Complement Receptor Deficiency Workup

1. Kubagawa H, Kubagawa Y, Jones D, Nasti TH, Walter MR, Honjo K. The Old but New IgM Fc Receptor (FcµR). Curr Top Microbiol Immunol. 2014. 382:3-28. [QxMD MEDLINE Link].

2. Weaver DJ Jr, Reis ES, Pandey MK, Köhl G, Harris N, Gerard C, et al. C5a receptor-deficient dendritic cells promote induction of Treg and Th17 cells. Eur J Immunol. 2010 Mar. 40(3):710-21. [QxMD MEDLINE Link].

3. Ghannam A, Fauquert JL, Thomas C, Kemper C, Drouet C. Human complement C3 deficiency: Th1 induction requires T cell-derived complement C3a and CD46 activation. Mol Immunol. 2014 Mar. 58(1):98-107. [QxMD MEDLINE Link].

4. Fairweather D, Frisancho-Kiss S, Njoku DB, Nyland JF, Kaya Z, Yusung SA, et al. Complement receptor 1 and 2 deficiency increases coxsackievirus B3-induced myocarditis, dilated cardiomyopathy, and heart failure by increasing macrophages, IL-1beta, and immune complex deposition in the heart. J Immunol. 2006 Mar 15. 176(6):3516-24. [QxMD MEDLINE Link].

5. Jacobson AC, Weis JJ, Weis JH. Complement receptors 1 and 2 influence the immune environment in a B cell receptor-independent manner. J Immunol. 2008 Apr 1. 180(7):5057-66. [QxMD MEDLINE Link].

6. Boos L, Campbell IL, Ames R, Wetsel RA, Barnum SR. Deletion of the complement anaphylatoxin C3a receptor attenuates, whereas ectopic expression of C3a in the brain exacerbates, experimental autoimmune encephalomyelitis. J Immunol. 2004 Oct 1. 173(7):4708-14. [QxMD MEDLINE Link].

7. Crider A, Feng T, Pandya CD, Davis T, Nair A, Ahmed AO, et al. Complement component 3a receptor deficiency attenuates chronic stress-induced monocyte infiltration and depressive-like behavior. Brain Behav Immun. 2018 May. 70:246-256. [QxMD MEDLINE Link].

8. Nikiforovich GV, Baranski TJ. Structural mechanisms of constitutive activation in the C5a receptors with mutations in the extracellular loops: molecular modeling study. Proteins. 2012 Jan. 80(1):71-80. [QxMD MEDLINE Link]. [Full Text].

9. Yu S, Wang D, Huang L, Zhang Y, Luo R, Adah D, et al. The complement receptor C5aR2 promotes protein kinase R expression and contributes to NLRP3 inflammasome activation and HMGB1 release from macrophages. J Biol Chem. 2019 Apr 10. [QxMD MEDLINE Link].

10. Nabizadeh JA, Manthey HD, Steyn FJ, Chen W, Widiapradja A, Md Akhir FN, et al. The Complement C3a Receptor Contributes to Melanoma Tumorigenesis by Inhibiting Neutrophil and CD4+ T Cell Responses. J Immunol. 2016 Jun 1. 196 (11):4783-92. [QxMD MEDLINE Link].

11. Tone Y, Wada T, Shibata F, Toma T, Hashida Y, Kasahara Y, et al. Somatic revertant mosaicism in a patient with leukocyte adhesion deficiency type 1. Blood. 2007 Feb 1. 109(3):1182-4. [QxMD MEDLINE Link].

12. Svensson L, Howarth K, McDowall A, Patzak I, Evans R, Ussar S, et al. Leukocyte adhesion deficiency-III is caused by mutations in KINDLIN3 affecting integrin activation. Nat Med. 2009 Mar. 15(3):306-12. [QxMD MEDLINE Link].

13. Yashoda-Devi BK, Rakesh N, Devaraju D, Santana N. Leukocyte adhesion deficiency type I--a focus on oral disease in a young child. Med Oral Patol Oral Cir Bucal. 2011 Mar 1. 16(2):e153-7. [QxMD MEDLINE Link].

14. Fearon DT. Identification of the membrane glycoprotein that is the C3b receptor of the human erythrocyte, polymorphonuclear leukocyte, B lymphocyte, and monocyte. J Exp Med. 1980 Jul 1. 152(1):20-30. [QxMD MEDLINE Link].

15. Asokan R, Banda NK, Szakonyi G, Chen XS, Holers VM. Human complement receptor 2 (CR2/CD21) as a receptor for DNA: implications for its roles in the immune response and the pathogenesis of systemic lupus erythematosus (SLE). Mol Immunol. 2013 Jan. 53(1-2):99-110. [QxMD MEDLINE Link]. [Full Text].

16. Dragon-Durey MA, Blanc C, Marinozzi MC, van Schaarenburg RA, Trouw LA. Autoantibodies against complement components and functional consequences. Mol Immunol. 2013 Dec 15. 56(3):213-21. [QxMD MEDLINE Link].

17. Sommerfeld O, Medyukhina A, Neugebauer S, Ghait M, Ulferts S, Lupp A, et al. Targeting Complement C5a Receptor 1 for the Treatment of Immunosuppression in Sepsis. Mol Ther. 2021 Jan 6. 29 (1):338-346. [QxMD MEDLINE Link].

18. Nord KM, Pappert AS, Grossman ME. Pyoderma Gangrenosum-like Lesions in Leukocyte Adhesion Deficiency I Treated with Intravenous Immunoglobulin. Pediatr Dermatol. 2011 Mar. 28(2):156-61. [QxMD MEDLINE Link].

19. Bautsch W, Hoymann HG, Zhang Q, Meier-Wiedenbach I, Raschke U, Ames RS, et al. Cutting edge: guinea pigs with a natural C3a-receptor defect exhibit decreased bronchoconstriction in allergic airway disease: evidence for an involvement of the C3a anaphylatoxin in the pathogenesis of asthma. J Immunol. 2000 Nov 15. 165(10):5401-5. [QxMD MEDLINE Link].

20. Kallenberg CG. Pathogenesis and treatment of ANCA-associated vasculitides. Clin Exp Rheumatol. 2015 Oct 12. [QxMD MEDLINE Link].

21. Merkel PA, Jayne DR, Wang C, Hillson J, Bekker P. Evaluation of the Safety and Efficacy of Avacopan, a C5a Receptor Inhibitor, in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Treated Concomitantly With Rituximab or Cyclophosphamide/Azathioprine: Protocol for a Randomized, Double-Blind, Active-Controlled, Phase 3 Trial. JMIR Res Protoc. 2020 Apr 7. 9 (4):e16664. [QxMD MEDLINE Link].

22. Qasim W, Cavazzana-Calvo M, Davies EG, Davis J, Duval M, Eames G, et al. Allogeneic hematopoietic stem-cell transplantation for leukocyte adhesion deficiency. Pediatrics. 2009 Mar. 123(3):836-40. [QxMD MEDLINE Link].

23. Moutsopoulos NM, Zerbe CS, Wild T, Dutzan N, Brenchley L, DiPasquale G, et al. Interleukin-12 and Interleukin-23 Blockade in Leukocyte Adhesion Deficiency Type 1. N Engl J Med. 2017 Mar 23. 376 (12):1141-1146. [QxMD MEDLINE Link].

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Isabelle Thomas, MD Associate Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School; Chief of Dermatology Service, Veterans Affairs Medical Center of East Orange

Isabelle Thomas, MD is a member of the following medical societies: American Academy of Dermatology, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article