Relative risk for cancer in mucous membrane pemphigoid associated with antibodies to the beta4 integrin subunit.
BACKGROUND
Mucous membrane pemphigoid (MMP) is a mucocutaneous vesiculobullous autoimmune disease characterized by autoantibodies to components of the basement membrane zone (BMZ). Recently, it has been reported that patients with MMP who have autoantibodies to laminin 5, known as anti-epiligrin cicatricial pemphigoid (AECP) have a high incidence of malignancy.


OBJECTIVE
The purpose of this study was to determine the association between malignancy and MMP in patients with antibodies to beta4 integrin.


METHODS
The incidence of cancer was studied in 79 patients with MMP and/or ocular cicatricial pemphigoid (OCP) who had antibodies to human beta4 integrin subunit. In each patient, the diagnosis was made by histology and confirmed by immunopathology of affected tissues. It was compared to the expected incidence, for age- and gender-matched individuals, in the National Cancer Institute's Surveillance, Epidemiology and End Results (NCISEER) database.


RESULTS
Of 79 patients, 3 had cancer. The relative risk (RR) for cancer in patients with MMP and/or OCP, with autoantibodies to human beta4 integrin subunit was 0.29 (95% CI 0.62-8.77). The expected number in the NCISEER database was 10.37. This difference was statistically significant (P < 0.01).


CONCLUSIONS
This incidence of cancer in MMP/OCP patients, with antibodies to human beta4 integrin subunit is considerably lower than expected. Preliminary observations in this and other studies suggest that serological subsets of MMP, based on antigen reactivity, have a different clinical course, prognosis and associations with cancer.
Letko
E
E
Immunology Service, Massachusetts Eye and Ear Infirmary, Medical School, Boston, MA, USA.
Gürcan
H M
HM
Papaliodis
G N
GN
Christen
W
W
Foster
C S
CS
Ahmed
A R
AR
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D015815:Cell Adhesion Molecules / Q000276:immunology; D005128:Eye Diseases / Q000150:complications / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D039663:Integrin beta4 / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000209:etiology* / Q000276:immunology; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000276:immunology*; D011379:Prognosis; D018570:Risk Assessment / Q000379:methods; D000097794:Kalinin
D001323:Autoantibodies; D015415:Biomarkers; D015815:Cell Adhesion Molecules; D039663:Integrin beta4; D000097794:Kalinin