Corticosteroids

Pulmonary hypertension is a rare presenting manifesta­tion of sarcoidosis. While it has been proposed that the pulmonary vascular disease associated with sarcoidosis may be amenable to corticosteroid therapy, there is only one published report of resolution of pulmonary hypertension in a patient with sarcoidosis. The present case is unusual both in its initial mode of presentation as concomitant severe pulmonary hypertension and biventricular cardiomyopathy, and in the documentation of successful therapy and sustained remission of pulmonary hypertension with corticosteroid treatment.

Case Report

A 30-year-old black woman was referred for evaluation of dyspnea and leg edema. At the time of her initial presentation, physical findings of biventricular congestive heart failure and chest roent­genogram findings of left ventricular failure were appreciated (Fig 1). Her past, family, occupational, and exposure histories were unremarkable, and she had been taking no medications. Treatment with diuretics, digoxin, angiotensin converting-enzyme inhibitor, and anticoagulant was initiated.


Figure 1 . Chest roentgenogram

FIGURE 1. Chest roentgenogram, a, (left) on presentation showing cardiomegally, alveolar and interstitial infiltrates, bilateral pleural effusions and bilateral hilar adenopathy, and b, (right) after four months of oral prednisone therapy showing decreased cardiac sillouette, and partial resolution of alveolar and intersitial infiltrates.

One year after her initial presentation, she was referred for eval­uation of worsening dyspnea and edema. Review of her chest roentgenogram suggested the possibility of hilar adenopathy (Fig 1). At that time, pulmonary function studies revealed a restrictive defect and decreased diffusing capacity (Table 1), gallium lung scan revealed bilateral hilar uptake, and ventilation-perfusion lung scan revealed matched subsegmental defects, not suggestive of pulmonary emboli. Angiotensin converting enzyme level was 20 IU (normal 10 to 30). Electrocardiogram showed sinus rhythm with frequent premature ventricular contractions and right axis devia­tion. A MUGA scan showed a left ventricular ejection fraction (LVEF) of 36 percent, with evidence of diffuse left ventricular hypokinesis and severe right ventricular hypokinesia Cardiac ultra­sound confirmed biventricular hypokinesis as well as biventricular hypertrophy and right ventricular dilatation. Right heart catheter­ization revealed a normal pulmonary capillary wedge pressure and severe pulmonary hypertension (Table 1) which did not improve with supplemental oxygen. Endomyocardial biopsy of the septum revealed focal myocardial necrosis, and endobronchial biopsy re­vealed multiple submucosal non-caseating granulomata (Fig 2). Fungal and myocobacterial stains and cultures were negative.
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FIGURE 2. Endobronchial biopsy showing submucosal non-caseating granulomata (hematoxylin-eosin, original magnification x 1000).

Therapy with supplemental oxygen and intensive diuresis were initiated. Despite a 13.6 kg (30-pound) weight loss over a 14-day period, there was no clearing of interstitial and alveolar infiltrates on chest roentgenogram, and no improvement in her dyspnea. Oral prednisone, 60 mg per day, was started. Symptomatic improvement ensued within six weeks. Chest roentgenogram obtained four months after initiation of therapy showed a marked decrease in cardiac size and clearing of interstitial infiltrates (Fig 1). Right-heart catheterization was repeated at that time and demonstrated im­proved (although still abnormal) pulmonary hemodynamics (Table 1). Over the subsequent three months, prednisone was tapered to 20 mg every other day, and discontinued after 18 months. Supple­mental oxygen was discontinued after two years. Pulmonary func­tion studies have demonstrated maintenance of improvement for the three-year period of observation, including the final 12 months during which she has received neither prednisone nor supplemental oxygen therapy (Table 1).
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Table 1—Pulmonary Function and Hemodynamic Studies Prior to Therapy, After Four Months of Prednisone Therapy and Six and 18 Months After Discontinuation of Therapy (Two and Three Years After Presentation)

Baseline

4 Months

2 Years

3 Years

FVC (L)

1.30

2.57

2.22

3.38

Deo (ml/mmHg/min)

9

20

23

22

02 Saturation (%)

77

93

92

92

Po2 (mm Hg)

36

66

Pco2 (mm Hg)

36

36

pH

7.46

7.45

LVEF (%)

36

36

PAP (mm Hg)

64/32

43/18

PA (mm Hg)

42

27

PCW (mm Hg)

9

6

CO (L/min)

4.9

4.3

TPR (dynes-s-cm 5)

686

502

PVR (dynes-s-cm 5)

555

391