Investigating an undetectable total complement (CH50)
Confirming hereditary angioedema (with low C1 inhibitor)
Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia
Nephelometry
C4 (4th Component of Complement), Serum
C4 (Fourth Component of Complement), Serum
C4 Complement Serum
C4, Serum
Complement C4, Serum
Fourth Component of Complement
Hereditary Angioedema Panel (3 tests)
Serum
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 1 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
If not ordering electronically, complete, print, and send a Renal Diagnostics Test Request (T830) with the specimen.
0.5 mL
Gross hemolysis | OK |
Gross lipemia | Reject |
Gross icterus | OK |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 7 days | |
Frozen | 28 days | ||
Ambient | 72 hours |
Investigating an undetectable total complement (CH50)
Confirming hereditary angioedema (with low C1 inhibitor)
Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia
The complement system is an integral part of the immune defenses. It can be activated via immune complexes (classic pathway) or by bacterial polysaccharides (alternative pathway). The classic complement pathway consists of recognition, (C1q, C1r, C1s), activation (C2, C3, C4), and attack (C5, C6, C7, C8, C9) mechanisms with respect to their role in antibody-mediated cytolysis. C4 is one of the activation proteins of the classic pathway.
In the absence of C4, immune complexes will not be cleared by C3 activation peptides, but bacterial infections can still be defended via the alternative pathway.
C4 may be decreased in systemic lupus erythematosus, early glomerulonephritis, immune complex disease, cryoglobulinemia, hereditary angioedema, and congenital C4 deficiency.
14-40 mg/dL
C4 levels will be decreased in acquired autoimmune disorders, in the active phase of lupus erythematosus, and in rheumatoid arthritis.
An undetectable C4 level (with normal C3) suggests a congenital C4 deficiency.
Levels will be increased in patients with autoimmune hemolytic anemia.
The results are dependent on appropriate specimen transport.
1. Willrich MAV, Braun KMP, Moyer AM, Jeffrey DH, Frazer-Abel A: Complement testing in the clinical laboratory. Crit Rev Clin Lab Sci. 2021 Nov;58(7):447-478. doi: 10.1080/10408363.2021.19072972
2. Wong EKS, Kavanagh D: Diseases of complement dysregulation-an overview. Semin Immunopathol. 2018 Jan;40(1):49-64. doi: 10.1007/s00281-017-0663-8
3. Prohaszka Z, Kirschfink M, Frazer-Abel A: Complement analysis in the era of targeted therapeutics. Mol Immunol. 2018 Oct;102:84-88. doi: 10.1016/j.molimm.2018.06.001
4. Brodszki N, Frazer-Abel A, Grumach AS, et al: 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. J Clin Immunol. 2020 May;40(4):576-591. doi: 10.1007/s10875-020-00754-1
C4 is measured by immunonephelometry. Antiserum to C4 is mixed with patient serum, the light scatter resulting from the antibody interaction with C4 is measured, and the signal is compared to standard concentrations of C4.(Instruction manual: Siemens Nephelometer II Operations. Siemens, Inc; Version 2.4, 07/2019; Addendum to the Instruction Manual 2.3, 08/2017)
Monday through Friday
This test has been cleared, approved, or is exempt by the US Food and Drug Administration and is used per manufacturer's instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements.
86160
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
C4 | Complement C4, S | 4498-2 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
C4 | Complement C4, S | 4498-2 |