Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome

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Background: The role of the alternative complement pathway (AP) abnormalities in the pathogenesis of aHUS is well studied. Clinical and morphological manifestations of atypical HUS and catastrophic APS are often similar. However, studies on the state of AP in patients with CAPS are virtually absent. 

Aims: The aim of our study was to assess the state of AP in patients with CAPS and aHUS. Patients and methods: The study enrolled 67 patients (pts) with a diagnosis of CAPS (28 pts) and aHUS (39 pts). Studies of the complement system are made of 10 pts with CAPS and 20 aHUS. Factor H, I, B, D content, functional activity of factor H, and complement components C3, C4 was determined in serum by ELISA kit. 

Results: Patients with CAPS and aHUS showed similar changes in complement biomarkers. The factor H level in the serum was significantly higher than the standard value. However, the specific activity of factor H reduced, mean rate 59% for aHUS and 26% for CAPS. The median value of factor D was twice higher than the normal range in both groups, indicating the activation of the AP. 

Conclusions: There are indications of an AP activation not only in pts with aHUS but in CAPS pts too. We suppose that the activity of factor H is a more sensitive indicator of complement system changes than factor H level. Patients with CAPS and aHUS have similar clinical and laboratory characteristics. However, CAPS is more severe, with the involvement of a larger number of vascular beds. Perhaps this is due to the double damaging effects on the endothelium ― of antiphospholipid antibodies (aPL) and activated complement. So we hypothesize that CAPS can be called aPL-mediated TMA in pts with a complement system defect.

About the authors

K. A. Demyanova

Sechenov First Moscow State Medical University

Author for correspondence.
ORCID iD: 0000-0001-8927-5841

Аспирант кафедра нефрологии и гемодиализа института профессионального образования. 

SPIN-код: 5474-1091

Russian Federation

N. L. Kozlovskaya

Sechenov First Moscow State Medical University

ORCID iD: 0000-0002-4275-0315

Доктор медицинских наук, профессор кафедры нефрологии и гемодиализа института профессионального образования 

SPIN 1110-4764

Russian Federation

L. A. Bobrova

Sechenov First Moscow State Medical University

ORCID iD: 0000-0001-6265-4091

Кандидат медицинских наук, старший научный сотрудник Научно-исследовательского отдела нефрологии НИЦ 

SPIN 7435-1504

Russian Federation

L. V. Kozlov

G.N. Gabrichevsky Moscow Research Institute of Epidemiology and Microbiology

Доктор биологических наук, до марта 2016 г. ― профессор, руководитель группы  Russian Federation

S. S. Andina

G.N. Gabrichevsky Moscow Research Institute of Epidemiology and Microbiology

ORCID iD: 0000-0002-0284-3787

Кандидат биологических наук, старший научный сотрудник 

SPIN-код: 8479-9628

Russian Federation

V. A. Yurova

Sechenov First Moscow State Medical University

ORCID iD: 0000-0002-7041-3391

Аспирант кафедры нефрологии и гемодиализа института профессионального образования 

SPIN-код: 3906-3609

Russian Federation

A. M. Kuchieva

Sechenov First Moscow State Medical University

ORCID iD: 0000-0002-8360-7734

Врач-нефролог отделения искусственная почка Университетской клинической больницы № 3 

SPIN-код: 3292-8178

Russian Federation

S. V. Roshchupkina

Sechenov First Moscow State Medical University

ORCID iD: 0000-0003-4695-1568

Зав.отделения нефрологии Университетской клинической больницы № 3, врач высшей категории

 SPIN-код: 4611-8464

Russian Federation

E. M. Shilov

Sechenov First Moscow State Medical University

ORCID iD: 0000-0002-2111-191X

Доктор медицинских наук, профессор, заведующий кафедрой нефрологии и гемодиализа института профессионального образования

SPIN: 1538-9845


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