Research & Literature

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

Turley AJ, Gathmann B, Bangs C, et al.

Journal of clinical immunology 2015; (35(2)):199-205 doi:10.1007/s10875-015-0137-5.

Case report: Benefits and challenges of long-term eculizumab in atypical hemolytic uremic syndrome.

Cullinan N, Gorman KM, Riordan M, et al.

Pediatrics 2015; (135(6)):e1506-9 doi:10.1542/peds.2014-3503.

C1q protein binds to the apoptotic nucleolus and causes C1 protease degradation of nucleolar proteins.

Cai Y, Teo BH, Yeo JG, Lu J

The Journal of biological chemistry 2015; (290(37)):22570-80 doi:10.1074/jbc.M115.670661.

Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway.

Macedo AC, Isaac L

Frontiers in immunology 2016; (7()):55 doi:10.3389/fimmu.2016.00055.

Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients.

Audemard-Verger A, Descloux E, Ponard D, et al.

Medicine 2016; (95(19)):e3548 doi:10.1097/MD.0000000000003548.

The case for a national service for primary immune deficiency disorders in New Zealand.

Ameratunga R, Steele R, Jordan A, et al.

The New Zealand medical journal 2016; (129(1436)):75-90.

Proteolytic inactivation of nuclear alarmin high-mobility group box 1 by complement protease C1s during apoptosis.

Yeo JG, Leong J, Arkachaisri T, et al.

Cell death discovery 2016; (2()):16069 doi:10.1038/cddiscovery.2016.69.

C1 Complex: An Adaptable Proteolytic Module for Complement and Non-Complement Functions.

Lu J, Kishore U

Frontiers in immunology 2017; (8()):592 doi:10.3389/fimmu.2017.00592.

The complement system as a potential therapeutic target in rheumatic disease.

Trouw LA, Pickering MC, Blom AM

Nature reviews. Rheumatology 2017; (13(9)):538-547 doi:10.1038/nrrheum.2017.125.

Periodontal manifestations of Ehlers-Danlos syndromes: A systematic review.

Kapferer-Seebacher I, Lundberg P, Malfait F, Zschocke J

Journal of clinical periodontology 2017; (44(11)):1088-1100 doi:10.1111/jcpe.12807.

Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation.

Cai Y, Wee SYK, Chen J, et al.

Journal of immunology (Baltimore, Md. : 1950) 2017; (199(12)):3981-3990 doi:10.4049/jimmunol.1700728.

Complement C5a Receptor 1 Exacerbates the Pathophysiology of N. meningitidis Sepsis and Is a Potential Target for Disease Treatment.

Herrmann JB, Muenstermann M, Strobel L, et al.

mBio 2018; (9(1)) doi:10.1128/mBio.01755-17.

Functional Complement Analysis Can Predict Genetic Testing Results and Long-Term Outcome in Patients With Complement Deficiencies.

Blazina Š, Debeljak M, Košnik M, et al.

Frontiers in immunology 2018; (9()):500 doi:10.3389/fimmu.2018.00500.

Analyzing complement activity in the serum and body homogenates of different fish species, using rabbit and sheep red blood cells.

Nayak S, Portugal I, Zilberg D

Veterinary immunology and immunopathology 2018; (199()):39-42 doi:10.1016/j.vetimm.2018.03.008.

Meningococcal B Vaccine Immunogenicity in Children With Defects in Complement and Splenic Function.

Martinón-Torres F, Bernatowska E, Shcherbina A, et al.

Pediatrics 2018; (142(3)) doi:10.1542/peds.2017-4250.

Inherited Classical and Alternative Pathway Complement Deficiencies in Children: A Single Center Experience.

Genel F, Erdem SB, Gülez N, et al.

Iranian journal of immunology : IJI 2018; (15(4)):309-320 doi:10.22034/IJI.2018.39400.

Development and Optimization of an ELISA to Quantitate C3(H O) as a Marker of Human Disease.

Elvington M, Liszewski MK, Liszewski AR, et al.

Frontiers in immunology 2019; (10()):703 doi:10.3389/fimmu.2019.00703.

Clinical and Genetic Spectrum of a Large Cohort With Total and Sub-total Complement Deficiencies.

El Sissy C, Rosain J, Vieira-Martins P, et al.

Frontiers in immunology 2019; (10()):1936 doi:10.3389/fimmu.2019.01936.

C1R Mutations Trigger Constitutive Complement 1 Activation in Periodontal Ehlers-Danlos Syndrome.

