Легочная гипертензия, ассоциированная с портальной гипертензией, и легочная гипертензия при саркоидозе органов дыхания: сложные патогенетические взаимоотношения

 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.В обзоре литературе представлены современные взгляды на особенности патогенеза и диагностику легочной гипертензии (ЛГ), ассоциированной с портальной гипертензией и саркоидозом органов дыхания, что обусловлено отсутствием убедительной доказательной базы, лежащей в основе четкого алгоритма диагностики и лечения данной патологии. Легочная гипертензия является одной из самых сложных кардиологических проблем и представляет собой патологическое состояние, в основе которого лежит повышение сопротивления току крови в малом круге кровообращения на любом из его участков. В отличие от системных артерий к легочной артерии нет прямого доступа для измерения артериального давления неинвазивными методами, поэтому диагностика ЛГ на ранних стадиях, до поражения органа-мишени, которым является правый желудочек сердца, практически невозможна. В реальной клинической практике ЛГ в лучшем случае диагностируется на стадии скрытой дисфункции правого желудочка, которая проявляется дилатацией его полости и (или) гипертрофией его стенок, в худшем – на стадии правожелудочковой сердечной недостаточности. В настоящее время отмечена тенденция к улучшению диагностики ЛГ при различных заболеваниях. Именно этим обусловлен особый интерес к данной междисциплинарной проблеме

Состояние больных саркоидозом исходно и 10 лет спустя при различной тактике их ведения (мультицентровый анализ)

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

Pulmonary hypertension associated with portal hypertension and pulmonary hypertension in sarcoididosis of breathing organs: complex pathogenetic relationships

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