Treatment: If sarcoidosis is a granulomatous response to an elusive, persistent antigen (unitarian view), the premise that its suppression would forestall the evolution of fatal or disabling pulmonary fibrosis is reasonable. Proof of principle would reside in demonstration of long-term efficacy. If, however, the granuloma, which is the defining characteristic of sarcoidosis, proves to be a regressive response to inefficient, antecedent, cell-mediated processing—probably to a variety of antigens—then its suppression might be inexpedient. I suspect that the belief that persons with sarcoidosis experience a 10% mortality, cited in several standard references, strongly influences the decision to intervene. However, sarcoidosis mortality in reporting population-based settings, which should resemble clinical practice, is approximately 0.5%.5
It follows under the (doubtful) assumption that, on balance, CST is beneficial long-term in individuals with pulmonary shadowing, sustained treatment of a large number of asymptomatic persons—most of whom would have a self-limited course—would be required to prevent one death. Hillerdal et al9–
furnished CST in a population-based setting only to persons exhibiting progressive pulmonary shadowing, and reported a mortality of 0.8% (4 of 409 patients; mean age at death, 74 years). Johnston,10–
who employed the same indication in a referral setting, furnished it in only 3% and reported a mortality of 0%. The redoubtable Professor Dame Margaret Turner-Warwick recommended observation for 6 to 12 months without treatment, hoping for spontaneous remission in persons with asymptomatic pulmonary shadowing and normal pulmonary function (written communication; March 30, 1999). Lacking evidence of sufficient benefit to offset the adverse effects of sustained CST in persons with pulmonary shadowing, the majority of whom would experience a favorable outcome in the absence of intervention, the framers of a joint statement on sarcoidosis were unable, absent compelling symptoms, to define its indications.11
I do not know to what extent these conservative indications have influenced physician’s practice. Whether “substantial progress” has been made in understanding the nature of sarcoidosis, its etiology, or its treatment since publication of Dr. DeRemee’s editorial. I leave to the reader and to time to judge.