TREATMENT



While the treatment of these disorders depends on the particular one being discussed, certain gen­eral statements can be made. The most rational decisions can be made only if a clear diagnosis has been obtained. If the offending substance is identified, as in the pneumoconioses and the hy­persensitivity pneumonitides, avoidance is the best solution. When there is an active alveolitis component, corticosteroids may be of benefit. Dosage should be high (60 to 100 mg prednisone) initially and then reduced to the lowest possible dose that successfully suppresses the inflamma­tory response. Immunosuppressive agents are added to effect remission in certain vasculitides, such as Wegener’s granulomatosis, but are of questionable value in Goodpasture’s syndrome and have little efficacy in idiopathic pulmonary fibrosis. Plasmapheresis should be reserved for illnesses in which circulating antibody is known to be the etiological factor, as in Goodpasture’s syndrome.

The decision to initiate therapy with one of these relatively high-risk agents and how long to maintain treatment depend on the ability to eval­uate the presence of active alveolitis and to judge the effect of the therapeutic regimen. The chest roentgenogram does not ordinarily determine the acuteness of the process, although a diffuse ho­neycomb pattern usually suggests severe fibrosis. It is useful, however, to follow the course of the disease, although in itself it may not accurately predict the course or response to therapy. Like the chest x-ray, the pulmonary function studies give an indication of the degree of involvement, but they are not reliable in separating reversible phases of inflammation from far-advanced fibrosis. They are used predominantly to follow the course of the disease and the response to therapy. Depending on the initial abnormalities, any of a number of studies may be used, including the measurement of lung volumes, diffusing capacity, arterial blood gases at rest or during exercise, and maximum exercise tolerance. Gallium lung scan­ning and bronchoalveolar lavage are providing in­teresting insights into the mechanism of these dis­orders but have no proven clinical utility.