- 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 |Favorite Table|Download (.pdf)
Table 63–1. Clinical Classification of Pulmonary Hypertension.a
Pulmonary arterial hypertension
- 1.1. Idiopathic
- 1.2. Heritable
- 1.2.1. BMPR2
- 1.2.2. ALK1, endoglin
- 1.2.3. Unknown
- 1.3. Drug- and toxin-induced
- 1.4. Associated with
- 1.4.1. Connective tissue disease (eg, scleroderma)
- 1.4.2. HIV infection
- 1.4.3. Portal hypertension (eg, due to autoimmune hepatitis)
- 1.4.4. Congenital heart disease
- 1.4.5. Schistosomiasis
- 1.4.6. Chronic hemolytic anemia
- 1.5. Persistent pulmonary hypertension of the newborn
- 1.5.1. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (well described in scleroderma)
Pulmonary hypertension owing to left heart disease
- 2.1. Systolic dysfunction
- 2.2. Diastolic dysfunction
- 2.3. Valvular disease
Pulmonary hypertension owing to lung disease or hypoxemia
- 3.1. Chronic obstructive lung disease
- 3.2. Interstitial lung disease (eg, associated with connective tissue diseases)
- 3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern
- 3.4. Sleep-disordered breathing
- 3.5. Alveolar hypoventilation disorders (eg, with neuromuscular disease)
- 3.6. Chronic exposure to high altitude
- 3.7. Developmental abnormalities
Chronic thromboembolic pulmonary hypertension (eg, antiphospholipid antibody syndrome)
Pulmonary hypertension with unclear multifactorial mechanisms
- 5.1. Hematologic disorders: myeloproliferative disorders, splenectomy
- 5.2. Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis
- 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
- 5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
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 ...