Sex differences in pulmonary arterial hypertension

Role of infection and autoimmunity in the pathogenesis of disease

Kyle A. Batton, Christopher O. Austin, Katelyn A. Bruno, Charles Dwayne Burger, Brian P Shapiro, DeLisa Fairweather

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

Registry data worldwide indicate an overall female predominance for pulmonary arterial hypertension (PAH) of 2-4 over men. Genetic predisposition accounts for only 1-5% of PAH cases, while autoimmune diseases and infections are closely linked to PAH. Idiopathic PAH may include patients with undiagnosed autoimmune diseases based on the relatively high presence of autoantibodies in this group. The two largest PAH registries to date report a sex ratio for autoimmune connective tissue disease-associated PAH of 9:1 female to male, highlighting the need for future studies to analyze subgroup data according to sex. Autoimmune diseases that have been associated with PAH include female-dominant systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, and thyroiditis as well as male-dominant autoimmune diseases like myocarditis which has been linked to HIV-associated PAH. The sex-specific association of PAH to certain infections and autoimmune diseases suggests that sex hormones and inflammation may play an important role in driving the pathogenesis of disease. However, there is a paucity of data on sex differences in inflammation in PAH, and more research is needed to better understand the pathogenesis underlying PAH in men and women. This review uses data on sex differences in PAH and PAH-associated autoimmune diseases from registries to provide insight into the pathogenesis of disease.

Original languageEnglish (US)
Article number15
JournalBiology of Sex Differences
Volume9
Issue number1
DOIs
StatePublished - Apr 18 2018

Fingerprint

hypertension
Autoimmunity
Pulmonary Hypertension
Sex Characteristics
Disease
Infection
Autoimmune Diseases
Registries
Inflammation
Thyroiditis
Connective Tissue Diseases
Systemic Scleroderma
Sex Ratio
Myocarditis
sex ratio
Gonadal Steroid Hormones
Genetic Predisposition to Disease
Systemic Lupus Erythematosus
Autoantibodies
Rheumatoid Arthritis

Keywords

  • Autoimmune disease
  • Inflammation
  • Myocarditis
  • Pulmonary arterial hypertension
  • Sex differences
  • Systemic sclerosis

ASJC Scopus subject areas

  • Gender Studies
  • Endocrinology

Cite this

Sex differences in pulmonary arterial hypertension : Role of infection and autoimmunity in the pathogenesis of disease. / Batton, Kyle A.; Austin, Christopher O.; Bruno, Katelyn A.; Burger, Charles Dwayne; Shapiro, Brian P; Fairweather, DeLisa.

In: Biology of Sex Differences, Vol. 9, No. 1, 15, 18.04.2018.

Research output: Contribution to journalReview article

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abstract = "Registry data worldwide indicate an overall female predominance for pulmonary arterial hypertension (PAH) of 2-4 over men. Genetic predisposition accounts for only 1-5{\%} of PAH cases, while autoimmune diseases and infections are closely linked to PAH. Idiopathic PAH may include patients with undiagnosed autoimmune diseases based on the relatively high presence of autoantibodies in this group. The two largest PAH registries to date report a sex ratio for autoimmune connective tissue disease-associated PAH of 9:1 female to male, highlighting the need for future studies to analyze subgroup data according to sex. Autoimmune diseases that have been associated with PAH include female-dominant systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Sj{\"o}gren's syndrome, and thyroiditis as well as male-dominant autoimmune diseases like myocarditis which has been linked to HIV-associated PAH. The sex-specific association of PAH to certain infections and autoimmune diseases suggests that sex hormones and inflammation may play an important role in driving the pathogenesis of disease. However, there is a paucity of data on sex differences in inflammation in PAH, and more research is needed to better understand the pathogenesis underlying PAH in men and women. This review uses data on sex differences in PAH and PAH-associated autoimmune diseases from registries to provide insight into the pathogenesis of disease.",
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