Gröbner R, Kapferer-Seebacher I, Amberger A, et al.

Frontiers in immunology 2019; (10()):2537 doi:10.3389/fimmu.2019.02537.

European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) Complement Guideline: Deficiencies, Diagnosis, and Management.

Brodszki N, Frazer-Abel A, Grumach AS, et al.

Journal of clinical immunology 2020; (40(4)):576-591 doi:10.1007/s10875-020-00754-1.

Parallel comparison of three methodologies for measuring functional C1-inhibitor in Hereditary angioedema patients.

Kapoor A, Garg BK, Zhou Z, et al.

International immunopharmacology 2020; (81()):106348 doi:10.1016/j.intimp.2020.106348.

Hereditary Deficiency of the Second Component of Complement: Early Diagnosis and 21-Year Follow-Up of a Family.

Dellepiane RM, Baselli LA, Cazzaniga M, et al.

Medicina (Kaunas, Lithuania) 2020; (56(3)) doi:10.3390/medicina56030120.

Revisiting the complement system in systemic lupus erythematosus.

Sharma M, Vignesh P, Tiewsoh K, Rawat A

Expert review of clinical immunology 2020; (16(4)):397-408 doi:10.1080/1744666X.2020.1745063.

Patterns of C1-Inhibitor/Plasma Serine Protease Complexes in Healthy Humans and in Hereditary Angioedema Patients.

Kajdácsi E, Jandrasics Z, Veszeli N, et al.

Frontiers in immunology 2020; (11()):794 doi:10.3389/fimmu.2020.00794.

Meningococcaemia causing necrotizing cellulitis associated with acquired complement deficiency after gastric bypass surgery: a case report.

Pletschette Z, De Groote E, Mattheus W, et al.

BMC infectious diseases 2020; (20(1)):361 doi:10.1186/s12879-020-05079-3.

Headless C1q: a new molecular tool to decipher its collagen-like functions.

Fouët G, Bally I, Signor L, et al.

The FEBS journal 2021; (288(6)):2030-2041 doi:10.1111/febs.15543.

Prospective clinical investigations of children with periodontal Ehlers-Danlos syndrome identify generalized lack of attached gingiva as a pathognomonic feature.

Kapferer-Seebacher I, Oakley-Hannibal E, Lepperdinger U, et al.

Genetics in medicine : official journal of the American College of Medical Genetics 2021; (23(2)):316-322 doi:10.1038/s41436-020-00985-y.

Meningococcal Vaccination: Recommendations of the Advisory Committee on Immunization Practices, United States, 2020.

Mbaeyi SA, Bozio CH, Duffy J, et al.

MMWR. Recommendations and reports : Morbidity and mortality weekly report. Recommendations and reports 2020; (69(9)):1-41 doi:10.15585/mmwr.rr6909a1.

C1q as a potential tolerogenic therapeutic in transplantation.

Baldwin WM, Valujskikh A, Fairchild RL

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2021; (21(11)):3519-3523 doi:10.1111/ajt.16705.

Circulating C1q levels in health and disease, more than just a biomarker.

van de Bovenkamp FS, Dijkstra DJ, van Kooten C, et al.

Molecular immunology 2021; (140()):206-216 doi:10.1016/j.molimm.2021.10.010.

The Alterations in Methylene Blue/Light-Treated Frozen Plasma Proteins Revealed by Proteomics.

Wu T, Wang X, Ren K, et al.

Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie 2021; (48(5)):298-305 doi:10.1159/000515119.

Clinical Outcome and Underlying Genetic Cause of Functional Terminal Complement Pathway Deficiencies in a Multicenter UK Cohort.

Shears A, Steele C, Craig J, et al.

Journal of clinical immunology 2022; (42(3)):665-671 doi:10.1007/s10875-022-01213-9.

The yin and the yang of early classical pathway complement disorders.

Sullivan KE

Clinical and experimental immunology 2022; (209(2)):151-160 doi:10.1093/cei/uxac056.

Short- and Long-Term Progression of Kidney Involvement in Systemic Lupus Erythematosus Patients with Low-Grade Proteinuria.

Wang S, Spielman A, Ginsberg M, et al.

Clinical journal of the American Society of Nephrology : CJASN 2022; (17(8)):1150-1158 doi:10.2215/CJN.01280122.

A Review of Lupus Nephritis.

