Клінічний випадок легеневої гіпертензії, асоційованої з рідкісною хворобою легень

This article is clinical presentation of pulmonary hypertension case, associated with rare lung disease – lymphangioleiomyomatosis. There are elucidated: diagnostic criteria of this disease, its prevalence, pathogenesis, including mechanisms of pulmonary hypertension development, clinical presentations and methods of treatment. Authors concluded some special features of this case: rare disease that could be diagnosed by only experienced staff; late patient’s age is atypical for this disease (usual this disease is diagnosed in younger women (childbirth potential age); moderate lung function disorders were accompanied by severe pulmonary hypertension, that needed additional examination in expert center; in spite of general recommendations not to use specific therapy in patients with lung disease the prostaglandins were used in this patient with improving of clinical symptoms. Based on summary of 6th World Symposium on Pulmonary Hypertension (Nice, 2018) there were discussed the particularities of pulmonary hypertension diagnosis and treatment in patients with lung disease or chronic hypoxia (group 3) and their differences with pulmonary arterial hypertension (group 1). It was stressed the necessity of individual approaches in specific therapy using in patients with lung diseases and high pulmonary artery pressure and providing the clinical trials for evaluation of this therapy influence on prognosis.