In the literature review, modern views on the features of pathogenesis and diagnosis of pulmonary hypertension associated with portal hypertension and sarcoidosis of respiratory organs are presented. A variety of views is due to the lack of a convincing evidence base underlying the algorithm for diagnosis and treatment of this pathology. Pulmonary hypertension is one of the most complex cardiological problems and represents a pathological condition that is based on increasing resistance to blood flow in a small circle of blood circulation at any of site. Unlike the systemic arteries, there is no direct access to the pulmonary artery for measuring blood pressure by non-invasive methods, so the diagnosis of pulmonary hypertension in the early stages, before the formation of a lesion in the target organ, which is the right ventricle of the heart, is practically impossible. In actual clinical practice, pulmonary hypertension is at best diagnosed at the stage of latent right ventricular dysfunction, which is manifested by dilatation of its cavity and / or hypertrophy of its walls, at worst at the stage of right ventricular heart failure. At present, there has been a trend towards an improvement in the diagnosis of pulmonary hypertension (LH) in various diseases. This is the reason for the special interest in this interdisciplinary problem.

Comparison of the state of 83 patients with histologically confirmed sarcoidosis observed with a 10 year interval revealed remission in 47% of the cases. The main factors having negative effect on prognosis of the disease included extrapulmonary symptoms, the use ofcorticosteroids (at all stages, especially at stage I and in Lofgren syndrome) and antituberculosis drugs, positive TB test. Risk factors of relapses were stage II sarcoidosis, the use of systemic corticosteroids in patients with Lofgren syndrome and antituberculosis drugs, initially low FEV1/FVLC ratio and the number of lymphocytes in peripheral blood.

Summary. A multicenter (12 centers) retrospective analysis of health status of 83 sarcoidosis patients in time of diagnosis and after 10 years of treatment has been performed. In 10 years after diagnosis, 47 % of the patients had complete remission of pulmonary manifestations; mean forced spirometric values have not reduced in 10 years (excluding patients initially treated with anti-TB drugs). Patients treated with systemic steroids initially or during 10 yrs were less likely to have the complete remission (36.5 %) and more likely to have recurrent sarcoidosis course (57.1 %) compared to those not treated with systemic steroids. Pentoxifylline administration positively influenced the remission rate (71.4 %) and relapse rate in patients who had not received immunosuppressive therapy (28.6 %). A tendency has been found to positive effects of essential phospholipids on relapse rate and remission rate in sarcoidosis. Anti-TB therapy or treatment of sarcoidosis patients in TB centers negatively influenced the outcome of sarcoidosis. The results disclose an urgent need to imply new approaches to treatment of sarcoidosis, eg. anti-TNF-α drugs.

Summary. A multicenter (12 centers) retrospective analysis of health status of 83 sarcoidosis patients in time of diagnosis and after 10 years of treatment has been performed. In 10 years after diagnosis, 47 % of the patients had complete remission of pulmonary manifestations; mean forced spirometric values have not reduced in 10 years (excluding patients initially treated with anti-TB drugs). Patients treated with systemic steroids initially or during 10 yrs were less likely to have the complete remission (36.5 %) and more likely to have recurrent sarcoidosis course (57.1 %) compared to those not treated with systemic steroids. Pentoxifylline administration positively influenced the remission rate (71.4 %) and relapse rate in patients who had not received immunosuppressive therapy (28.6 %). A tendency has been found to positive effects of essential phospholipids on relapse rate and remission rate in sarcoidosis. Anti-TB therapy or treatment of sarcoidosis patients in TB centers negatively influenced the outcome of sarcoidosis. The results disclose an urgent need to imply new approaches to treatment of sarcoidosis, eg. anti-TNF-α drugs.