PULMONARY
ARTERIAL HYPERTENSION (PAH) RELATED TO OTHER CONDITIONS
According to
the WHO classification, some diseases are grouped together as pulmonary
arterial hypertension (PAH). Besides primary pulmonary hypertension
(PPH), these diseases have a specific common histology and their
underlying pathophysiology (vascular damage) is presumed to be similar.
They are therefore referred to as “PAH related to other conditions”.
Diseases which are known as associated conditions include:
1. Scleroderma (systemic sclerosis)
2. Other connective tissue diseases (such
as systemic lupus erythematosus)
3. Congenital heart disease
4. HIV infection
5. Portal hypertension
6. Pulmonary hypertension of the newborn (PPHN).
Of these, the most common association is with scleroderma. The occurrence
of PAH in scleroderma is difficult to determine, but the reported
range has varied from nine percent to as much as 65 percent. PAH
is a leading cause of death in scleroderma and greatly reduces life
expectancy in these patients.
PAH is also found in seven to 15 percent of patients with undifferentiated
or mixed connective tissue disease and in five to 10 percent of
those with SLE.
Persistent pulmonary hypertension of the newborn is to be distinguished
from congenital abnormalities of the heart and pulmonary vasculature.
It represents an entity similar to PPH and is typically more responsive
to acute and chronic vasodilator therapies. Untreated, it can be
rapidly fatal.
Recent advances in Doppler echocardiography allow non-invasive monitoring
of pulmonary artery pressure and right ventricular function. The
early diagnosis and treatment of PAH in high-risk patients will
broaden the options available for treatment, improve quality of
life and may prolong survival.
References
1. MacGregor et al. (2001) Pulmonary hypertension in systemic sclerosis:
risk factors for progression and consequences for survival. Rheumatology;
40:453-59
2. D’Angelo WA et al. Am J Med (1969);46:428-40
3. Stupi AM et al. Arthritis Rheum (1986) 29:515-24
4. Alpert MA et al. Circulation 1983 Dec;68(6):1182-93
5. Rich S, A New Classification of Pulmonary Hypertension. Advances
in Pulmonary Hypertension. PHA Journal 2002 1(1):3-6
6. Asherson RA, Pulmonary hypertension in systemic lupus erythematosus.
J Rheumatol. 1990 Mar;17(3):414-5
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