(selexipag)
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UPTRAVI® (selexipag)
Medical Information
UPTRAVI - Treatment of Pulmonary Arterial Hypertension in Patients With Comorbid Interstitial Lung Disease
Last Updated: 08/06/2024
SUMMARY
- Company sponsored studies designed to investigate the safety and efficacy of UPTRAVI in the treatment of interstitial lung disease (ILD), or pulmonary hypertension associated with ILD have not been performed.
- A search of the internal database identified limited information from the pivotal GRIPHON (PGI2 Receptor agonist In Pulmonary arterial HypertensiON) study, which investigated the long-term efficacy, safety and tolerability of UPTRAVI in adult patients with symptomatic pulmonary arterial hypertension (PAH).1
Of the 1156 patients enrolled, 16 (2.8%) in the UPTRAVI group and 22 (3.8%) in the placebo group had ILD listed as a previous or concomitant disease at baseline.2 Of note, patients with moderate or severe restrictive lung disease were excluded from enrollment.1, 3 ,4 Subgroup analysis designed to determine overall efficacy and/or safety outcomes in this specific patient population was not undertaken. - An analysis of data from the United States (US)-based SPHERE (SelexiPag: tHe usErs dRug rEgistry) registry (Data cut: December 20, 2019; N=500), an observational study designed to provide information on the disease characteristics, clinical outcomes, and dosing regimens of patients treated with UPTRAVI in routine clinical practice, revealed that 60 (12%) of patients had interstitial lung disease. However, details concerning outcomes of patients with comorbid ILD in the SPHERE registry are not available.5
- A search of the scientific literature retrieved 3 case reports6
- 8 , published as congress abstracts, describing the administration of UPTRAVI to treat PAH in patients with comorbid ILD.
CLINICAL DATA
Information From GRIPHON
The total number of patients in GRIPHON with a previous or concomitant disease at baseline denoting ILD was 2.8% (n=16) in the UPTRAVI group and 3.8% (n=22) in the placebo group.2
Of note, patients with moderate or severe restrictive lung disease, defined as a total lung capacity <60% of predicted, were excluded from enrollment.1,3,4
A subgroup analysis designed to determine overall efficacy and/or safety outcomes in this specific patient population with previous or concomitant ILD was not undertaken.2
Information From SPHERE
SPHERE was a US-based, multi-center, prospective, observational drug registry that enrolled patients actively treated with UPTRAVI, which was designed to provide information on the disease characteristics, clinical outcomes, and dosing regimens of patients treated with UPTRAVI in routine clinical practice.5
At an interim data analysis (data cut: December 20, 2019; N=500), 60 (12%) of patients had comorbid interstitial lung disease.5
Details concerning outcomes in these patients with comorbid ILD in the SPHERE registry are not available.
Overview of Identified Information From Other Studies
| Reference and Study Design | Patients | UPTRAVI Treatment | Efficacy Outcomes | Safety Outcomes |
|---|---|---|---|---|
| Ahluwalia et al (2020)6 [congress abstract] | 52-year-old female with connective tissue disease-associated interstitial lung disease (CTDILD), Overlap Syndrome and myeloperoxidase (MPO) positive vasculitis who developed pulmonary hypertension group I disease and was started on UPTRAVI | Dose information not reported | Not reported | Not reported |
| Wittner et al (2019)7 | 58-year-old female History of PSS and COP. Presented with breathlessness, reduced exercise tolerance, dilated right ventricle with impaired systolic function, severe tricuspid regurgitation and PASP=81 mmHg Treated with IV epoprostenol, dopamine and furosemide. | Discharged on oral UPTRAVI, tadalafil, macitentan, furosemide and spironolactone. Dose information not reported. | Post discharge exercise tolerance improved, symptoms remained less severe than prior to her hospital admission, although worse than they had been as an inpatient on IV therapy. | Not reported |
| Clegg and Bensimhon (2017)8 [congress abstract] | 42-year-old female History of SSc complicated by esophageal dysmotility, ILD, chronic respiratory failure and PAH Presented with RV failure, marked hypoxemia and WHO FC III-IV symptoms Started on supplemental oxygen Referred for LT Treated with successive additions of PAH therapies (bosentan-sildenafil- UPTRAVI). | Added to existing bosentan-sildenafil combination following persistence of mild-to-moderate PAH with RV strain Up-titrated to 1600 mcg BID over 4 months | Several months after commencement of triple combination therapy:
The patient was subsequently delisted for LT and was reported to be well. | Not reported |
| Abbreviations: 6MWD, 6-minute walk distance; BID, twice daily; CO, cardiac output; COP, cryptogenic organizing pneumonia; ECG, echocardiogram; FC, functional class; ILD, interstitial lung disease; IV, intravenous; LT, lung transplant; PAH, pulmonary arterial hypertension; PASP, pulmonary arterial systolic pressure; PSS, primary Sjogren’s syndrome; PVR, pulmonary vascular resistance; RHC, right heart catheterization; RV, right ventricular; SSc, systemic sclerosis. | ||||
Literature Search
A literature search of MEDLINE®
References
| 1 | Sitbon O, Channick R, Chin K, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373(26):2522-2533. |
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