DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER

Abstract. During attacks of familial mediterranean fever (FMF), multiple systemic events are triggered, most of which promote autoinflammatory reactions. A molecular pattern of immune abnormalities in FMF is yet unclear. There is an increasing evidence to suggest an involvement of the complement sys...

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Autores principales:	G. M. Mkrtchyan, L. P. Hovhannisyan, A. A. Ayvazyan, E. Y. Nazaretyan, A. S. Boyajyan
Formato:	article
Lenguaje:	RU
Publicado:	SPb RAACI 2014
Materias:	alternative complement pathway classic complement pathway familial mediterranean fever Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/c707b270c16e44c184e53f834f5c00c1
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spelling	oai:doaj.org-article:c707b270c16e44c184e53f834f5c00c12021-11-18T08:03:38ZDYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER1563-06252313-741X10.15789/1563-0625-2009-1-85-90https://doaj.org/article/c707b270c16e44c184e53f834f5c00c12014-07-01T00:00:00Zhttps://www.mimmun.ru/mimmun/article/view/221https://doaj.org/toc/1563-0625https://doaj.org/toc/2313-741XAbstract. During attacks of familial mediterranean fever (FMF), multiple systemic events are triggered, most of which promote autoinflammatory reactions. A molecular pattern of immune abnormalities in FMF is yet unclear. There is an increasing evidence to suggest an involvement of the complement system, the major inflammatory mediator, in FMF pathogeneses. In present study, we examined functional activities of the alternative and the classical complement cascades, and some relationships between alterations in the functional activities of these cascades in FMF. To this purpose, we measured hemolytic activities of classic (CH50) and alternative complement pathways (AH50), and of the complement components C3 (C3H50), factor B (fBH50) and factor D (fDH50) in blood serum of twenty-eight colchicine-free FMF patients and twenty-five healthy subjects. According to the data obtained, a decrease in serum levels of AH50 and increase in CH50 and C3H50 were detected in FMF patients, as compared to normal values. No significant difference was detected between the affected persons and healthy subjects for fBH50 and fDH50. Correlation analysis revealed a positive relationship between alterations in CH50 and C3H50 and a negative correlation between alterations in AH50 and CH50. From the data obtained, following conclusions have been made: 1) pathogenesis of FMF is characterized by a complement dysfunction, including hyperactivation of classical complement pathway and hypoactivation state of alternative pathway; 2) alterations in functional activities of classical and alternative complement activation pathways in FMF are interdependent; 3) the alternative pathway is suppressed on the initial stage of its activation; 4) high blood levels of C-reactive protein, serum amyloid P component, and circulating immune complexes, associated with FMF, might be responsible for hyperactivation of classical complement pathway in this disease.G. M. MkrtchyanL. P. HovhannisyanA. A. AyvazyanE. Y. NazaretyanA. S. BoyajyanSPb RAACIarticlealternative complement pathwayclassic complement pathwayfamilial mediterranean feverImmunologic diseases. AllergyRC581-607RUMedicinskaâ Immunologiâ, Vol 11, Iss 1, Pp 85-90 (2014)
institution	DOAJ
collection	DOAJ
language	RU
topic	alternative complement pathway classic complement pathway familial mediterranean fever Immunologic diseases. Allergy RC581-607
spellingShingle	alternative complement pathway classic complement pathway familial mediterranean fever Immunologic diseases. Allergy RC581-607 G. M. Mkrtchyan L. P. Hovhannisyan A. A. Ayvazyan E. Y. Nazaretyan A. S. Boyajyan DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
description	Abstract. During attacks of familial mediterranean fever (FMF), multiple systemic events are triggered, most of which promote autoinflammatory reactions. A molecular pattern of immune abnormalities in FMF is yet unclear. There is an increasing evidence to suggest an involvement of the complement system, the major inflammatory mediator, in FMF pathogeneses. In present study, we examined functional activities of the alternative and the classical complement cascades, and some relationships between alterations in the functional activities of these cascades in FMF. To this purpose, we measured hemolytic activities of classic (CH50) and alternative complement pathways (AH50), and of the complement components C3 (C3H50), factor B (fBH50) and factor D (fDH50) in blood serum of twenty-eight colchicine-free FMF patients and twenty-five healthy subjects. According to the data obtained, a decrease in serum levels of AH50 and increase in CH50 and C3H50 were detected in FMF patients, as compared to normal values. No significant difference was detected between the affected persons and healthy subjects for fBH50 and fDH50. Correlation analysis revealed a positive relationship between alterations in CH50 and C3H50 and a negative correlation between alterations in AH50 and CH50. From the data obtained, following conclusions have been made: 1) pathogenesis of FMF is characterized by a complement dysfunction, including hyperactivation of classical complement pathway and hypoactivation state of alternative pathway; 2) alterations in functional activities of classical and alternative complement activation pathways in FMF are interdependent; 3) the alternative pathway is suppressed on the initial stage of its activation; 4) high blood levels of C-reactive protein, serum amyloid P component, and circulating immune complexes, associated with FMF, might be responsible for hyperactivation of classical complement pathway in this disease.
format	article
author	G. M. Mkrtchyan L. P. Hovhannisyan A. A. Ayvazyan E. Y. Nazaretyan A. S. Boyajyan
author_facet	G. M. Mkrtchyan L. P. Hovhannisyan A. A. Ayvazyan E. Y. Nazaretyan A. S. Boyajyan
author_sort	G. M. Mkrtchyan
title	DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
title_short	DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
title_full	DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
title_fullStr	DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
title_full_unstemmed	DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER
title_sort	dysfunction of complement system in familial mediterranean fever
publisher	SPb RAACI
publishDate	2014
url	https://doaj.org/article/c707b270c16e44c184e53f834f5c00c1
work_keys_str_mv	AT gmmkrtchyan dysfunctionofcomplementsysteminfamilialmediterraneanfever AT lphovhannisyan dysfunctionofcomplementsysteminfamilialmediterraneanfever AT aaayvazyan dysfunctionofcomplementsysteminfamilialmediterraneanfever AT eynazaretyan dysfunctionofcomplementsysteminfamilialmediterraneanfever AT asboyajyan dysfunctionofcomplementsysteminfamilialmediterraneanfever
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DYSFUNCTION OF COMPLEMENT SYSTEM IN FAMILIAL MEDITERRANEAN FEVER

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