Tags

Type your tag names separated by a space and hit enter

Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study.
Br J Dermatol. 2013 Jan; 168(1):80-4.BJ

Abstract

BACKGROUND

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune mucocutaneous bullous disease caused by autoantibodies against type VII collagen, a component of anchoring fibrils that stabilizes dermoepidermal adherence. Type VII collagen is composed of a collagenous domain linked by the noncollagenous (NC)1 and NC2 domains.

OBJECTIVES

To assess the repeatability, sensitivity and specificity of a recently developed enzyme-linked immunosorbent assay (ELISA) for detection of anti-type VII collagen autoantibodies, and to ascertain whether they may be a marker of disease activity in EBA.

METHODS

Using this ELISA, which was able to recognize autoantibodies against the NC1 and NC2 epitopes of type VII collagen, we tested 14 EBA sera, 30 healthy control sera and 113 disease control sera.

RESULTS

In the EBA sera group, 12 out of the 14 samples were positive in ELISA, with autoantibody titres varying from 7·2 to 127·9UmL(-1) (cutoff value <6), the sensitivity of the method being 86%. Among the controls, only two bullous pemphigoid sera tested positive, the specificity being 98·6%. A good correlation was found between EBA disease severity, expressed as autoimmune bullous skin disorder intensity score, and the serum levels of anti-collagen VII autoantibodies, measured by ELISA (n =14; r=0·965; P=0·0001). The intra- and interassay coefficients of variation of the ELISA method ranged from 6·3% to 18·3%.

CONCLUSIONS

This NC1+NC2 ELISA can be a practical assay for the diagnosis of EBA. The correlation between autoantibody titres and disease severity suggests its usefulness as a marker of disease activity in EBA However, this should be confirmed by studies on larger series of patients.

Authors+Show Affiliations

Unità di Dermatologia, Dipartimento di Fisiopatologia e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Via Pace 9, 20122, Milano, Italy. angelovalerio.marzano@policlinico.mi.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Evaluation Study
Journal Article
Multicenter Study

Language

eng

PubMed ID

22913489

Citation

Marzano, A V., et al. "Diagnosis and Disease Severity Assessment of Epidermolysis Bullosa Acquisita By ELISA for Anti-type VII Collagen Autoantibodies: an Italian Multicentre Study." The British Journal of Dermatology, vol. 168, no. 1, 2013, pp. 80-4.
Marzano AV, Cozzani E, Fanoni D, et al. Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study. Br J Dermatol. 2013;168(1):80-4.
Marzano, A. V., Cozzani, E., Fanoni, D., De Pità, O., Vassallo, C., Berti, E., Parodi, A., Crosti, C., & Cugno, M. (2013). Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study. The British Journal of Dermatology, 168(1), 80-4. https://doi.org/10.1111/bjd.12011
Marzano AV, et al. Diagnosis and Disease Severity Assessment of Epidermolysis Bullosa Acquisita By ELISA for Anti-type VII Collagen Autoantibodies: an Italian Multicentre Study. Br J Dermatol. 2013;168(1):80-4. PubMed PMID: 22913489.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Diagnosis and disease severity assessment of epidermolysis bullosa acquisita by ELISA for anti-type VII collagen autoantibodies: an Italian multicentre study. AU - Marzano,A V, AU - Cozzani,E, AU - Fanoni,D, AU - De Pità,O, AU - Vassallo,C, AU - Berti,E, AU - Parodi,A, AU - Crosti,C, AU - Cugno,M, Y1 - 2012/11/21/ PY - 2012/8/24/entrez PY - 2012/8/24/pubmed PY - 2013/6/8/medline SP - 80 EP - 4 JF - The British journal of dermatology JO - Br J Dermatol VL - 168 IS - 1 N2 - BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune mucocutaneous bullous disease caused by autoantibodies against type VII collagen, a component of anchoring fibrils that stabilizes dermoepidermal adherence. Type VII collagen is composed of a collagenous domain linked by the noncollagenous (NC)1 and NC2 domains. OBJECTIVES: To assess the repeatability, sensitivity and specificity of a recently developed enzyme-linked immunosorbent assay (ELISA) for detection of anti-type VII collagen autoantibodies, and to ascertain whether they may be a marker of disease activity in EBA. METHODS: Using this ELISA, which was able to recognize autoantibodies against the NC1 and NC2 epitopes of type VII collagen, we tested 14 EBA sera, 30 healthy control sera and 113 disease control sera. RESULTS: In the EBA sera group, 12 out of the 14 samples were positive in ELISA, with autoantibody titres varying from 7·2 to 127·9UmL(-1) (cutoff value <6), the sensitivity of the method being 86%. Among the controls, only two bullous pemphigoid sera tested positive, the specificity being 98·6%. A good correlation was found between EBA disease severity, expressed as autoimmune bullous skin disorder intensity score, and the serum levels of anti-collagen VII autoantibodies, measured by ELISA (n =14; r=0·965; P=0·0001). The intra- and interassay coefficients of variation of the ELISA method ranged from 6·3% to 18·3%. CONCLUSIONS: This NC1+NC2 ELISA can be a practical assay for the diagnosis of EBA. The correlation between autoantibody titres and disease severity suggests its usefulness as a marker of disease activity in EBA However, this should be confirmed by studies on larger series of patients. SN - 1365-2133 UR - https://www.unboundmedicine.com/medline/citation/22913489/Diagnosis_and_disease_severity_assessment_of_epidermolysis_bullosa_acquisita_by_ELISA_for_anti_type_VII_collagen_autoantibodies:_an_Italian_multicentre_study_ L2 - https://doi.org/10.1111/bjd.12011 DB - PRIME DP - Unbound Medicine ER -