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Test Code BPAB Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum


Specimen Required


Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission container/tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions: Centrifuge and aliquot serum into a plastic vial.


Useful For

Initial screening test in the diagnosis of bullous pemphigoid and its variants

 

Complementing the standard serum test of indirect immunofluorescence utilizing primate esophagus substrate and primate salt-split skin substrate (CIFS / Cutaneous Immunofluorescence Antibodies [IgG], Serum)

Method Name

Enzyme-Linked Immunosorbent Assay (ELISA)

Reporting Name

BP 180 and 230, Serum

Specimen Type

Serum Red

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Red Refrigerated (preferred) 14 days
  Frozen  30 days
  Ambient  14 days

Reject Due To

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK

Clinical Information

Bullous pemphigoid (BP) is a chronic pruritic blistering disorder found mainly in aged persons, characterized by the development of tense blisters over an erythematous or urticarial base. IgG anti-basement membrane zone antibodies are found in the serum of patients, and linear IgG and complement component 3 sediment is found on the basement membrane zone of the lesion. Several well characterized variants exist including localized, mucous membrane predominant and pemphigoid gestationis, also referred to as herpes gestationis.

 

Target antigens of the autoantibodies in BP patient serum are BP230 and BP180, also called BPAG1 and BPAG2. Molecular weight of these antigens is 230 kDa and 180 kDa, respectively. BP180 is thought to be the direct target of the autoantibody because of its location along the basement membranes, and the autoantibody against BP230 is thought to be secondarily produced.

Reference Values

BULLOUS PEMPHIGOID 180:

<20 RU/mL (negative)

≥20 RU/mL (positive)

 

BULLOUS PEMPHIGOID 230:

<20 RU/mL (negative)

≥20 RU/mL (positive)

Interpretation

Antibodies to bullous pemphigoid (BP) BP180 and BP230 have been shown to be present in most patients with pemphigoid. Adequate sensitivities and specificity for disease are documented and Mayo Clinic's experience demonstrates a very good correlation between BP180 and BP230 results and the presence of pemphigoid (see Supportive Data). However, in those patients strongly suspected to have pemphigoid, either by clinical findings or by routine biopsyor direct immunofluorescence, and in whom the BP180/BP230 assay is negative, follow-up testing by CIFS / Cutaneous Immunofluorescence Antibodies (IgG), Serum is recommended.

 

Antibody titer may correlate with disease activity in some patients. Patients with severe disease may be expected to have high titers of antibodies to BP. Titers may decrease with clinical improvement.

Cautions

As with other diagnostic test procedures, the results obtained with bullous pemphigoid (BP) BP180 and BP230 enzyme-linked immunosorbent assay kit serve only as an aid to diagnosis and should not be interpreted as diagnostic in themselves.

 

Performance of the assay in pediatric patients has not been established.

 

Performance of the assay on other matrices besides serum has not been established.

Day(s) Performed

Varies

Report Available

1 to 5 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

This test has been modified from the manufacturer's instructions. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

83516 x 2

LOINC Code Information

Test ID Test Order Name Order LOINC Value
BPAB BP 180 and 230, Serum 92671-7

 

Result ID Test Result Name Result LOINC Value
606816 BP 180, S 53842-1
606817 BP 230, S 53843-9