Pulmonary hypertension epidemiology and demographics: Difference between revisions
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== Overview == | == 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. | 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== | ==Epidemiology and Demographics== | ||
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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 2-3 cases per million per year. | 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=== | ||
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===Genetics=== | ===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. | '''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=== | ===Association with Diseases=== | ||
Revision as of 15:34, 28 February 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 2-3 cases per million per year.[1]
Prevalence
The prevalence is about 15 per million.
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.
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.[1]
Association with Diseases
- PAH is also associated with:[2]
- 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
- ↑ 1.0 1.1 1.2 1.3 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) - ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension