An update on sarcoidosis-associated pulmonary hypertension.
Endothelin Receptor Antagonists
/ therapeutic use
Glucocorticoids
/ therapeutic use
Humans
Hypertension, Pulmonary
/ diagnosis
Immunosuppressive Agents
/ therapeutic use
Lung
/ physiopathology
Prognosis
Pulmonary Arterial Hypertension
/ diagnosis
Pulmonary Artery
/ physiopathology
Pyrimidines
/ therapeutic use
Retrospective Studies
Sarcoidosis
/ complications
Sarcoidosis, Pulmonary
/ complications
Sulfonamides
/ therapeutic use
Vasodilator Agents
/ therapeutic use
Journal
Current opinion in pulmonary medicine
ISSN: 1531-6971
Titre abrégé: Curr Opin Pulm Med
Pays: United States
ID NLM: 9503765
Informations de publication
Date de publication:
09 2020
09 2020
Historique:
pubmed:
3
8
2020
medline:
25
6
2021
entrez:
3
8
2020
Statut:
ppublish
Résumé
Pulmonary hypertension in sarcoidosis is a well known entity. Sarcoidosis-associated pulmonary hypertension (SAPH) incurs substantial morbidity and mortality. This review examines recent literatures published on epidemiology, prognosis and therapeutic management in SAPH. Several registries have been published between 2017 and 2020. The consensus conclusion - SAPH is a harbinger for poor prognosis. Several factors were noted for predicting adverse outcome in SAPH like reduced 6-min walk distance and diffusing capacity for carbon monoxide. Given its adverse outcome, experts have now focused on methods for early screening of SAPH in sarcoid patients. The exploration of pulmonary vasodilator drugs in SAPH is ongoing. In recent times, trials have been published utilizing Macitentan and parenteral prostacyclin in severe SAPH. Although these trials show encouraging results, the evidence from these studies are limited to approve these agents as preferred drugs for treating SAPH. A large multicentric trial of drugs used for pulmonary arterial hypertension with meaningful, yet feasible, event driven endpoint is still lacking. Lately, interventional treatment by pulmonary artery balloon pulmonary angioplasty and stenting has gained traction for treating pulmonary artery stenosis and chronic thromboembolic pulmonary hypertension. However, the conclusion is still based on small cohorts or case series. Several registries have highlighted SAPH portends an unfavorable consequence. On the contrary, no published guideline exists to treat SAPH. The precise role of immunosuppressive agents is unclear. The limited evidence favoring use of pulmonary vasodilators arise from small retrospective case series and/or single-center nonrandomized observational studies. Further multicenter randomized research is warranted to better define patient population to treat and how best to treat them.
Identifiants
pubmed: 32740377
doi: 10.1097/MCP.0000000000000701
pii: 00063198-202009000-00029
doi:
Substances chimiques
Endothelin Receptor Antagonists
0
Glucocorticoids
0
Immunosuppressive Agents
0
Pyrimidines
0
Sulfonamides
0
Vasodilator Agents
0
macitentan
Z9K9Y9WMVL
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
582-590Références
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