in 104 patients (54%). In this series of 192 patients there were 7 deaths, with 22 deaths in the total 15-yr series of 250 patients. Of these, 18 were directly attributed to the sarcoidosis. Clinical improvement coincident with corticosteroid treatment and the frequency of relapse as treatment is withdrawn strongly support the benefits of corticosteroid treatment for symptomatic pulmonary sarcoidosis. When relapses occur and recur, retreatment is necessary and often long-term maintenance doses of prednisone (10-15 mg daily) are required for many years. The relatively infrequent problems related to this treatment are greatly exceeded by the clinical benefits.