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Epic Code LAB734 Complement C1q, Serum

Important Note

Fasting specimen required

Additional Codes

Mayo Code: C1Q

Epic Code: LAB 734

Interface Order Alias: 10343

Cerner code: 4179

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Useful For

Assessment of an undetectable total complement (CH50) level

 

Diagnosing congenital C1 (first component of complement) deficiency

 

Diagnosing acquired deficiency of C1 inhibitor

Specimen Type

Serum


Specimen Required


Patient Preparation: Fasting for 12 hours

Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions: Centrifuge and aliquot serum into plastic vial


Laboratory Test Directory Note:

COLLECTION NOTE: Volumes listed are in serum or plasma, draw approximately 2 1/2 times the requested volume in whole blood.

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 28 days
  Frozen  28 days
  Ambient  21 days

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Day(s) Performed

Monday through Friday

Reference Values

12-22 mg/dL

Clinical Information

The first component of complement (C1) is composed of 3 subunits designated as C1q, C1r, and C1s. C1q recognizes and binds to immunoglobulin complexed to antigen and initiates the complement cascade. Congenital deficiencies of any of the early complement components (C1, C2, C4) results in an inability to clear immune complexes. Inherited deficiency of C1 is rare.

 

Like the more common C2 deficiency, C1 deficiency is associated with increased incidence of immune complex disease (systemic lupus erythematosus, polymyositis, glomerulonephritis, and Henoch-Schonlein purpura). Low C1 levels have also been reported in patients with abnormal immunoglobulin levels (Bruton and common variable hypogammaglobulinemia and severe combined immunodeficiency). This is most likely due to increased catabolism.

 

The measurement of C1q is an indicator of the amount of C1 present.

Cautions

This is a different assay than C1q binding, which is an assay for circulating immune complexes.

Interpretation

An undetectable C1q in the presence of an absent total complement (CH50) and normal C2, C3, and C4 suggests a congenital C1 (first component of complement) deficiency.

 

A low C1q in combination with a low C1 inhibitor and low C4 suggests an acquired C1 inhibitor deficiency.

Reporting Name

Complement C1q, S

Method Name

Nephelometry

Method Description

Nephelometry and anti- C1q antiserum are used to quantitate the C1q antigen level.(Instruction manual: Siemens Nephelometer II. Siemens, Inc; Version 2.3, 2008; Addendum to the Instruction Manual 2.3, 08/2017)

CPT Code Information

86160

LOINC Code Information

Test ID Test Order Name Order LOINC Value
C1Q Complement C1q, S 4478-4

 

Result ID Test Result Name Result LOINC Value
C1Q Complement C1q, S 4478-4

Report Available

2 to 5 days

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

Clinical Reference

1. Stegert M, Bock M, Trendelenburg M: Clinical presentation of human C1q deficiency: How much of a lupus? Mol Immunol. 2015 Sep;67(1):3-11

2. Tangye SG, Al-Herz W, Bousfiha A, et al: Human inborn errors of immunity: 2022 update on the classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022 Oct;42(7):1473-1507

3. Beurskens FJ, van Schaarenburg RA, Trouw LA: C1q, antibodies and anti-C1q autoantibodies. Mol Immunol. 2015 Nov;68(1):6-13