TY - JOUR
T1 - Pulmonary arterial hypertension associated with connective tissue diseases
T2 - A review focusing on distinctive clinical aspects
AU - Kato, Masaru
AU - Atsumi, Tatsuya
N1 - Publisher Copyright:
© 2017 Stichting European Society for Clinical Investigation Journal Foundation
PY - 2018/2
Y1 - 2018/2
N2 - 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.
AB - 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.
KW - connective tissue diseases
KW - pulmonary arterial hypertension
KW - pulmonary hypertension
KW - systemic lupus erythematosus
KW - systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85039151908&partnerID=8YFLogxK
U2 - 10.1111/eci.12876
DO - 10.1111/eci.12876
M3 - 総説
C2 - 29285766
AN - SCOPUS:85039151908
SN - 0014-2972
VL - 48
JO - European Journal of Clinical Investigation
JF - European Journal of Clinical Investigation
IS - 2
M1 - e12876
ER -