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Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects
Author(s) -
Kato Masaru,
Atsumi Tatsuya
Publication year - 2018
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/eci.12876
Subject(s) - medicine , pulmonary hypertension , connective tissue , connective tissue disease , immunosuppression , pulmonary arterial pressure , interstitial lung disease , cardiology , ctd , intensive care medicine , helpfulness , lung , pathology , disease , psychology , autoimmune disease , social psychology , oceanography , geology
Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases ( CTD ‐ PAH ) has some distinctive clinical aspects from other PAH , such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre‐ PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD ‐ PAH and garner attention to areas where further evidence is desired.