Indications for the treatment of sarcoidosis include some 10verbar;Xpercnt; of patients with critical extrathoracic involvement and some 15verbar;Xpercnt; with progressive pulmonary involvement. Corticosteroids provide the mainstay of treatment; there is no doubt as to their efficacy in the short or medium term, but their long-term efficacy remains uncertain. Relapse rates severe enough to require further treatment are significant, the rate depending on ethnic group, extent and severity of disease, and patient compliance. Osteopenia is frequent among patients receiving long-term corticosteroid therapy and can be detected in 50verbar;Xpercnt; of these patients. Intermittent etidronate therapy prevents loss of vertebral and trochanteric bone and significantly reduces the rate of new vertebral fractures. In long-term corticosteroid treatment, deflazacort may have fewer overall side effects and be significantly more sparing in bone demineralization. Hydroxy chioroquine, methotrexate, and azathioprine are steroid sparing and may be particularly helpful in the management of refractory forms of sarcoidosis. Inhaled corticosteroids yield no easily recognizable measured therapeutic benefit in pulmonary sarcoidosis but are helpful in the management of troublesome cough. Chloroquine and ketoconazole inhibit production of excess 1, 25-dihydroxyvitamin D and are helpful in the management of hypercalcemia. Organ transplantation (lung, kidney, heart, and liver) provides excellent medium-term results comparable to those resulting from these procedures in other disorders. Recurrence may occur in the allograft; it is usually asymptomatic and does not compromise patient survival. Cardiac conduction disturbances, arrhythmias, and cardiomyopathy attributable to sarcoidosis may present without obvious cause; sudden death remains likely among those with unrecognized or untreated cardiac lesions. Despite the use of intravenous or oral corticosteroids and other drugs including methotrexate, azathioprine, hydroxychloroquine, cyclosporine, and cyclophosphamide, with occasional recourse to cranial irradiation, the prognosis of CNS involvement by sarcoidosis remains poor with significant mortality and morbidity. Future prospects for therapy are discussed and await a more precise elucidation of the pathogenesis.
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