Sarcoidosis-Associated Pulmonary Hypertension

Abstract

Sarcoidosis-associated pulmonary hypertension (SAPH) is found in 5 to 20% of sarcoidosis patients. Elevated pulmonary artery pressure may be due to multiple factors, including vasculocentric, parenchymal, and mechanical, as well as comorbidities such as cardiac sarcoidosis and sleep apnea. Most SAPH patients have fibrotic lung disease, but SAPH may be present in those without advanced parenchymal lung disease. Several features have been shown to suggest SAPH, including reduced DLCO, shortened 6-minute walk distance, with or without desaturation, and the presence of increased pulmonary artery to aorta ratio on CT scanning. Echocardiography remains an important tool for the evaluation of SAPH but may both over- or underestimate the severity of pulmonary artery pressure. Right heart catheterization remains the definitive test to make the diagnosis. There have been several reports on the value of different modalities of treating SAPH. These include prospective clinical trials and one double-blind placebo-controlled randomized trial. Evidence-based guidelines for treatment of SAPH are discussed in this review.

Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?

“…TLC/ DLCO > 1.67 was also the most specific indicator of PH diagnosed by echocardiography. The ability to predict PH due to ILD was documented both in sarcoidosis [39,40] and in IPF patients with decreased DLCO [3,21,22,41]. Lettieri et al found that DLCO < 40% pred.…”

Tattoo Koebnerization and Severe Pulmonary Hypertension

“…Sarcoidosis-associated pulmonary hypertension (SAPH) is an increasingly recognized cause of morbidity and mortality whose prevalence ranges from 5% to 20%. 6 It is categorized as WHO class 5 pulmonary hypertension, occurring through various mechanisms including intrinsic vasculopathy, mechanical obstruction of vasculature by lymphadenopathy, fibrotic destruction of vascular beds, and hypoxic vasoconstriction. 7 SAPH usually occurs in radiographically advanced cases, but vasculopathic SAPH does rarely present in radiologic stage 0, as in our patient.…”

“…7 SAPH usually occurs in radiographically advanced cases, but vasculopathic SAPH does rarely present in radiologic stage 0, as in our patient. 6 SAPH should be suspected when dyspnea exceeds the degree of radiographic abnormalities, and if respiratory symptoms are resistant to anti-inflammatory therapy. Echocardiography and 6-minute walk test are reasonable screening tools; however, right heart catheterization is the gold standard for diagnosis.…”

“…Echocardiography and 6-minute walk test are reasonable screening tools; however, right heart catheterization is the gold standard for diagnosis. 6 Unfortunately, the prognosis is grave, with a 5-year mortality of 45%. 8 While the patient's ethnicity, uveitis, interstitial lung disease, and skin biopsy findings support the diagnosis of SAPH, the degree of chest CT findings are not as extensive as one may expect, and the positive ANA is unusual.…”

Advanced Pulmonary Sarcoidosis

“…►Table 5 lists some of the more common factors associated with SAPH. 93 Since sarcoidosis can be caused by multiple factors, 94 it has been placed in World Health Organization (WHO) group V.…”