Many connective tissue diseases such as systemic sclerosis and systemic lupus erythematosus can be combined with pulmonary hypertension, and their correct and active treatment directly affects the prognosis of patients. (I) General treatment Current therapeutic measures for pulmonary hypertension are not ideal, and some drugs are expensive and unaffordable for most patients, so general treatment should not be neglected. Patients with pulmonary hypertension should try to avoid excessive physical load, bathing in excessively hot water, oral contraceptives, pregnancy and staying in an environment above 800m above sea level in their daily life. 1, anticoagulants: a group of retrospective analysis shows that long-term use of anticoagulants can improve survival. Most scholars now advocate long-term treatment with warfarin for patients with pulmonary hypertension to maintain INR between 2 and 3. 2, long-term oxygen therapy: hypoxia can aggravate pulmonary microvascular spasm, oxygen therapy may be beneficial to patients, but so far there is no controlled trial to confirm that oxygen therapy can improve the survival rate of patients with pulmonary hypertension. At present, it is advocated for those with severe hypoxia (PaO215 hours. 3. Diuretics: Patients presenting with right heart failure can be treated with diuretics, tachypnea alone or in combination with spironolactone. Excessive diuresis should be avoided to avoid excessive reduction of right ventricular preload, resulting in reduced cardiac output. 4, digitalis preparations: short-term sedation can increase cardiac output, the long-term use of efficacy is not yet certain. Daily administration of 0.125-0.25mg digoxin may be beneficial for those with right heart failure, but patients should be alerted to digitalis toxicity because of hypoxemia. (II) Glucocorticoids Glucocorticoids applied to patients with lupus combined with pulmonary hypertension can inhibit pulmonary vasculitis and reduce pulmonary vascular resistance, thus lowering pulmonary arterial pressure, and there are reports of hormone shock therapy, but the opposite has also been reported. (iii) Immunosuppressants The pathogenesis of immunosuppressant therapy for pulmonary hypertension secondary to connective tissue disease is unknown. It has been found that there are deposits of antinuclear antibodies, rheumatoid factor, and complement fragments in the pulmonary vascular wall of patients with connective tissue disease with pulmonary hypertension, and therefore it is proposed that abnormal immune function can promote the progression of pulmonary artery disease. However, the current articles on immunosuppressive therapy for pulmonary arterial hypertension complicated by connective tissue disease are mostly case reports, and there is no recommended treatment protocol. From the available reports, cytotoxic drugs such as cyclophosphamide and methotrexate are more effective when combined with corticosteroids. (iv) Vasodilators Pulmonary vasospasm is an important component in the pathogenesis of pulmonary hypertension, and the aim of vasodilator therapy is to reduce pulmonary artery pressure and increase cardiac output. In theory, vasodilators should benefit patients with pulmonary hypertension, but in fact only 10-15% of patients with pulmonary hypertension may benefit from vasodilator therapy. Before treating patients with pulmonary hypertension with calcium antagonists, an acute vasodilator trial must be performed via right heart catheterization. Drug trials (adenosine 50-200 ng/kg/min IV drip, epoprostenol 2-24 ng/kg/min IV drip, nitric oxide 10-80 ppm inhalation) have shown that long-term calcium antagonists are only effective if they reduce pulmonary vascular resistance and pulmonary arterial pressure by more than 30% and 20%, respectively, from basal values. The current clinical treatment of patients with pulmonary hypertension blindly with calcium antagonists is harmful and not recommended. Acute vasodilator test is currently commonly used drugs for inhaled nitric oxide. Where inhaled nitric oxide is effective, long-term use of calcium antagonists is also effective, and vice versa. In the past, some people used oral calcium antagonists for acute vasodilator test, because of the serious side effects, is now abandoned. 