Test Catalog

Test Id : BPAB

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Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum

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Overview

Test Catalog

Useful For
Suggests clinical disorders or settings where the test may be helpful

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
A short description of the method used to perform the test

Enzyme-Linked Immunosorbent Assay (ELISA)

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

BP 180 and 230, Serum

Aliases
Lists additional common names for a test, as an aid in searching

BP180

BP230

Bullous Pemphigoid

Specimen Type
Describes the specimen type validated for testing

Serum Red

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

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.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

0.5 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

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

Useful For
Suggests clinical disorders or settings where the test may be helpful

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)

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

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
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

BULLOUS PEMPHIGOID 180:

<20 RU/mL (negative)

> or =20 RU/mL (positive)

 

BULLOUS PEMPHIGOID 230:

<20 RU/mL (negative)

> or =20 RU/mL (positive)

Interpretation
Provides information to assist in interpretation of the test results

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
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

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.

Supportive Data

Thirty-two classic bullous pemphigoid (BP), 15 mucous membrane pemphigoid, and 7 other pemphigoid variants, diagnosed by direct immunofluorescence, routine histology, and clinical presentation were tested. Controls included 47 patients with other autoimmune blistering disorders and 42 age-matched controls without skin disease. Forty of 54 (74%) patients with BP and variants tested positive for BP180 and/or BP230 autoantibodies. Of these patients, 28 of 32 (88%) with classical BP, 8 of 15 (53%) with mucous membrane predominant (MMP), and 4 of 7 (57%) of other pemphigoid variants, tested positive.

 

The calculated sensitivities in classical BP were 54% for BP180 alone and 56% for BP230 alone. The sensitivity increased to 88% with both tests combined, which is comparable to that of indirect immunofluorescence (IIF) (88%). In MMP the calculated sensitivities were 47% for BP180 alone, 13% for BP230 alone, and 53% for both combined. This was slightly less than the sensitivity of IIF (67%). Only 5 of 47 (11%) and 2 of 47 (4%) control patients with other autoimmune blistering disorders were positive for BP180 and BP230 autoantibodies respectively. Interestingly, the 2 patients positive for BP230 autoantibody had paraneoplastic pemphigus. One of 42 (2%) and 0 normal controls tested positive for BP180 and BP230 respectively.

 

The calculated specificities for BP180, BP230, and IIF were 93%, 98%, and 92% respectively.

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest. 1993;92(5):2480-2488

2. Matsumura K, Amagai M, Nishikawa T, Hashimoto T. The majority of bullous pemphigoid and herpes gestationes serum samples react with the NC16a domain of the e180-kD bullous pemphigoid antigen. Arch Dermatol Res. 1996;288(9):507-509

3. Stanley JR, Hawley-Nelson P, Yuspa SH, Shevach EM, Katz SI. Characterization of bullous pemphigoid antigen: a unique basement membrane protein of stratified aqueous epithelia. Cell. 1981;24(3):897-903

4. Hamada T, Nagata Y, Tomita M, Salmhofer W, Hashimoto T. Bullous pemphigoid sera react specially with various domains of BP230, most frequently with C-terminal domain, by immunblot analyses using bacterial recombinant proteins covering the entire molecule. Exp Dermatol. 2001;10(4):256-263

5. Rico MJ, Korman NJ, Stanley JR, Tanaka T, Hall RP. IgG antibodies from patients with bullous pemphigoid bind to localized epitopes on synthetic peptides encoded by bullous pemphigoid antigen cDNA. J Immunol. 1990;145(11):3728-3733

6. Wieland CN, Comfere NI, Gibson LE, Weaver AL, Krause PK, Murray JA. Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaffected subjects. Arch Dermatol. 2010;146(1):21-25

7. Montagnon CM, Tolkachjov SN, Murrell DF, Camilleri MJ, Lehman JS. Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. J Am Acad Dermatol. 2021;85(1):1-14

8. Montagnon CM, Lehman JS, Murrell DF, Camilleri MJ, Tolkachjov SN. Subepithelial autoimmune bullous dermatoses: disease activity assessment and therapy. J Am Acad Dermatol. 2021;85(1):18-27

Method Description
Describes how the test is performed and provides a method-specific reference

This enzyme-linked immunosorbent assay (ELISA) method detects and measures serum levels of antibodies of certain pemphigoid diseases. Calibrators and patient sera are added to microwells coated with bullous pemphigoid (BP) BP180 and BP230 antigens, allowing antibodies to react with the immobilized antigens. After washing to remove any unbound serum proteins, horseradish peroxidase-conjugated IgG is added and incubated. Following another wash step, the peroxidase substrate is added and allowed to incubate for an additional period. Stop solution is then added to each well to cancel the enzyme reaction and to stabilize the color development. The assay can be quantified by measuring the reaction photometrically and plotting the results. The amount of antigen specific bound antibody is proportional to the color intensity.(Package inserts: Anti-BP180-NC16A-4X ELISA [IgG], Form EA_1502-2G_A_US_D04. EuroImmun; 07/08/2020; Anti-BP230-CF ELISA [IgG], Form EA_1502-1G_A_UK_C03.doc. EuroImmun; 10/23/2020)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Varies

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

1 to 5 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

30 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
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Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

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
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

83516 x 2

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
BPAB BP 180 and 230, Serum 92671-7
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.
606816 BP 180, S 53842-1
606817 BP 230, S 53843-9

Test Setup Resources

Setup Files
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Excel | PHP Pdf | CMS Pdf

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports