The ClinicianThe ClinicianКлиницист1818-83382412-8775Publishing House ABV Press64610.17650/1818-8338-2025-19-1-K740REVIEWОБЗОРPathogenetic features of pulmonary hypertension in systemic sclerosisПатогенетические особенности легочной гипертензии при системной склеродермииhttps://orcid.org/0000-0003-3757-2152VaseninaV. S.ВасенинаВ. С.
Russian Federation

Vera Sergeevna Vasenina 

1 Ostrovityanova St., Moscow 117997

Вера Сергеевна Васенина 

117997 Москва, ул. Островитянова, 1

vasenina_ru@mail.ruhttps://orcid.org/0000-0001-6890-8777DemidovaN. A.ДемидоваН. А.
Russian Federation

1 Ostrovityanova St., Moscow 117997, 

8 Leninskiy Prospekt, Moscow 117049

117997 Москва, ул. Островитянова, 1,

117049 Москва, Ленинский пр­кт, 8

https://orcid.org/0000-0001-9152-3234KondrashovA. A.КондрашовА. А.
Russian Federation

1 Ostrovityanova St., Moscow 117997

117997 Москва, ул. Островитянова, 1

https://orcid.org/0000-0001-8266-6022AndriyashkinaD. Yu.АндрияшкинаД. Ю.
Russian Federation

1 Ostrovityanova St., Moscow 117997

117997 Москва, ул. Островитянова, 1

https://orcid.org/0000-0002-7410-9784KlimenkoA. A.КлименкоА. А.
Russian Federation

1 Ostrovityanova St., Moscow 117997, 

8 Leninskiy Prospekt, Moscow 117049

 

117997 Москва, ул. Островитянова, 1,

117049 Москва, Ленинский пр­кт, 8

N.I. Pirogov National Research Medical University, Ministry of Health of RussiaФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииN.I. Pirogov City Clinical Hospital No. 1, Moscow Healthcare DepartmentГБУЗ г. Москвы «Городская клиническая больница № 1 им. Н.И. Пирогова Департамента здравоохранения г. Москвы»1501202519112200107202501072025Copyright ©; 2025, Vasenina V.S., Demidova N.A., Kondrashov A.A., Andriyashkina D.Y., Klimenko A.A.Copyright ©; 2025, Васенина В.С., Демидова Н.А., Кондрашов А.А., Андрияшкина Д.Ю., Клименко А.А.2025Vasenina V.S., Demidova N.A., Kondrashov A.A., Andriyashkina D.Y., Klimenko A.A.Васенина В.С., Демидова Н.А., Кондрашов А.А., Андрияшкина Д.Ю., Клименко А.А.https://creativecommons.org/licenses/by/4.0https://klinitsist.abvpress.ru/Klin/article/view/646

Systemic connective tissue diseases may be associated with various forms of pulmonary hypertension (PH). The prevalence of PH varies among different systemic connective tissue diseases, with systemic scleroderma (SSc) having the highest rate, ranging from 8 to 12 %. With SSc, PH is one of the severe and life-threatening manifestations, which necessitates the study of this problem. The pathogenesis of PH in SSc varies and depends on the predominant organ damage. PH can be represented by pulmonary arterial hypertension (PAH) (group 1) with isolated damage to the pulmonary vessels, or with more rare conditions such as veno-occlusive disease, portopulmonary hypertension, or drug toxicity. Venous PH can also develop due to damage to the left chambers of the heart, such as myocardial fibrosis, diastolic dysfunction of the left ventricle, or valve pathology (group 2). PH associated with lung damage may develop due to interstitial lung Systemic connective tissue diseases may be associated with various forms of pulmonary hypertension (PH). The prevalence of PH varies among different systemic connective tissue diseases, with systemic scleroderma (SSc) having the highest rate, ranging from 8 to 12 %. With SSc, PH is one of the severe and life-threatening manifestations, which necessitates the study of this problem. The pathogenesis of PH in SSc varies and depends on the predominant organ damage. PH can be represented by pulmonary arterial hypertension (PAH) (group 1) with isolated damage to the pulmonary vessels, or with more rare conditions such as veno-occlusive disease, portopulmonary hypertension, or drug toxicity. Venous PH can also develop due to damage to the left chambers of the heart, such as myocardial fibrosis, diastolic dysfunction of the left ventricle, or valve pathology (group 2). PH associated with lung damage may develop due to interstitial lung diseases, such as nonspecific and usual interstitial pneumonia (group 3). Patients with SSc are more susceptible to thrombosis and pulmonary embolism due to formation of antiphospholipid antibodies, which can lead to the development of chronic thromboembolic PH (group 4). A characteristic feature of SSc is that one patient may have several reasons for PH development. The variety of pathogenetic variants of PH in SSc requires personalized and comprehensive diagnostic approaches to each patient. Management of the patient and determination of indications for appointment of PAH-specific therapy depend on the variant and pathogenesis of PH.

Системные заболевания соединительной ткани могут быть связаны с различными формами легочной гипертензии (ЛГ). Распространенность ЛГ варьируется среди различных системных заболеваний соединительной ткани, при этом системная склеродермия (ССД) имеет самый высокий показатель и составляет от 8 до 12 %. при ССД ЛГ является одним из тяжелых и жизнеугрожающих проявлений, что обусловливает необходимость изучения данной проблемы. патогенез ЛГ при ССД различен и зависит от преимущественного поражения органов. ЛГ может быть представлена легочной артериальной гипертензией (ЛАГ) (1-я группа) при изолированном поражении легочных сосудов или при более редких состояниях, таких как веноокклюзионная болезнь, портопульмональная гипертензия, или при лекарственной токсичности. при поражениях левых камер сердца, таких как фиброз миокарда, диастолическая дисфункция левого желудочка или патология клапанов, развивается венозная ЛГ (2-я группа). при интерстициальных заболеваниях легких, таких как неспецифическая и обычная интерстициальная пневмония, может возникать ЛГ, связанная с поражением легких (3-я группа). пациенты с ССД подвержены развитию тромбозов и тромбоэмболии легочной артерии в связи с образованием антифосфолипидных антител, что может привести к хронической тромбоэмболической ЛГ (4-я группа). Характерным для ССД является сочетание у одного пациента нескольких причин формирования ЛГ. Многообразие патогенетических вариантов ЛГ при ССД требует индивидуального и комплексного диагностического подхода к каждому пациенту. Именно от варианта и патогенеза ЛГ зависят дальнейшая тактика ведения больного и определение показаний к назначению ЛАГ-специфической терапии.

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