Abstract

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

Highlights

  • 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

  • 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 have stayed in reference ranges after 32 weeks of therapy, no new episodes of the disease were recorded

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Summary

Клиническое наблюдение

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. Представлено наблюдение семейного случая тяжелого течения ССЛ. Описано успешное применение моноклонального антитела к интерлейкину 1 канакинумаба у сестер с колхицинрезистентной формой заболевания. Через 32 нед терапии лабораторные показатели активности болезни (скорость оседания эритроцитов и концентрация С-реактивного белка) сохранялись в пределах референсных значений, приступов болезни не было. За период наблюдения (32 нед) на фоне терапии канакинумабом развития нежелательных реакций не отмечено. Показана высокая эффективность канакинумаба у пациентов с тяжелым течением ССЛ, резистентной к терапии колхицином. Ю. Терапия канакинумабом сестер с колхицинрезистентной формой семейной средиземноморской лихорадки: клиническое наблюдение. Two Sisters with Colchicine-Resistant form of Familial Mediterranean Fever on the Canakinumab Therapy: Clinical Case

Background
Отсутствие активности заболевания
СПИСОК ЛИТЕРАТУРЫ
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