Alforaih N, Whittall-Garcia L, Touma Z

The journal of applied laboratory medicine 2022; (7(6)):1450-1467 doi:10.1093/jalm/jfac036.

Streptococcus pneumoniae interactions with the complement system.

Gil E, Noursadeghi M, Brown JS

Frontiers in cellular and infection microbiology 2022; (12()):929483 doi:10.3389/fcimb.2022.929483.

Complement C1s as a diagnostic marker and therapeutic target: Progress and propective.

Ye J, Yang P, Yang Y, Xia S

Frontiers in immunology 2022; (13()):1015128 doi:10.3389/fimmu.2022.1015128.

Vaccination status in patients at risk for invasive disease with encapsulated bacteria at a children's hospital in the City of Buenos Aires.

Del Pino M, Castellano VE, Pacchiotti AC, et al.

Archivos argentinos de pediatria 2023; (121(1)):e202202648 doi:10.5546/aap.2022-02648.eng.

A homozygous loss-of-function C1S mutation is associated with Kikuchi-Fujimoto disease.

Alshekaili J, Nasr I, Al-Rawahi M, et al.

Clinical immunology (Orlando, Fla.) 2023; (252()):109646 doi:10.1016/j.clim.2023.109646.

[Reducing the risk of infections in hereditary and acquired complement deficiencies.]

Karászi É, Onozó B, Kulcsár A, et al.

Orvosi hetilap 2023; (164(25)):971-980 doi:10.1556/650.2023.32798.

The axis of complement C1 and nucleolus in antinuclear autoimmunity.

Wu S, Chen J, Teo BHD, et al.

Frontiers in immunology 2023; (14()):1196544 doi:10.3389/fimmu.2023.1196544.

Differentiating between activation via the lectin or the classical complement pathway in patients with systemic lupus erythematosus.

Larsen ML, Troldborg A, Toonen EJM, et al.

Clinical and experimental immunology 2023; (214(1)):18-25 doi:10.1093/cei/uxad070.

Complement C1s deficiency in a male Caucasian patient with systemic lupus erythematosus: a case report.

Kleer JS, Skattum L, Dubler D, et al.

Frontiers in immunology 2023; (14()):1257525 doi:10.3389/fimmu.2023.1257525.

Immunodeficiency: Complement disorders.

McMurray JC, Schornack BJ, Weskamp AL, et al.

Allergy and asthma proceedings 2024; (45(5)):305-309 doi:10.2500/aap.2024.45.240050.

Silent lupus nephritis: a pediatric center experience of baseline kidney biopsies and review of the published evidence.

Laestadius A, Grisaru S, Twilt M, et al.

Pediatric nephrology (Berlin, Germany) 2025; (40(9)):2837-2844 doi:10.1007/s00467-025-06737-x.

Impact of a Heterozygous C1RR301P/WT Mutation on Collagen Metabolism and Inflammatory Response in Human Gingival Fibroblasts.

Qu C, Koskinen Holm C

Cells 2025; (14(7)) doi:10.3390/cells14070479.

Biomarkers in Lupus Nephritis: An Evidence-Based Comprehensive Review.

Vrabie A, Obrișcă B, Sorohan BM, Ismail G

Life (Basel, Switzerland) 2025; (15(10)) doi:10.3390/life15101497.

Invasive Meningococcal Disease in a Patient With Complement 7 Deficiency.

Nishioka H, Maegawa K, Hara S

Journal of general and family medicine 2025; (26(6)):646-649 doi:10.1002/jgf2.70075.

Use of the GSK MenACWY-CRM/MenB-4C Pentavalent Meningococcal Vaccine Among Persons Aged ≥10 Years: Recommendations of the Advisory Committee on Immunization Practices - United States, 2025.

Amin AB, Collins JP, Dong X, et al.

MMWR. Morbidity and mortality weekly report 2026; (75(1)):7-14 doi:10.15585/mmwr.mm7501a2.

Recurrent Pneumococcal Meningitis From Post-traumatic Frontoethmoidal Encephalocele: A Case Report.

Mazen Y, Darweesh A, Al Ani M, et al.

Cureus 2025; (17(12)):e98862 doi:10.7759/cureus.98862.

Invasive Non-groupable Meningococcal Disease Complicated by Brain Abscess Revealing C6 Complement Deficiency in a Young Adult.

Caceres-Cardenas G, Bove A, Ortega J, Berger J

Cureus 2026; (18(8)):e114004 doi:10.7759/cureus.114004.