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Corticosteroid therapy can relieve symptoms and suppress inflammation and granuloma formation. Absolute indications for steroid therapy include central nervous system, ocular, and cardiac involvement, as well as nephrocalcinosis and hypercalcemia. The decision to use steroids to treat pulmonary sarcoidosis is based on a combination of clinical, functional, and radiologic findings and remains a subject of debate (38,39) (Figure 28). Initial response to steroid treatment does not, however, preclude progression to pulmonary fibrosis and death.
The course and prognosis of sarcoidosis correlate with the mode of onset of the disease, the patient's race, and the presenting stage (3,5,6). An acute onset (eg, Löfgren syndrome) usually heralds a self-limited course with spontaneous resolution, whereas an insidious onset may be followed by relentless progressive fibrosis. Nonwhite patients tend to have extrathoracic manifestations of the disease and therefore a poorer prognosis. Overall, two-thirds of African-American patients with sarcoidosis require treatment, compared with one-third of white American patients (5). Resolution within 1-2 years occurs in 60% of patients presenting with stage I disease, 46% with stage II, and 12% with stage III (8).
There is a 5% mortality rate due directly to sarcoidosis (8). Patients may succumb to cor pulmonale, respiratory failure, or massive hemoptysis. A very small percentage of patients die of sudden cardiac death due to granulomatous involvement of the conduction pathways and myocardium (33).
Treatment and Prognosis