Sex Disparities In Survival Of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension Patients

Christopher Pasarikovski¹, John T. Granton², Peter Lee³, Adrienne M. Roos¹, Amie T. Kron¹, Cathy Chau⁴ and Sindhu R. Johnson⁵, ¹Medicine, Toronto Scleroderma Research Program, Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada, ²Medicine, Toronto Pulmonary Hypertension Programme, Toronto General Hospital and University of Toronto, Toronto, ON, Canada, ³Lebovic Bldg, Mt. Sinai Hospital, Toronto, ON, Canada, ⁴Medicine, Toronto Scleroderma Program, Toronto Western Hospital, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Medicine, Toronto Western Hospital, Toronto General Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: scleroderma and systemic sclerosis

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Systemic sclerosis (SSc) associated pulmonary arterial hypertension (PAH) and idiopathic PAH (IPAH) are conditions with poor survival. There is evidence to suggest that sex affects survival. The primary objective of this study was to evaluate the effect of sex on survival in SSc-PAH and IPAH. We secondarily evaluated the effect of sex on disease onset, time to diagnosis, disease progression and treatment.

Methods:

Patients were included if they attended the Toronto Scleroderma Program or the University Health Network Pulmonary Hypertension Program; had a diagnosis of SSc-PAH or IPAH defined as a mean pulmonary artery pressure >25mmHg and age > 16 years. Sex was defined as self-reported biological and physiological characteristics at birth (male, female). The primary outcome was the time from diagnosis to death from all causes. Secondary outcomes were sex differences in age of diagnosis, disease duration and SSc manifestations. Cox proportional hazards model were used to evaluate survival.

Results:

52 male and 267 female SScPAH; and 47 male and 107 female IPAH patients were identified. Male SSc patients had a shorter mean (standard deviation) time from SSc diagnosis to PAH diagnosis (5.6 (8.7)) versus (8.4 (9.6)), p=0.047), increased frequency of renal crisis (19% versus 9%, p= 0.04), interstitial lung disease (67% versus 49%, p=0.02), and digital ulcers (29% versus 19%, p<0.001). Male IPAH patients had a higher frequency of diabetes (30% versus 12%). Despite adjusting for these differences, male SScPAH patients have decreased 1-, 2-, 3-, and 5-year survival (82.6%, 70.6%, 60.8%, 48.2%) compared to females (84.4%, 73.4%, 64.2%, 52.8%). Similarly, male IPAH patients have decreased 1-, 2-, 3-, and 5-year survival (93.4%, 87.9%, 84.8%, 77.7%) compared to females (94.5%, 91.0%, 88.7%, 83.2%).

Conclusion:

Sex disparities appear to exist in survival of SSc-PAH and IPAH patients. Further investigation is needed to evaluate this disparity, mechanisms for disparity, and the role of a targeted screening and treatment approach.

Disclosure:

C. Pasarikovski,
None;

J. T. Granton,

Support respirology program at the hospital foundation.,

Pfizer support of research study via CIHR grant.,

P. Lee,
None;

A. M. Roos,
None;

A. T. Kron,
None;

C. Chau,
None;

S. R. Johnson,
None.

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