What are the symptoms of primary pulmonary hypertension

Many people may not know much about primary pulmonary arterial hypertension, but in fact, there is not a single cause of these diseases. Common autoimmune system diseases, systemic lupus erythematosus, and rheumatoid arthritis may all be associated with primary pulmonary arterial hypertension.

1. Pulmonary thromboembolism Initially, some authors believed that many small clinical subtypes of thromboembolism may be the cause of most primary pulmonary hypertension, because pathological findings showed that about half of the patients with clinically diagnosed primary pulmonary hypertension had chronic pulmonary thromboembolism. However, subsequent studies found that histologically thromboembolic pulmonary hypertension was different from plexogenic primary pulmonary hypertension. In thromboembolic pulmonary hypertension, the pulmonary vascular media is mildly thickened, the intimal fibrosis is eccentric, and dilatational lesions never occur. Fibrinoid arteritis and plexus-like lesions are formed, which is different from plexigenic pulmonary artery disease.

2. Autoimmune diseases such as systemic lupus erythematosus, Raynaud's disease, CREST syndrome, scleroderma, rheumatoid arthritis, dermatomyositis, polymyositis and mixed connective tissue disease can all be complicated by primary pulmonary hypertension. In patients with primary pulmonary hypertension, 29% to 40% of circulating anti-

3. Nuclear antibody was positive, and 11% had Raynaud's phenomenon. Among 8 patients with CREST syndrome [calcification, Raynaud's phenomenon, esophageal dysmotility, scleroderma of the fingers and toes, and telangiectasia], 7 had pulmonary hypertension, suggesting that primary pulmonary hypertension may be partly related to autoimmune diseases.

4. Factors related to pregnancy and menstrual cycle: Primary pulmonary hypertension is often first discovered during pregnancy, and hemodynamic changes worsen during pregnancy. The menstrual cycle can affect the reactivity of systemic blood vessels, but it is not clear whether it also affects pulmonary blood vessels. The cause of the increase in primary pulmonary hypertension in women of childbearing age is still unclear, but it is obviously not caused by a single etiological factor. It has been suggested that amniotic fluid embolism may be the etiology of primary pulmonary hypertension, but histological examination has failed to confirm the presence of residual amniotic fluid embolism.