title: Elevated pulmonary arterial systolic pressure in patients with sarcoidosis: prevalence and risk factors.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Outpatient Sarcoidosis Clinic, General Hospital of Chest Diseases Sotiria, Magrioti 7, Nea Smyrni, 17121, Athens, Greece.
authors.forename: Aggeliki
authors.initials: A
authors.lastname: Rapti
authors.forename: Vasileios
authors.initials: V
authors.lastname: Kouranos
authors.forename: Elias
authors.initials: E
authors.lastname: Gialafos
authors.forename: Konstantina
authors.initials: K
authors.lastname: Aggeli
authors.forename: John
authors.initials: J
authors.lastname: Moyssakis
authors.forename: Anastasios
authors.initials: A
authors.lastname: Kallianos
authors.forename: Charalampos
authors.initials: C
authors.lastname: Kostopoulos
authors.forename: Ourania
authors.initials: O
authors.lastname: Anagnostopoulou
authors.forename: Petros P
authors.initials: PP
authors.lastname: Sfikakis
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.forename: George E
authors.initials: GE
authors.lastname: Tzelepis
mesh_terms: D000328:Adult; D000368:Aged; D062186:Arterial Pressure / Q000502:physiology*; D015415:Biomarkers / Q000378:metabolism; D002248:Carbon Monoxide / Q000378:metabolism; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011653:Pulmonary Diffusing Capacity / Q000502:physiology; D011658:Pulmonary Fibrosis / Q000503:physiopathology; D012189:Retrospective Studies; D012307:Risk Factors; D017565:Sarcoidosis, Pulmonary / Q000453:epidemiology* / Q000503:physiopathology*; D012680:Sensitivity and Specificity; D013599:Systole / Q000502:physiology*; D018487:Ventricular Dysfunction, Left / Q000503:physiopathology
chemical_list: D015415:Biomarkers; D002248:Carbon Monoxide