Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach

AS Grumach, M Kirschfink - Molecular immunology, 2014 - Elsevier
Complement deficiencies comprise between 1 and 10% of all primary immunodeficiencies
(PIDs) according to national and supranational registries. They are still considered rare and …

European society for immunodeficiencies (ESID) and European reference network on rare primary immunodeficiency, autoinflammatory and autoimmune diseases …

N Brodszki, A Frazer-Abel, AS Grumach… - Journal of clinical …, 2020 - Springer
This guideline aims to describe the complement system and the functions of the constituent
pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis …

Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management

J Schröder-Braunstein, M Kirschfink - Molecular immunology, 2019 - Elsevier
Complement defects are associated with an enhanced risk of a broad spectrum of infectious
as well as systemic or local inflammatory and thrombotic disorders. Inherited complement …

Clinical and genetic spectrum of a large cohort with total and sub-total complement deficiencies

C El Sissy, J Rosain, P Vieira-Martins… - Frontiers in …, 2019 - frontiersin.org
The complement system is crucial for defense against pathogens and the removal of dying
cells or immune complexes. Thus, clinical indications for possible complete complement …

Complement deficiency and disease: an update

AG Sjöholm, G Jönsson, JH Braconier, G Sturfelt… - Molecular …, 2006 - Elsevier
Complement deficiencies are probably vastly under-diagnosed within clinical medicine.
Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of …

Translational mini-review series on complement factor H: renal diseases associated with complement factor H: novel insights from humans and animals

MC Pickering, HT Cook - Clinical & Experimental Immunology, 2008 - academic.oup.com
Summary OTHER ARTICLES PUBLISHED IN THIS TRANSLATIONAL MINI-REVIEW
SERIES ON COMPLEMENT FACTOR H Genetics and disease associations of human …

Inherited kidney complement diseases

M Lemaire, D Noone, AL Lapeyraque… - Clinical Journal of the …, 2021 - journals.lww.com
In the past 20 years, we have witnessed tremendous advances in our ability to diagnose and
treat genetic diseases of the kidney caused by complement dysregulation. Staggering …

Complement in human diseases: lessons from complement deficiencies

M Botto, M Kirschfink, P Macor, MC Pickering… - Molecular …, 2009 - Elsevier
Complement deficient cases reported in the second half of the last century have been of
great help in defining the role of complement in host defence. Surveys of the deficient …

Complement diagnostics: concepts, indications, and practical guidelines

B Nilsson, KN Ekdahl - Journal of Immunology Research, 2012 - Wiley Online Library
Aberrations in the complement system have been shown to be direct or indirect
pathophysiological mechanisms in a number of diseases and pathological conditions such …

Spectrum and management of complement immunodeficiencies (excluding hereditary angioedema) across Europe

AJ Turley, B Gathmann, C Bangs, M Bradbury… - Journal of clinical …, 2015 - Springer
Introduction Complement immunodeficiencies (excluding hereditary angioedema and
mannose binding lectin deficiency) are rare. Published literature consists largely of case …