Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy.

STUDY OBJECTIVE

To determine the demographic, clinical, and radiographic characteristics of corticosteroid-treated patients with sarcoidosis who developed relapse following a period of clinical stability lasting 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 study.

SETTING

Ambulatory sarcoidosis clinic in a university city hospital.

PATIENTS

Over a 4-year calendar period, 337 patients with sarcoidosis 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 remission 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 severity of symptoms or lack of compliance. We defined relapse as a recurrence of symptoms of sufficient severity to warrant treatment with corticosteroids, following a remission without treatment lasting greater than 1 month.

INTERVENTION

Patients who were judged to be sufficiently symptomatic to preclude observation without treatment or who failed to respond to conservative treatment with topical or inhaled corticosteroids or nonsteroidal anti-inflammatory agents were treated with systemic 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 spontaneous remission group (p < 0.01). Relapse occurred with similar frequency in 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 frequency of African-Americans (58% vs 29%; p < 0.01). During relapse, 40% of chest radiographs showed no change in type, but there was a significant increase in interstitial profusion (p < 0.05). Initial presentation with asymptomatic chest radiographic abnormalities, erythema nodosum, or peripheral adenopathy portended a favorable prognosis, with sustained remission in 60% of such patients lasting 130 +/- 226 months from time of diagnosis. In contrast, patients who presented with musculoskeletal complaints were nine times, and those with symptoms from hepatic involvement were three times more likely to suffer relapse than to sustain remission without receiving corticosteroids. Most relapses (50%) occurred between 2 and 6 months after discontinuing steroid therapy, but late relapse was not unusual, occurring more than 12 months after discontinuing steroid therapy in 20% of patients with induced remission.

CONCLUSIONS

Relapse occurred frequently in patients with sarcoidosis who had been treated with corticosteroids, and rarely occurred in patients who had not been treated with corticosteroids in the past. The striking difference in relapse rate between treated and untreated patients suggests that patients with disease that would later be severe and protracted were almost unerringly identified early in their course. One explanation is that severe presenting symptoms portend a protracted and recurrent course; an alternative explanation is that corticosteroids contributed to the prolongation of the disease by delaying resolution.