Primary Biliary Cholangitis (PBC) Diagnostic Panel

Test Code

19876

CPT Codes
86015, 86038, 86235 (x2), 86381, 86376 (x2)

Includes

ANA Screen, IFA, with Reflex to Titer and Pattern
Sjögren's Antibodies (SS-A, SS-B)
Mitochondrial Antibody with Reflex to Titer
Actin (Smooth Muscle) Antibody (IgG)
Thyroid Peroxidase Antibody (Anti-TPO)
Liver Kidney Microsome (LKM-1) Antibody (IgG)

If ANA Screen, IFA is positive, then ANA Titer and Pattern will be performed at an additional charge (CPT code(s): 86039).
If Mitochondrial Antibody Screen is positive, then Mitochondrial Antibody Titer will be performed at an additional charge (CPT code(s): 86381).

Preferred Specimen

4.5 mL serum

Minimum Volume

1.6 mL

Transport Container

Transport tube

Transport Temperature

Refrigerated (cold packs)

Specimen Stability

Room temperature: 4 days
Refrigerated: 7 days
Frozen: 28 days

Reject Criteria (Eg, hemolysis? Lipemia? Thaw/Other?)

Hemolysis • Lipemic • Grossly icteric

Methodology
Immunoassay (IA)

Setup Schedule

Set up and Report available: See individual tests

Reference Range

See individual tests

Clinical Significance

This panel may be helpful in the diagnostic evaluation of individuals with suspected primary biliary cholangitis (PBC). This panel includes anti-mitochondrial antibody (AMA), with reflex to titer; an antinuclear antibody (ANA) immunofluorescence assay (IFA) screen, performed on human epithelial type 2 (HEp-2) cells, with reflex to titer and pattern; actin (smooth muscle) antibody (SMA; IgG); Sjogren antibodies (SSA, SSB); thyroid peroxidase (TPO) antibody; and liver kidney microsome type 1 (LKM-1) antibody (IgG).

PBC can be diagnosed by fulfilling 2 of the 3 diagnostic criteria that comprise biochemical evidence, histologic evidence, and the presence of PBC-specific antibodies [1]. Presence of AMAs is the serologic hallmark of PBC and can be detected in approximately 95% of patients. ANAs are detected in 10% to 30% of patients with PBC. The presence of certain ANAs patterns including those associated with antibodies to sp100 or gp210 have diagnostic value, especially in AMA-negative PBC [1, 2].

Other antibodies included in this panel may aid in differential diagnosis or identify concomitant autoimmune diseases. Antibodies to SSA and SSB are associated with Sjogren syndrome, an autoimmune disease frequently diagnosed in patients with PBC [1]. SMAs are present in most patients with type 1 autoimmune hepatitis (AIH) but can be detected in a range of other conditions, such as viral hepatitis, malignancies, rheumatic diseases, and nonalcoholic fatty liver disease. LKM-1 antibody is characteristic for type 2 AIH in the absence of hepatitis C. TPO antibodies are associated with autoimmune thyroid disease, which is common in patients with PBC [2]. Note that the presence of these antibodies does not confirm the coexistence of associated autoimmune diseases [1].

The results of this test should be interpreted in the context of pertinent clinical and family history and physical examination findings.

References
1. Lindor KD, et al. Hepatology. 2019;69(1):394-419.
2. Neuberger J, et al, eds. Autoimmune Liver Disease: Management and Clinical Practice. 1st ed. John Wiley & Sons Ltd, 2020

The CPT Codes provided in this document are based on AMA guidelines and are for informational purposes only. CPT coding is the sole responsibility of the billing party. Please direct any questions regarding coding to the payor being billed. Any Profile/panel component may be ordered separately. Reflex tests are performed at an additional charge.