References

mrj

Современная ревматология

Modern Rheumatology Journal

1996-70122310-158X

IMA-PRESS, LLC

10.14412/1996-7012-2014-2-48-57

mrj-541

Research Article

ЛЕКЦИИ

LECTURES

Синдром Рейно в практике ревматолога

Raynaud’s phenomenon in the rheumatologist’s practice

Алекперов

Р.Т.

Alekperov

R.T.

1@ru.ru

ФГБУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой» РАМН, Москва, РоссияV.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Moscow, Russia

2014

08052014

824857

2014

Алекперов Р.

Alekperov R.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://mrj.ima-press.net/mrj/article/view/541

Синдром Рейно (СР) широко распространен в популяции, преобладает среди женщин и в большинстве случаев дебютирует в возрасте до 30 лет. Впервые синдром описан М. Рейно более 150 лет назад, в дальнейшем был выделен первичный (болезнь Рейно) и вторичный СР. Патофизиология СР до конца неясна, по-видимому, СР является мультифакторным заболеванием. Считается, что причиной чрезмерно выраженного вазоспазма в ответ на провоцирующие стимулы является дефект центральных и локальных механизмов регуляции сосудистого тонуса. Среди этих факторов важными представляются сосудистые эндотелиальные, внутрисосудистые и нейрональные нарушения. Существенную роль в патофизиологии вторичного СР играют структурные изменения сосудов, наблюдаемые при капилляроскопии ногтевого ложа. Диагностика СР предполагает в первую очередь определение его клинического варианта, так как вторичный СР бывает проявлением или предвестником некоторых аутоиммунных, гематологических, эндокринных, неопластических и иных заболеваний. Наиболее часто вторичный СР отмечается у больных с системными ревматическими заболеваниями. Лечение вторичного СР остается неудовлетворительным вследствие неполного представления о патофизиологических механизмах его развития. В настоящее время в лечении СР применяются препараты разных лекарственных групп, включая блокаторы кальциевых каналов, ингибиторы фосфодиэстеразы 5-го типа, антагонисты рецепторов эндотелина и др. В обзоре рассматриваются вопросы патогенеза, алгоритм ранней и дифференциальной диагностики СР, современные подходы к лечению на основе анализа данных литературы и результатов собственных исследований.

Raynaud’s phenomenon (RP) is a common condition that predominantly occurs among women. The onset age in most cases is under 30 years. The phenomenon was first described by M. Raynaud over 150 years ago. Primary (Raynaud’s disease) and secondary RP were subsequently distinguished. Pathophysiology of RP has not been fully elucidated yet; RP seems to be a multifactor disease. A pronounced vasospasm in response to stimuli is believed to be caused by disruption in the central and local mechanisms of regulation of vascular tonus. Vascular endothe- lial, intravascular, and neuronal disorders are the key ones among these factors. Structural changes in vessels that can be observed by nailfold capillaroscopy play a significant role in pathophysiology of secondary RP. The key step in the diagnosis of RP is identifying its clinical variant, since secondary RP may be a sign or a premonitory symptom of certain autoimmune, hematologic, endocrine, neoplastic, and other disorders. Secondary RP is most frequently observed in patients with systemic rheumatoid diseases. Therapy of secondary RP provides no satisfactory out- comes, since there is no complete conception about the pathophysiological mechanisms of its development. Different groups of drugs are cur- rently used to treat RP, including calcium channels blockers, phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, etc. This review focuses on pathogenesis, the algorithm of early and differential diagnosis of RP, and current approaches to therapy based on the analysis of the published and our own data.

синдром Рейнопатогенездифференциальная диагностикалечениекальциевые блокаторысилденафилпростаноиды.

Raynaud’s phenomenonpathogenesisdifferential diagnosistreatmentcalcium antagonistssildenafilprostanoids.

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