Sarcoidosis associated pulmonary hypertension: an update.
Curr Opin Pulm Med
; 27(5): 285-295, 2021 09 01.
Article
em En
| MEDLINE
| ID: mdl-34127623
ABSTRACT
PURPOSE OF REVIEW Sarcoidosis associated pulmonary hypertension (SAPH) is a well-recognised complication, associated with a seven-fold increase in mortality. This comprehensive review will summarise these recent developments and proposes the use of a phenotype-based management approach in SAPH. RECENT FINDINGS:
Recent registry-based studies have highlighted the adverse outcomes associated with SAPH and shown that reduced 6-min walk distance and diffusion capacity for carbon monoxide are predictive of poor prognosis. There is increasing interest in methods for early detection of SAPH, although whether early diagnosis impacts on survival remains uncertain. The pathophysiology underpinning SAPH is complex and often incorporates multiple mechanisms. Once the diagnosis is confirmed, understanding the underlying phenotypes of SAPH is key to providing the most effective management plan. There is some evidence that treating patients with precapillary PH with pulmonary vasodilators may improve some haemodynamic and quality life measures. However, more work is needed to determine whether mortality is affected.SUMMARY:
SAPH is associated with worsened survival. A range of phenotypes are recognised in SAPH. Multimodality risk assessment in patients with SAPH is likely to be important and is an area that requires further work. Published evidence for pulmonary vasodilator therapies in SAPH with a Pulmonary arterial hypertension-like phenotype is encouraging so far, but multiple confounding factors affects the quality of the evidence. The role of immunosuppressive agents for improving pulmonary pressures is unclear. Urgent controlled trials are needed.
Texto completo:
1
Coleções:
01-internacional
Contexto em Saúde:
6_ODS3_enfermedades_notrasmisibles
Base de dados:
MEDLINE
Assunto principal:
Sarcoidose
/
Sarcoidose Pulmonar
/
Hipertensão Arterial Pulmonar
/
Hipertensão Pulmonar
Tipo de estudo:
Etiology_studies
/
Prognostic_studies
/
Screening_studies
Limite:
Humans
Idioma:
En
Revista:
Curr Opin Pulm Med
Ano de publicação:
2021
Tipo de documento:
Article