Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience.
BACKGROUND
Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined.


METHODS
We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed.


RESULTS
Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n = 18) increased by 59 m (p = 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 +/- 4.3 to 39.4 +/- 2.8 mm Hg (p = 0.008). The 1- and 3-year transplant-free survival rates were 90% and 74%, respectively.


CONCLUSIONS
PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.
Barnett
Christopher F
CF
Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD. Electronic address: cbarnett@ucsd.edu.
Bonura
Eric J
EJ
Department of Internal Medicine, Georgetown University, Washington, DC.
Nathan
Steven D
SD
Inova Fairfax Hospital, Falls Church, VA.
Ahmad
Shahzad
S
Inova Fairfax Hospital, Falls Church, VA.
Shlobin
Oksana A
OA
Inova Fairfax Hospital, Falls Church, VA.
Osei
Kwabena
K
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
Zaiman
Ari L
AL
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
Hassoun
Paul M
PM
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
Moller
David R
DR
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
Barnett
Scott D
SD
Inova Fairfax Hospital, Falls Church, VA.
Girgis
Reda E
RE
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD.
D000280:Administration, Inhalation; D000284:Administration, Oral; D000368:Aged; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D015331:Cohort Studies; D004359:Drug Therapy, Combination; D011464:Epoprostenol / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics / Q000187:drug effects* / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000401:mortality; D016285:Iloprost / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000008:administration & dosage; D011336:Probability; D011687:Purines / Q000008:administration & dosage; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D018570:Risk Assessment; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D018709:Statistics, Nonparametric; D013449:Sulfonamides / Q000008:administration & dosage; D013450:Sulfones / Q000008:administration & dosage; D015996:Survival Rate; D016896:Treatment Outcome
D000959:Antihypertensive Agents; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D016285:Iloprost; D000077300:Bosentan