vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v18i5.2060vsp-2234Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSТерапия канакинумабом сестер с колхицинрезистентной формой семейной средиземноморской лихорадки: клиническое наблюдениеTwo Sisters with Colchicine-Resistant form of Familial Mediterranean Fever on the Canakinumab Therapy: Clinical Casehttps://orcid.org/0000-0001-6724-0883ЛигостаеваЕ. А.LigostaevaElena A.

Лигостаева Елена Алексеевна - кандидат медицинских наук, заведующая педиатрическим отделением.

344015, Ростов-на-Дону, ул. 339-й Стрелковой дивизии, д. 14, тел.: +7 (863) 219-04-17

Rostov-on-Don

elena-odb@ya.ruhttps://orcid.org/0000-0002-3946-430XЦуриковаН. А.TsurikovaNadezhda A.

Ростов-на-Дону

Rostov-on-Don

ЦыганокИ. А.TsiganokIrina A.

Ростов-на-Дону

Rostov-on-Don

КобзеваН. Н.KobzevaNataliya N.

Ростов-на-Дону

Rostov-on-Don

АвдеенкоВ. Ю.AvdeenkoVadim Yu.

Ростов-на-Дону

Rostov-on-Don

Областная детская клиническая больницаРоссияRegional Children's Clinical HospitalRussian Federation201905012020185362368Copyright © Лигостаева Е.А., Цурикова Н.А., Цыганок И.А., Кобзева Н.Н., Авдеенко В.Ю., 20202020Лигостаева Е.А., Цурикова Н.А., Цыганок И.А., Кобзева Н.Н., Авдеенко В.Ю.Ligostaeva E.A., Tsurikova N.A., Tsiganok I.A., Kobzeva N.N., Avdeenko V.Y.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/2234Обоснование

Обоснование. Колхицинрезистентные случаи семейной средиземноморской лихорадки (ССЛ) сопряжены с высоким риском развития амилоидоза почек и тяжелым течением приступных периодов болезни, характеризующихся фебрильной лихорадкой, артритами, плевритами, перикардитами. Контроль болезни у колхицинрезистентных больных труднодостижим и требует использования генно-инженерных биологических препаратов.

Описание клинического случая. Представлено наблюдение семейного случая тяжелого течения ССЛ. Описано успешное применение моноклонального антитела к интерлейкину 1 канакинумаба у сестер с колхицинрезистентной формой заболевания. Уже через 4 нед лечения были полностью купированы лихорадка, суставной и абдоминальный синдромы, отмечена нормализация лабораторных признаков воспаления. Через 32 нед терапии лабораторные показатели активности болезни (скорость оседания эритроцитов и концентрация С-реактивного белка) сохранялись в пределах референс-ных значений, приступов болезни не было. За период наблюдения (32 нед) на фоне терапии канакинумабом развития нежелательных реакций не отмечено.

Заключение

Заключение. Показана высокая эффективность канакинумаба у пациентов с тяжелым течением ССЛ, резистентной к терапии колхицином.

Background

Background. Colchicine-resistant cases of familial Mediterranean fever (FMF) are associated with high risk of kidney amyloidosis and severe episodes of the disease with pyretic fever, arthritis, pleurisies, pericarditis. It is difficult to achieve disease control in colchicine-resistant patients and it is required to use genetically engineered biologic drugs.

Clinical Case Description. The observation of the family with severe course of FFL is presented. The successful use of canakinumab (monoclonal antibody to interleukin-1) in sisters with colchicine-resistant form of disease is described. Fever, articular and abdominal syndromes were completely reversed after 4 weeks of treatment as well as normalisation of laboratory tests was noted. Laboratory indexes of disease activity (erythrocyte sedimentation rate and C-reactive protein level) have stayed in reference ranges after 32 weeks of therapy, no new episodes of the disease were recorded. There were no adverse effect on the canakinumab therapy during the observation period (32 weeks).

Conclusion

Conclusion. The high canakinumab efficacy in patients with severe colchicine-resistant forms of FMF is shown.

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