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===Incidence===
===Incidence===
 
* While previously considered a rare disease, the most recent evidence from a French registry suggests that the [[incidence]] of new cases of pulmonary arterial hypertension is 0.20-0.30 cases per 100,000 individuals.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>
While previously considered a rare disease, the most recent evidence from a French registry suggests that the [[incidence]] of new cases of pulmonary arterial hypertension is 2-3 cases per million per year.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>


===Prevalence===
===Prevalence===
 
* The [[prevalence]] of pulmonary hypertension is approximately 1.5 per 100,000 individuals.
The [[prevalence]] is about 15 per million.


===Age===
===Age===
 
* Pulmonary hypertension usually develops between the ages of 20 and 60, but it can occur at any age. The mean age of patients in the U.S. registry was approximately 36 years.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>
Pulmonary hypertension usually develops between the ages of 20 and 60, but it can occur at any age. The mean age of patients in the U.S. registry was approximately 36 years.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>


===Gender===
===Gender===
The female-to-male ratio  for PH is approximately 1.7:1.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>
* The female-to-male ratio  for PH is approximately 1.7:1.<ref name="Rich-1987">{{Cite journal  | last1 = Rich | first1 = S. | last2 = Dantzker | first2 = DR. | last3 = Ayres | first3 = SM. | last4 = Bergofsky | first4 = EH. | last5 = Brundage | first5 = BH. | last6 = Detre | first6 = KM. | last7 = Fishman | first7 = AP. | last8 = Goldring | first8 = RM. | last9 = Groves | first9 = BM. | title = Primary pulmonary hypertension. A national prospective study. | journal = Ann Intern Med | volume = 107 | issue = 2 | pages = 216-23 | month = Aug | year = 1987 | doi =  | PMID = 3605900 }}</ref>
 
* Idiopathic pulmonary hypertension (IPAH) which is more prevalent in women (3x more common), was considered the most common type of pulmonary arterial hypertension in a French registry.  
Idiopathic pulmonary hypertension (IPAH) which is more prevalent in women (3x more common), was considered the most common type of Pulmonary arterial hypertension in a French registry.
 
===Genetics===
 
'''Familial PAH''' often results from a [[mutation]] in the bone morphogenic protein receptor-2 (BMPR2) and is inherited as an [[autosomal dominant]] disease with incomplete [[penetrance]] and anticipation.<ref name="Rich-1987" />
 
===Association with Diseases===
 
*PAH is also associated with:<ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>
#[[Congenital heart disease]](30% of untreated)
#[[Connective tissue diseases]](12% of patients with scleroderma and up to 21% of patients with rheumatoid arthritis)
#[[HIV]] (0.5%)
#[[Portal hypertension]] (2-6%)
#[[Sickle cell disease]] (20 to 40%)
#[[Systemic lupus erythematosus]] (4 to 14%)
#[[Hemoglobinopathies]]
#[[Myeloproliferative disorders]]
#Drugs and toxins
 
*Diet pills such as [[Fen-Phen]] produced an annual incidence of 25-50 per million per year.


==References==
==References==

Revision as of 14:14, 27 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ralph Matar

Overview

Pulmonary arterial hypertension has been considered as a disease of young women. The mean age of patients in the U.S. registry was 36 years and the overall female-to-male ratio was 1.7:1.

Epidemiology and Demographics

Incidence

  • While previously considered a rare disease, the most recent evidence from a French registry suggests that the incidence of new cases of pulmonary arterial hypertension is 0.20-0.30 cases per 100,000 individuals.[1]

Prevalence

  • The prevalence of pulmonary hypertension is approximately 1.5 per 100,000 individuals.

Age

  • Pulmonary hypertension usually develops between the ages of 20 and 60, but it can occur at any age. The mean age of patients in the U.S. registry was approximately 36 years.[1]

Gender

  • The female-to-male ratio for PH is approximately 1.7:1.[1]
  • Idiopathic pulmonary hypertension (IPAH) which is more prevalent in women (3x more common), was considered the most common type of pulmonary arterial hypertension in a French registry.

References

  1. 1.0 1.1 1.2 Rich, S.; Dantzker, DR.; Ayres, SM.; Bergofsky, EH.; Brundage, BH.; Detre, KM.; Fishman, AP.; Goldring, RM.; Groves, BM. (1987). "Primary pulmonary hypertension. A national prospective study". Ann Intern Med. 107 (2): 216–23. PMID 3605900. Unknown parameter |month= ignored (help)