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2017 ; 50
(2
): ä Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Selexipag for the treatment of connective tissue disease-associated pulmonary
arterial hypertension
#MMPMID28818881
Gaine S
; Chin K
; Coghlan G
; Channick R
; Di Scala L
; Galiè N
; Ghofrani HA
; Lang IM
; McLaughlin V
; Preiss R
; Rubin LJ
; Simonneau G
; Sitbon O
; Tapson VF
; Hoeper MM
Eur Respir J
2017[Aug]; 50
(2
): ä PMID28818881
show ga
Patients with connective tissue disease-associated pulmonary arterial
hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The
underlying CTD can influence treatment response and outcomes. We characterised
the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334
patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in
170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82.
For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and
95% CI were calculated using Cox proportional hazard models.Compared with the
overall GRIPHON population, the CTD subgroup was slightly older with a greater
proportion of females and shorter time since diagnosis. Patients with PAH-SSc
appeared to be more impaired at baseline, with a more progressive disease course.
The converse was observed for PAH-SLE. Selexipag reduced the risk of composite
morbidity/mortality events in patients with PAH-CTD by 41% (HR 0.59; 95% CI
0.41-0.85). Treatment effect was consistent irrespective of baseline PAH therapy
or CTD subtype (interaction p=0.87 and 0.89, respectively). Adverse events were
predominately prostacyclin-related and known for selexipag treatment.GRIPHON has
allowed the comprehensive characterisation of patients with PAH-CTD. Selexipag
delayed progression of PAH and was well-tolerated among PAH-CTD patients,
including those with PAH-SSc and PAH-SLE.