OUTCOME IN SARCOIDOSIS - THE RELATIONSHIP OF RELAPSE TO CORTICOSTEROID-THERAPY

Study objective: To determine the demographic, clinical and radiograph ic characteristics of corticosteroid-treated patients with sarcoidosis who developed relapse following a period of clinical stability lastin g longer than 1 month, and to compare these characteristics with those of a group of patients with sarcoidosis who were not treated. Design: Historic, concurrent and prospective, nonrandomized, observational st udy. Setting: Ambulatory sarcoidosis clinic in a university city hospi tal. Patients: Over a 4-year calendar period, 337 patients with sarcoi dosis were prospectively enrolled in a registry. One hundred eighteen patients were assigned to a spontaneous remission group when symptoms resolved without treatment, and 103 were assigned to an induced remiss ion group when symptoms resolved following corticosteroid therapy and successful discontinuation. In 116 patients assigned to a recalcitrant group, therapy could not be stopped for 1 month or more owing to seve rity of symptoms or lack of compliance. We defined relapse as a recurr ence of symptoms of sufficient severity to warrant treatment with cort icosteroids, following a remission without treatment lasting greater t han 1 month. Intervention: Patients who were judged to be sufficiently symptomatic to preclude observation without treatment or who failed t o respond to conservative treatment with topical or inhaled corticoste roids or nonsteroidal anti-inflammatory agents were treated with syste mic corticosteroids at a target dose of 20 mg prednisone per day for 1 year. Measurements and results: We observed a 74% relapse rate in the induced remission group, but only an 8% relapse rate in the spontaneo us remission group (p<0.01). Relapse occurred with similar frequency i n whites and African-Americans (20% vs 28%), despite a lower treatment rate in white patients than in African-Americans (43% vs 76%; p<0.01) . White patients maintained a sustained remission with twice the frequ ency of African-Americans (58% vs 29%; p<0.01). During relapse, 40% of chest radiographs showed no change in type, but there was a significa nt increase in interstitial profusion (p<0.05). Initial presentation w ith asymptomatic chest radiographic abnormalities, erythema nodosum, o r peripheral adenopathy portended a favorable prognosis, with sustaine d remission in 60% of such patients lasting 130+/-226 months from time of diagnosis. In contrast, patients who presented with musculoskeleta l complaints were nine times, and those with symptoms from hepatic inv olvement were three times more likely to suffer relapse than to sustai n remission without receiving corticosteroids, Most relapses (50%) occ urred between 2 and 6 months after discontinuing steroid therapy, but late relapse was not unusual, occurring more than 12 months after disc ontinuing steroid therapy in 20% of patients with induced remission. C onclusions: Relapse occurred frequently in patients with sarcoidosis w ho had been treated with corticosteroids, and rarely occurred in patie nts who had not been treated with corticosteroids in the past. The str iking difference in relapse rate between treated and untreated patient s suggests that patients with disease that would later be severe and p rotracted were almost unerringly identified early in their course. One explanation is that severe presenting symptoms portend a protracted a nd recurrent course; an alternative explanation is that corticosteroid s contributed to the prolongation of the disease by delaying resolutio n.