medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2024-401medsovet-8559Research ArticleРЕВМАТОЛОГИЯRHEUMATOLOGYСистемная склеродермия, ассоциированная с легочной артериальной гипертензией: фокус на висцеральную форму болезниSystemic sclerosis associated with pulmonary arterial hypertension: Focus on the visceral form of the diseasehttps://orcid.org/0000-0001-8469-8423ЮдкинаН. Н.YudkinaN. N.

Юдкина Наталья Николаевна - к.м.н., научный сотрудник.

115522, Москва, Каширское шоссе, д. 34А

Natalia N. Yudkina - Cand. Sci. (Med.), Researcher, Nasonova Research Institute of Rheumatology.

34А, Kashirskoe Shosse, Moscow, 115522

natudkina@mail.ruhttps://orcid.org/0009-0002-7761-1048НевретдиновТ. И.NevretdinovT. I.

Невретдинов Тимур Ильдарович – студент.

117198, Москва, ул. Миклухо-Маклая, д. 6

Timur I. Nevretdinov - Student, Peoples’ Friendship University of Russia named after Patrice Lumumba.

6, Miklukho-Maklai St., Moscow, 117198

timur.nevretdinov@mail.ruhttps://orcid.org/0000-0003-1784-3699ВолковА. В.VolkovA. V.

Волков Александр Витальевич - к.м.н., заведующий лабораторией.

115522, Москва, Каширское шоссе, д. 34А

Alexander V. Volkov - Cand. Sci. (Med.), Head of Laboratory, Nasonova Research Institute of Rheumatology.

34А, Kashirskoe Shosse, Moscow, 115522

sandyvlk@yahoo.comНаучно-исследовательский институт ревматологии имени В.А. НасоновойРоссияNasonova Research Institute of RheumatologyRussian FederationРоссийский университет дружбы народов имени Патриса ЛумумбыРоссияPeoples’ Friendship University of Russia named after Patrice LumumbaRussian Federation2024240920240228795Copyright © Юдкина Н.Н., Невретдинов Т.И., Волков А.В., 20242024Юдкина Н.Н., Невретдинов Т.И., Волков А.В.Yudkina N.N., Nevretdinov T.I., Volkov A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/8559Введение

Введение. Системная склеродермия – заболевание соединительной ткани с развитием облитерирующей артериолопатии и активным фиброзообразованием как во внутренних органах, так и в коже. Легочная артериальная гипертензия – жизнеугрожающее проявление системной склеродермии, при поздней диагностике приводящее к летальному исходу. Поиск предикторов, а также ассоциированных фенотипов болезни может способствовать ранней диагностике и улучшению прогноза.

Цель

Цель. Охарактеризовать особенности висцеральной формы в сравнении с лимитированным вариантом у пациентов с легочной артериальной гипертензией, ассоциированной с системной склеродермией.

Материалы и методы

Материалы и методы. В исследование включены 14 пациентов с висцеральной и 63 – с лимитированной системной склеродермией, ассоциированной с легочной артериальной гипертензией. Диагноз системной склеродермии устанавливался на основании критериев EULAR 2013, легочная артериальная гипертензия верифицировалась при катетеризации сердца. У всех пациентов были исключены другие причины легочной гипертензии – заболевания сердца, легких, тромбофилии.

Результаты

Результаты. Пациенты с висцеральной системной склеродермией были моложе (48 (35; 56) лет), чем с лимитированной (54 (49; 63) года), однако различия лишь приближались к достоверным (р = 0,057). У всех пациентов имелся синдром Рейно, при лимитированной системной склеродермии чаще отмечались дигитальные ишемические нарушения (41% в сравнении с 14%, р = 0,11). У большинства выявлялись антицентромерные антитела, антитела к топоизомеразе-I обнаружены только у двух пациентов с лимитированной системной склеродермией. Индекс тяжести был достоверно выше у пациентов с лимитированной системной склеродермией (р < 0,05). Проявления легочной артериальной гипертензии в обеих группах также совпадали. При исследовании центральной гемодинамики значимых различий не обнаружено. Медиана наблюдения составила 68 (39; 111) мес. Выживаемость также не различалась: при висцеральной системной склеродермии она составила 63 (40; 99) мес., при лимитированной – 69 (36; 116) мес.

Заключение

Заключение. Сравнительный анализ продемонстрировал схожесть двух рассматриваемых фенотипов системной склеродермии, что предполагает универсальность подходов к ранней диагностике легочной артериальной гипертензии.

 

 

Introduction

Introduction. Systemic sclerosis is a connective tissue disease with the development of obliterating arteriolopathy and active fibrosis formation both in internal organs and in the skin. Pulmonary arterial hypertension is a life-threatening manifestation of systemic sclerosis, leading to death if diagnosed late. The search for predictors, as well as associated disease phenotypes, can facilitate early diagnosis and improve prognosis.

Aim

Aim. To characterize the features of the visceral form in comparison with the limited variant in patients with pulmonary arterial hypertension associated with systemic sclerosis.

Materials and methods

Materials and methods. 14 patients with visceral and 63 with a limited variant of systemic sclerosis associated with pulmonary arterial hypertension were studied. The diagnosis of systemic sclerosis was established based on the 2013 ACR-EULAR criteria; pulmonary arterial hypertension was verified by right heart catheterization. In all patients, other causes of pulmonary hypertension – heart disease, lung disease, thrombophilia were excluded.

Results

Results. At the time of inclusion in the study, patients with visceral systemic sclerosis were younger (48 (35; 56) years) than those with limited systemic sclerosis (54 (49; 63) years, but the differences only approached significant (p = 0.057). All patients had the Raynaud’s syndrome, with limited systemic sclerosis, digital ischemic disorders were more often observed (41% compared to 14%, p = 0.11). Anticentromere antibodies caused by pulmonary arterial hypertension predominated; antibodies to topoisomerase-I were detected only in two patients with limited systemic sclerosis. The severity index was significantly higher in patients with limited systemic sclerosis (p < 0.05). The clinical manifestations of pulmonary arterial hypertension in both groups were also the same. When studying central hemodynamics, no significant differences were found. The median follow-up of patients was 68 (39; 111) months. Survival also did not differ: with visceral systemic sclerosis it was 63 (40; 99) months, with limited systemic sclerosis – 69 (36; 116) months.

Conclusion

Conclusion. A comparative analysis demonstrated the similarity of the two systemic sclerosis phenotypes, which suggests the universality of approaches to the early diagnosis of pulmonary arterial hypertension.

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The authors declare that there are no conflicts of interest present.