OVERVIEW
Connective tissue disease is a common cause of pulmonary hypertension, and almost all types of connective tissue disease can be complicated by arterial pulmonary hypertension. Pulmonary hypertension can occur in a variety of connective tissue diseases, such as systemic lupus erythematosus, scleroderma (systemic sclerosis), mixed connective tissue disease, and rheumatoid arthritis.
Etiology
The cause of this disease is not clear, and its pathogenesis may be related to the following factors.
1. Pulmonary vasospasm and contraction, when stimulated by hypoxia, cold and other stimuli, will lead to spasm of small pulmonary arteries, pulmonary vascular resistance increases, which will cause hypoxic injury to lung tissue.
2. Interstitial lung lesions.
3. Thickening of the wall lining and middle membrane of small pulmonary arteries, which narrows the lumen.
4. Small pulmonary vasculitis.
5. Thromboembolism.
6. Local disorders of vasoactive mediators affecting pulmonary artery tone.
7. Autoimmune factors.
Symptoms
1. The onset of the disease is usually insidious, mostly occurring in young and middle-aged women, and is usually asymptomatic in the early stage.
2. The main manifestations are dyspnea, palpitation, and fatigue.
3. In the late stage, it may manifest as chest pain, syncope during activity and signs of right heart failure, such as lower limb edema and abdominal distension.
4. Intermittent fever, which may be characterized by abnormalities of the skin, muscles, joints and bones.
5. Clinical manifestations associated with connective tissue disease.
Examination
1. Increased erythrocyte sedimentation rate (sedimentation rate), increased levels of C-reactive protein and rheumatoid factor.
2. For asymptomatic patients, symptomatic patients with scleroderma and other connective tissue diseases, echocardiography can assess pulmonary hypertension, enlarged right atrium and ventricle and signs of right heart failure.
3. Lung CT examination can help to clarify the presence of pulmonary embolism or interstitial lung disease.
4. Abnormal serum immunologic findings.
Diagnosis
Patients already diagnosed with connective tissue disease are diagnosed when the presence of pulmonary hypertension is confirmed by examination.
Treatment
1. Treatment of the cause of the disease
The initial treatment for pulmonary hypertension is aggressive treatment of the underlying disease and predisposing factors.
2. Drug therapy
Depending on the condition, glucocorticoids and immunosuppressive drugs are used to treat connective tissue disease. Drugs such as prostacyclin, sildenafil and bosentan are effective in the treatment of pulmonary hypertension.
3. Surgery
Surgery or interventional intervention may be considered for severe pulmonary hypertension associated with connective tissue disease. In patients being considered for transplantation, the presence of other organ involvement, especially renal disease, must be carefully investigated. Since such patients often have a combination of multisystem pathologies and have been treated with immunosuppressive drugs, transplant therapy must be approached with caution.