Pulmonary Hypertension Caused by Sarcoidosis
Section snippets
History
The modern history of sarcoidosis probably began in 1898, when Hutchinson [11], a London dermatologist, described a case of a patient with “Mortimer's malady,” allegedly a patient with evidence of cutaneous disease. In the initial description, the disease was thought to be a form of cutaneous lupus. Sarcoidosis was recognized as a distinct pathologic entity a year later, when Boeck [12] described the histology of the “sarkoid” granulomas. By 1929, Bernstein [13] had described cardiopulmonary
Epidemiology
The actual prevalence of SAPH has not been established. In patients with significant parenchymal lung disease, the disease was historically thought to be rare, with involvement of the pulmonary vasculature in less than 5% of the cases. Unfortunately, most of the early cohorts were identified retrospectively based on autopsies or case series of patients with clinically diagnosed right heart failure. For example, Mayock and colleagues [20] performed a review of 145 patients with sarcoidosis from
Pathophysiology
The World Health Organization (WHO) currently classifies pulmonary hypertension into five groups, with sarcoidosis included as Class V (miscellaneous) [32]. Compared with idiopathic pulmonary fibrosis, where pulmonary hypertension is probably caused by fibrotic destruction of the distal capillary bed, several mechanisms may be relevant in SAPH. Thus, it is not incorrect to state that the mechanisms for SAPH could be grouped into all five WHO categories [33].
The granulomatous inflammation in
Clinical presentation
Pulmonary hypertension should be suspected in any sarcoidosis patient with dyspnea, hypoxemia, or clinical evidence suggesting right heart failure, particularly if symptoms appear to be out of proportion to the degree of parenchymal lung disease. Unfortunately, these symptoms are often present in patients without PH. Other considerations when patients present with unexplained dyspnea or exercise limitation include myopathy (skeletal or respiratory muscle), large airway obstruction, occult
Management
The optimal management strategy for pulmonary hypertension associated with sarcoidosis is unknown. Treatment has typically involved use of systemic anti-inflammatory medications, anticoagulants, pulmonary vasodilators, endothelin receptor antagonists, and supplemental oxygen. The available literature regarding these therapeutic strategies is limited to small cohort studies, retrospective analyses, and case reports.
Prognosis
Sarcoidosis is generally considered a disease with a favorable long-term prognosis. Although the disease remits spontaneously in nearly two-thirds of patients, about 1% to 5% of patients die from progressive respiratory failure, central nervous system disease, or myocardial involvement [8], [91], [92]. Right ventricular failure has been described in up to 30% of sarcoidosis-related deaths [20], [93]. Patients with substantial pulmonary fibrosis have a particularly elevated risk. A single-center
Summary
The authors conclude that SAPH may be present irrespective of the degree of parenchymal involvement. It may account for a substantial proportion of dyspnea among all sarcoidosis patients, and appears to be common in those with severe parenchymal disease. A major gap in current clinical practice is identification of a cost-effective, reliable, noninvasive method to screen for the presence of SAPH. Further studies are needed to characterize the natural history more closely, to establish whether
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Cited by (0)
D.A. Culver's work is supported by NIH grant HL081538, from the National Heart Lung and Blood Institute.
D.A. Culver and J. Parambil are site investigators on an investigator-sponsored trial (Actelion).