BACKGROUND: Sarcoidosis-related pulmonary hypertension (SRPH) is an entity associated with significant morbidity and mortality irrespective of disease severity, while the pathogenic mechanisms remain poorly understood. METHODS: This cross-sectional study included consecutive patients with biopsy-proven sarcoidosis (n = 313) who were followed up in an outpatient setting from October 2002 through June 2010. All patients underwent clinical and cardiopulmonary evaluation, including cardiac MRI, to assess prevalence of SRPH and identify possible underlying pathophysiological mechanisms. RESULTS: By Doppler echocardiographic criteria, 37 (11.8 %) patients were found to have pulmonary arterial systolic pressure (PASP) >40 mmHg. Twelve of the 37 patients agreed to undergo right heart catheterization and SRPH was confirmed in nine patients. Compared to patients without SRPH, those with SRPH were significantly older and had greater lung function impairment; disease duration did not differ between patients with and without SRPH. Multiple logistic regression analysis showed that diffusing capacity for carbon monoxide (DLCO) and age were independent determinants of SRPH. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidities accounted for SRPH in the majority of patients. In the nonpulmonary fibrosis group, DLCO ≤ 50.65 (% predicted) was associated with SRPH (sensitivity = 77.8 %, specificity = 72.2 %; p = 0.031, AUC = 0.759). CONCLUSION: In a large cohort of sarcoidosis patients, this study found a prevalence of SRPH of about 12 %. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidities are frequent pathogenic mechanisms.
BACKGROUND:Sarcoidosis-related pulmonary hypertension (SRPH) is an entity associated with significant morbidity and mortality irrespective of disease severity, while the pathogenic mechanisms remain poorly understood. METHODS: This cross-sectional study included consecutive patients with biopsy-proven sarcoidosis (n = 313) who were followed up in an outpatient setting from October 2002 through June 2010. All patients underwent clinical and cardiopulmonary evaluation, including cardiac MRI, to assess prevalence of SRPH and identify possible underlying pathophysiological mechanisms. RESULTS: By Doppler echocardiographic criteria, 37 (11.8 %) patients were found to have pulmonary arterial systolic pressure (PASP) >40 mmHg. Twelve of the 37 patients agreed to undergo right heart catheterization and SRPH was confirmed in nine patients. Compared to patients without SRPH, those with SRPH were significantly older and had greater lung function impairment; disease duration did not differ between patients with and without SRPH. Multiple logistic regression analysis showed that diffusing capacity for carbon monoxide (DLCO) and age were independent determinants of SRPH. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidities accounted for SRPH in the majority of patients. In the nonpulmonary fibrosis group, DLCO ≤ 50.65 (% predicted) was associated with SRPH (sensitivity = 77.8 %, specificity = 72.2 %; p = 0.031, AUC = 0.759). CONCLUSION: In a large cohort of sarcoidosispatients, this study found a prevalence of SRPH of about 12 %. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidities are frequent pathogenic mechanisms.
Authors: N B Schiller; P M Shah; M Crawford; A DeMaria; R Devereux; H Feigenbaum; H Gutgesell; N Reichek; D Sahn; I Schnittger Journal: J Am Soc Echocardiogr Date: 1989 Sep-Oct Impact factor: 5.251
Authors: R P Baughman; M A Judson; E E Lower; K Highland; S Kwon; N Craft; P J Engel Journal: Sarcoidosis Vasc Diffuse Lung Dis Date: 2009-07 Impact factor: 0.670
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Authors: Allan Lawrie; Neil Hamilton; Steven Wood; Fernando Exposto; Ruvimbo Muzwidzwa; Louise Raiteri; Amélie Beaudet; Audrey Muller; Rafael Sauter; Nadia Pillai; David G Kiely Journal: Pulm Circ Date: 2022-09-30 Impact factor: 2.886