Successful Treatment of Sarcoidosis-Associated Pulmonary Hypertension with Corticosteroids: DISCUSSION

Pulmonary hypertension is an unusual manifestation of sarcoidosis, most often presenting as a sequela of diffuse fibrosis. However, several vascular lesions have been reported to cause pulmonary hypertension as an early, primary manifestation of sarcoidosis, including extrinsic compression of large pulmonary arteries, granulomatous infiltration of large and small pulmonary arteries and veins, and vasoreactive pulmonary hypertension. Most of the reports of pulmonary hypertension due to sarcoidosis have described a rapidly fatal outcome despite oxygen and corticosteroid therapy.

The English language literature contains one other report of successful therapy of sarcoidosis-associated pulmonary hypertension with oral corticosteroid. In both that case and the present one, decreased pulmonary vascular resistance and pulmonary artery pressure, improved gas exchange, and improved pulmonary mechanics were documented to occur within 16 weeks of the initiation of therapy. In the earlier report, corticosteroid therapy was discontinued after eight months, while in the present case, therapy with every-other-day corticosteroids was continued for an addi­tional eight months. In both cases, the remission was well maintained after discontinuation of therapy. These reports suggest that in cases of sarcoidosis-associated pulmonary hypertension, aggressive corticosteroid therapy can have a beneficial effect on pulmonary hemodynamics and that long- term remission can be achieved. It is also possible that in both cases, supplemental oxygen therapy aided in the resolution of the pulmonary hypertension. However, the lack of an acute salutary effect of oxygen on pulmonary hemodynamics and the reported rapidly fatal outcome in such patients treated with supplemental oxygen alone sug­gests that the major therapeutic intervention in these cases was oral corticosteroid.

In addition to severe pulmonary hypertension, our patient presented with biventricular cardiomyopathy. Myocardial involvement is a well recognized manifestation of sarcoidosis, present clinically in up to 5 percent of cases and at necroscopy in an additional 20 to 25 percent of cases. While this patient presented with signs of both left and right sided heart failure, the right sided failure was much more severe, presumably due to the combination of under­lying cardiomyopathy and pulmonary hypertension. Since therapy with corticosteroids, this patients LVEF has been unchanged, while her heart failure has been easily controlled with digoxin and small doses of furosemide. This clinical improvement is likely due to resolution of right ventricular dysfunction secondary to pulmonary hypertension. suhagra 100

In summary, this case supplements one previous report that severe sarcoidosis-associated pulmonary hypertension can be reversed and remain quiescent with corticosteroid therapy. Clinical response to therapy occurred within six weeks, was sustained during an additional 18 months of therapy with administration of every-day and every-other- day corticosteroids and during an 18-month period of observation after therapy was discontinued. We recommend that in cases of sarcoidosis presenting with signs of significant pulmonary hypertension, pulmonary hemodynamics be measured and used as an index of severity of disease and followed as an indicator of therapeutic efficacy.


Category: Disease

Tags: Corticosteroids, hypertension

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