Clinical and immunologic factors associated with bullous pemphigoid relapse during the first year of treatment: a multicenter, prospective study.

Abstract

IMPORTANCE:

Although predisposing factors for bullous pemphigoid (BP) have been recently established, no clinical or immunologic factors have yet been identified to predict disease outcome.

OBJECTIVE:

To identify risk factors for BP relapse during the first year of treatment.

DESIGN, SETTING, AND PARTICIPANTS:

Multicenter prospective study of 120 consecutive patients with newly diagnosed BP in 8 French dermatology departments. Baseline and 6 follow-up visits were planned to record disease activity and collect blood samples for measurement of serum anti-BP180 and anti-BP230 levels by means of enzyme-linked immunosorbent assay (ELISA).

MAIN OUTCOMES AND MEASURES:

The end point was clinical relapse within the first year of therapy. Associations of clinical and immunologic (including serum levels of anti-BP180 and anti-BP230 autoantibodies) parameters with clinical relapse were assessed using univariate and multivariate analyses.

RESULTS:

During the 1-year follow-up, 35 patients (29.2%) experienced relapse, whereas anti-BP180 and anti-BP230 ELISA results were similar at baseline between patients who did and did not experience relapse. Factors at baseline independently associated with relapse were extensive disease at inclusion (hazard ratio [HR], 2.37 [95% CI, 1.2-4.8]) and an associated dementia (HR, 2.09 [95% CI, 1.0-4.2]). Use of superpotent topical corticosteroids alone (by 100 patients [83.3%]) induced a dramatic, early decrease in serum levels of anti-BP180 and anti-BP230 autoantibodies. Mean early decreases in autoantibody levels between baseline and day 60 were lower in patients with relapse compared with patients with ongoing remission (-10.0% and -45.2%, respectively, for anti-BP180 levels [P < .001] and -11.8% and -35.4%, respectively, for anti-BP230 levels [P = .046]). A higher serum level of anti-BP180 at day 150, with a cutoff of 23 U/mL, provided 84.2% sensitivity, 44.8% specificity, 33.3% positive predictive value, and 89.7% negative predictive value for the occurrence of relapses between days 150 and 360.

CONCLUSIONS AND RELEVANCE:

The pronounced decrease in the level of anti-BP180 autoantibodies and, to a lesser extent, those directed against BP230 confirmed the use of superpotent topical corticosteroids alone as a reference BP treatment. Furthermore, our study suggests that neurological diseases play a major role in BP, not only as a predisposing but also as a prognostic factor.

• Authors: Fanny Fichel, Coralie Barbe, Pascal Joly, Christophe Bedane, Pierre Vabres, François Truchetet, François Aubin, Catherine Michel, Juliette Jegou, Florent Grange, Frank Antonicelli, Philippe Bernard
• Journal: JAMA dermatology (JAMA Dermatol) Vol. 150 Issue 1 Pg. 25-33 (Jan 2014) ISSN: 2168-6084 [Electronic] United States
• PMID: 24226428 (Publication Type: Journal Article, Multicenter Study, Observational Study, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Autoantigens
• Carrier Proteins
• Cytoskeletal Proteins
• DST protein, human
• Dystonin
• Glucocorticoids
• Membrane Glycoproteins
• Nerve Tissue Proteins
• Non-Fibrillar Collagens

Topics

• Aged
• Aged, 80 and over
• Autoantibodies (blood)
• Autoantigens (immunology)
• Carrier Proteins
• Cytoskeletal Proteins
• Dystonin
• Enzyme-Linked Immunosorbent Assay
• Female
• Follow-Up Studies
• Glucocorticoids (administration & dosage)
• Humans
• Male
• Membrane Glycoproteins (immunology)
• Multivariate Analysis
• Nerve Tissue Proteins
• Non-Fibrillar Collagens (immunology)
• Pemphigoid, Bullous (drug therapy, immunology, pathology)
• Predictive Value of Tests
• Prognosis
• Proportional Hazards Models
• Prospective Studies
• Recurrence
• Risk Factors
• Sensitivity and Specificity
• Collagen Type XVII