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PAH associated with connective tissue disease (PAH-CTD)

PAH: A potential complication of CTD affecting up to approximately 1 in 10 patients1

PAH is a well-known complication in some patients with CTD2:

  • PAH affects approximately 3% to 13% of patients with CTD and 5% to 12% of patients with systemic sclerosis (SSc)1,3
  • Patients with PAH-SSc have a worse prognosis than most other PAH-associated disease populations4

Exploratory subgroup analysis: Disease progression in patients with PAH-CTD

Overall, in SERAPHIN, OPSUMIT® (macitentan) reduced the risk of disease progression by 45% vs placebo (HR 0.55; 97.5% CI, 0.39-0.76; P<0.0001).5 In the SERAPHIN trial, 31% of the overall patient population had PAH-CTD.5

The information below represents an exploratory subgroup analysis. Results should be interpreted with caution.

POST HOC ANALYSIS: Time to first disease progression event in patients with PAH-CTD6,7

Exploratory subgroup analysis: Time to first disease progression event in patients with PAH-CTD

Summary of primary endpoint events in patients with PAH-CTD6

  OPSUMIT® 10 mg
(n=73), n (%)		 Placebo
(n=82), n (%)
Patients with a primary endpoint event* 20 (27.4) 31 (37.8)
Component as first event
Worsening PAH 16 (21.9) 25 (30.5)
Death 3 (4.1) 5 (6.1)
Initiation of IV/SC prostanoids 1 (1.4) 1 (1.2)

*No patients experienced an event of lung transplantation or atrial septostomy in the placebo or OPSUMIT® 10 mg treatment groups.


Differences in PAH-CTD subgroup baseline characteristics compared with SERAPHIN overall population6,8

  • Larger percentage of female patients (92% vs 76.5%)
  • Older average age (49.7 years vs 45.6 years)
  • Larger percentage of patients with WHO FC II symptoms (58.5% vs 52.4%)
  • Lower percentage of patients with WHO FC Ill symptoms (39.3% vs 45.6%)
  • Shorter time from diagnosis (mean 2.0 years vs 2.7 years)

Adverse reactions in the PAH-CTD subgroup different from the overall population6†

  OPSUMIT® 10 mg
n=73		 Placebo
n=82
Upper respiratory tract infection 23.3% 13.4%
Urinary tract infection 12.3% 6.1%
Skin ulcer 9.6% 3.7%
Sinusitis 6.8% 1.2%
Lower respiratory tract infection 5.5% 1.2%
Nausea 8.2% 4.9%

†Only adverse reactions occurring ≥3% on OPSUMIT® compared with placebo and placebo-corrected difference of ≥3% in the PAH-CTD subgroup vs the overall population are shown here.

Eight (9.8%) patients receiving placebo and 8 (11.0%) patients receiving OPSUMIT® 10 mg in the PAH-CTD subgroup discontinued treatment due to adverse reactions, compared with approximately 11% for both the placebo and OPSUMIT® in the overall population.6,8

Exploratory subgroup analysis: PAH-related hospitalization

Overall, in SERAPHIN, OPSUMIT® reduced the risk of PAH-related hospitalization by 50% vs placebo (HR 0.50; 97.5% CI, 0.34-0.75; P<0.0001).5

The information below represents an exploratory subgroup analysis. Results should be interpreted with caution.

POST HOC ANALYSIS: Time to PAH-related death or hospitalization in patients with PAH-CTD6,9

Exploratory subgroup analysis: Time to PAH-related death or hospitalization in patients with PAH-CTD

PAH-CTD Subgroup: Summary of death due to PAH and hospitalization due to PAH6

  OPSUMIT® 10 mg
(n=73), n (%)		 Placebo
(n=82), n (%)
Death due to PAH or hospitalization for PAH 13 (17.8) 22 (26.8)
Component as first event
Death due to PAH 1 (1.4) 1 (1.2)
Hospitalization for PAH 12 (16.4) 21 (25.6)

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CI=confidence interval; CTD=connective tissue disease; FC=Functional Class; HR=hazard ratio; IV=intravenous; PAH=pulmonary arterial hypertension; PAH-SSc=PAH associated with systemic sclerosis; SC=subcutaneous; SERAPHIN=Study with an Endothelin Receptor Antagonist in Pulmonary Arterial Hypertension to Improve CliNical Outcome; WHO=World Health Organization.
References: 1. Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138:1383-1394. 2. Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65:3194-3201. 3. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022;43(38):3618-3731. 4. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D34-D41. 5. OPSUMIT® [prescribing information]. Actelion Pharmaceuticals US, Inc. 6. Data on file. Actelion Pharmaceuticals US, Inc. 7. Data on file. Actelion Pharmaceuticals US, Inc. 8. Pulido T, Adzerikho I, Channick RN, et al; SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809-818 and suppl 1-21. doi:10.1056/NEJMoa1213917 9. Data on file. Actelion Pharmaceuticals US, Inc.

To report suspected adverse reactions, contact Janssen at 1-800-JANSSEN (1-800-526-7736), or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

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