nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1518Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSОсобенности поражения почек, обусловленного сочетанием гломерулонефрита и АФС-ассоциированной нефропатии при системной красной волчанке (обзор литературы и собственное наблюдение)Combination of glomerulonephritis and antiphospholipid syndrome nephropathy in systemic lupus erythematosus (review and case report)КозловскаяН. Л.KozlovskajaN. L.noemail@neicon.ruЗахароваЕ. В.ZakharovaE. V.noemail@neicon.ruЗверевД. В.ZverevD. V.noemail@neicon.ruСухановА. В.SukhanovA. V.noemail@neicon.ruКоэнА. .CohenA. H.noemail@neicon.ruАвдееваО. Н.AvdeevaO. N.noemail@neicon.ruЕпифановаС. Н.EpifanovaS. N.noemail@neicon.ruКафедра нефрологии и гемодиализа ММА имени И.М. СеченоваRussian FederationМосковская городская клиническая больница имени С.П. БоткинаRussian FederationДетская городская клиническая больница Святого ВладимираRussian FederationНаучно-исследовательский институт трансплантологии и искусственных органов, г. МоскваRussian FederationОтдел патологии и лабораторной медицины, медицинский центр Cedars-Sinai, Лос-Анджелес, СШАRussian Federation20072306202594439446Copyright © Козловская Н.Л., Захарова Е.В., Зверев Д.В., Суханов А.В., Коэн А..., Авдеева О.Н., Епифанова С.Н., 20252025Козловская Н.Л., Захарова Е.В., Зверев Д.В., Суханов А.В., Коэн А..., Авдеева О.Н., Епифанова С.Н.Kozlovskaja N.L., Zakharova E.V., Zverev D.V., Sukhanov A.V., Cohen A.H., Avdeeva O.N., Epifanova S.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/1518

У больных с СКВ, протекающей с АФС, возможно сочетание АФС-ассоциированной нефропатии с волчаночным нефритом, частота такого сочетания может составлять до 32-68% случаев. Клинические проявления АФСН и тяжелого ВН - артериальная гипертензия, нарастающая почечная недостаточность - сходны, и зачастую только данные биопсии почки позволяют провести дифференциальный диагноз, влияющий на тактику лечения. Повторные нефробиопсии нередко позволяют выявить трансформацию одного класса ВН в другой, трансформации диффузного пролиферативного ВН в мембранозный может способствовать успешная иммуносупрессивная терапия. Приводимый случай длительного течения СКВ с ВН IV класса и развитием в период обострения тяжелой почечной недостаточности, обусловленной присоединением АФСН, иллюстрирует трудности диагностики, которая оказалась возможной только при выполнении двух нефробиопсий с интервалом в 7 месяцев и повторном исследовании крови на наличие различных антител к фосфолипидам. Показана также возможность успешного лечения как ВН, так и АФСН при применении массивной иммуносупрессивной, а затем и антикоагулянтной терапии в сочетании с плазмаферезом. На фоне лечения отмечена трансформация ВН IV класса в V класс, после 22 месяцев терапии удалось достичь частичного восстановления функции почек и прекратить процедуры гемодиализа, ремиссия в течение последующих полутора лет поддерживается применением небольших доз кортикостероидов и микофенолатов в сочетании с непрямыми антикоагулянтами.

Combination of lupus nephritis and antiphospholipid syndrome nephropathy may occure in patients with SLE, complicated with antiphospholipid syndrome, in 32-68% of cases. Clinical manifestations of antiphospholipid syndrome nephropathy and proliferative lupus nephritis, such as arterial hypertension and progressive renal failure may be similar, and often only renal biopsy can help to make a proper diagnosis and choose adequate treatment strategy. Serial renal biopsies may help not only in assessing degree of activity and the progression of chronicity of lupus nephritis, but may also show possible class transformation. Transitions in lupus nephritis are not unusual and almost any class has been documented to go to any other class. Successful treatment of diffuse proliferative lupus nephritis may result in the transformation to membranous LN. The pediatric patient with biopsy proven LN class IV developed flare with dialysis-dependent renal failure due to superimposition of thrombotic microangiopathy. The case illustrates the difficulties of diagnostics, only the second biopsy, performed 7 months later and after 4 month of aggressive immunosupression with steroids and cyclophosphamide, indicated the presence of thrombotic microangiopathy with glomerular ischemia and transformation of the LN to class V. Interestingly, there were no other clinical symptoms of APS, anticardiolipin antybodies were in normal range, and only presence of lupus anticoagulant, found after second biopsy, supported diagnosis of APS. Addition of long-term anticoagulant therapy lead to partial restoration of renal function, so that patient became dialysis-free after 22 months of treatment. Supportive treatment with low-dose steroids, mycophenolates and warfarin allows to maintain remission over 18 months.

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