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  • Mean pulmonary artery pressure ≥25 mm Hg defines pulmonary hypertension.
  • Values between 21 mm Hg and 24 mm Hg are considered borderline elevated.
  • Pulmonary hypertension is classified as precapillary when the left heart filling pressure or pulmonary artery wedge pressure is ≤15 mm Hg.

Pulmonary hypertension is among the most serious complications of rheumatic diseases. Pulmonary hypertension can be classified into one of five broad categories (Table 63–1). While rheumatic conditions can be associated with any classification of pulmonary hypertension (see Table 63–1), the most common category in which rheumatic conditions fall is pulmonary arterial hypertension—a pulmonary vasculopathy in the absence of left heart dysfunction, underlying parenchymal lung disease, thromboembolism, or other causes.

Table 63–1. Clinical Classification of Pulmonary Hypertension.a

Connective tissue diseases accounted for one-quarter of all pulmonary arterial hypertension cases in REVEAL, a large US registry. Pulmonary arterial hypertension is particularly common in scleroderma or systemic sclerosis with a prevalence of 4–8%, and an estimated incidence of 0.61 cases per 100 patient years, accounting for 65% of connective tissue diseases associated with pulmonary arterial hypertension. In systemic sclerosis, pulmonary arterial hypertension typically occurs in older women (>80% female; mean age of 60 years) with limited scleroderma (up to 90%) and is more common among patients with late-onset systemic sclerosis. The average duration of systemic sclerosis prior to pulmonary arterial hypertension diagnosis varies from 4 to 14 years, but it can occur concomitant with or soon after disease onset.

The frequency of pulmonary arterial hypertension in other connective tissue diseases is not well characterized, ...

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