Charlotte Colp

Course of pulmonary sarcoidosis with and without corticosteriod therapy as determined by pulmonary function studies

Abstract Serial pulmonary function studies over a six year period in forty-three patients with pulmonary sarcoidosis are reported. Twenty patients received corticosteroid therapy at some time during the course of their illness. The single breath carbon monoxide diffusing capacity (Dco), the vital capacity (Vc), and the maximal mid-expiratory flow rate (MMF) were the principal indices followed. On the basis of the single breath diffusing capacity for carbon monoxide, the patients were divided into two groups: those with mild impairment of pulmonary function (Dco greater than 65 per cent of predicted normal), and those with severe impairment of pulmonary function (Dco 65 per cent of predicted normal or less). Pulmonary function improved spontaneously in only a small percentage of patients with sarcoidosis. Indeed, the diffusing capacity fell in some patients despite complete clearing on the x-ray film of the chest. Corticosteroids produced improvement in all but one of the patients with severe impairment of pulmonary function, but with the cessation of therapy reversion to the previous status occurred in most. Corticosteroid therapy had no consistent beneficial effect on patients in whom pulmonary function was only mildly impaired initially.

Pulmonary function studies as an aid in the differential diagnosis of pulmonary hypertension

Abstract Pulmonary function studies in patients with pulmonary hypertension secondary to interstitial fibrosis reveal reduced lung volumes, and the carbon monoxide diffusing capacity, measured by the single breath method, is one third or less of predicted. In patients with pulmonary hypertension secondary to primary pulmonary vascular disease the lung volumes were found to be generally normal and the diffusing capacity averaged 55 per cent of predicted. The relative preservation of the carbon monoxide diffusing capacity in the presence of severe obstruction to the pulmonary arterial system contrasts with the severe impairment of oxygen diffusion which may occur in this situation. To illustrate the diagnostic usefulness of pulmonary function studies in this connection, a patient is cited who presented with symptoms of pulmonary hypertension initially diagnosed as primary pulmonary hypertension. However, severe impairment of carbon monoxide diffusing capacity led to the correct diagnosis of interstitial pulmonary fibrosis, despite a normal x-ray film of the chest; this was confirmed by open lung biopsy.

PULMONARY FUNCTION FOLLOW-UP OF 120 PATIENTS WITH SARCOIDOSIS1

At Bronx Municipal Hospital Center for the past fifteen years we have been engaged in a study of sarcoidosis. In order to elucidate the natural history of the disease, pulmonary function studies were performed on all patients with sarcoidosis when first seen, repeated whenever possible several times within the first year of observation, and then subsequently a t least yearly. In treated cases follow-up function studies were performed even more frequently t o evaluate the effects of treatment and changes of corticosteroid dosage. Studies performed in all cases were lung volumes, carbon monoxide diffusing capacity by the single-breath method, and the timed vital capacities and maximum midexpiratory flow rate determined by rapid-speed spirometry. One hundred and twenty patients with pathological proof of diagnosis were followed for over 6 months. No attempt was made to evaluate the effect of treatment in a controlled fashion. Corticosteroid treatment was given when deemed indicated for extrapulmonary lesions or for severe pulmonary function impairment. In addition, early in the course of this study, 6 patients were treated for 2 months only for experimental purposes. Most other patients received 30-40 mg of prednisone daily for 1-2 months followed by gradual tapering of dosages as tolerated. Based on the initial pulmonary function data the patients in this study are divided into mild and severe groups, i.e., vital capacity (VC) and diffusing capacity ( D L ~ ~ ) greater than or less than 65% of predicted. TABLE 1 shows a comparison of the mild and severe groups. Note that the latter had a slightly higher percentage of females and a far higher percentage of Blacks than Whites and Puerto Ricans. Radiological evaluation indicated pulmonary parenchymal lesions more commonly, but not invariably, in the severe group. There were also a large number of Stage I 1 and 111 patients in the group with good function, so that functional rather than radiological classification is essential. Nor could classification be based upon a patients symptoms, as many with poor function did not complain of dyspnea and, on the other hand, some with good function did. Finally, the incidence of extrapulmonary sarcoid lesions did not differ in the two groups. I should like to comment first upon the course of the untreated patients. The mean follow-up period for the untreated cases was 2 yr and 8 months. TABLE 2 (left column) shows the status of patients whose function was initially only mildly impaired. As you see, there were 67 patients followed whose vital capacity was originally greater than 65% of predicted. Of these only 3 improved, most remained