1, long-term oral calcium antagonists: nitric oxide inhalation can be effective long-term oral calcium antagonists (other vasodilators are not effective), commonly used drugs for nifedipine and diltiazem. Long-term oral calcium antagonists not only improve symptoms and hemodynamics, but may also improve survival. The choice of nifedipine (cardioplegia) or diltiazem (thiodiazepine) may depend on the patient’s basal heart rate; a heart rate of 80 beats/min should be chosen over diltiazem. Doses of calcium antagonists for pulmonary hypertension are much higher, with doses of 90 to 180 mg/d for nifedipine (available up to 240 mg/d) and 360 to 720 mg/d for diltiazem (available up to 900 mg/d). The side effects of high-dose calcium antagonists are generally not serious, but hypotension and edema are common. 2. Epoprostenol (epoproste nitric oxide l): a synthetic prostacyclin (PGI2). Several clinical trials have confirmed that continuous intravenous administration of epoprostenol in patients with pulmonary hypertension of cardiac function class III-IV (NYHA) not only improves exercise tolerance and hemodynamic indices, but also improves survival rates. It has a half-life of only 3 min, so continuous intravenous pumping is required. The price of this product is very expensive, nuggets estimated that the annual cost of drugs in France about 100,000 U.S. dollars. 3, nitric oxide: with vasodilator function, Rolla and Kharito nitric oxide v study found that the exhaled gas nitric oxide concentration of systemic sclerosis patients with pulmonary hypertension is lower than that of those without pulmonary hypertension, and there is a negative correlation between exhaled gas nitric oxide concentration and pulmonary artery pressure. After inhalation of nitric oxide gas, the pulmonary circulatory resistance of patients with systemic sclerosis with pulmonary hypertension can be reduced by 34%, and the mean pulmonary artery pressure can be reduced by 17%. However, there is a lack of studies on the long-term application of nitric oxide in the treatment of pulmonary hypertension associated with connective tissue disease. There are also reports of certain efficacy of oral administration of levorotatory arginine, a synthetic precursor of nitric oxide, in the treatment of pulmonary hypertension, but the safety of this treatment needs to be further verified. 4. Endothelin (ET) receptor antagonists Some patients with connective tissue diseases have elevated serum levels of endothelin, especially in patients with lupus, and pulmonary hypertension is associated with elevated endothelin. Endothelin 1 plays an important role in vasoconstriction and vessel wall reconstruction in pulmonary hypertension. A multicenter randomized double-blind placebo-controlled trial showed that the endothelin receptor antagonist bosentan (Bosentan) reduced pulmonary vascular resistance and pulmonary arterial pressure and significantly increased activity tolerance in patients with pulmonary hypertension in primary and secondary connective tissue disease. 5, adenosine: no significant effect on body circulation, heart rate, cardiac output, resulting in a significant decrease in pulmonary vascular resistance and mean pulmonary artery pressure. Drug: Adenosine. Mechanism of action: direct anti-sympathetic and adenosine A2 receptors on vascular endothelial cells and smooth muscle cells and produce vasodilator effect. 6, angiotensin-converting enzyme inhibitor (ACEI): treatment of pulmonary hypertension with pulmonary vasoconstriction as the main cause of effective treatment. Mechanism of action: inhibition of renin an angiotensin. Aldosterone system, lower blood pressure, increase cardiac output. Inhibits sympathetic neurotransmitter release and reduces angiotensin II production. Induces prostacyclin and nitric oxide, which produce vasoprotective effect and diastolic, and also has protective effect on vascular endothelial cells. Commonly used drugs: captopril and enalapril, etc. 7. The long-acting phosphodiesterase-5 inhibitor Sidenafil (sindenafil, Viagra): by increasing the cyclic guanine mononucleotide to make pulmonary vasodilation. Application of this drug in persistent pulmonary hypertension has a synergistic effect with nitric oxide, prolonging the duration of action without causing a rebound in pulmonary vasoconstriction. In patients with chronic thrombotic pulmonary hypertension, the application of this drug in combination with prostacyclin has a synergistic effect, causing vasodilation.