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


METHODS
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).


METHODS
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
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.
Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France.
Fanny
F
Fichel
Clinical Research Unit, Reims University Hospital, Reims, France.
Coralie
C
Barbe
Department of Dermatology, Institute for Research and Innovation in Biomedicine, Rouen University Hospital, University of Normandy, Rouen, France.
Pascal
P
Joly
Department of Dermatology, Limoges University Hospital, Limoges, France.
Christophe
C
Bedane
Department of Dermatology, Dijon University Hospital, Dijon, France.
Pierre
P
Vabres
Department of Dermatology, Beauregard Hospital, Thionville, France.
François
F
Truchetet
Department of Dermatology, Besançon University Hospital, Besançon, France.
François
F
Aubin
Department of Dermatology, Mulhouse Hospital, Mulhouse, France.
Catherine
C
Michel
Department of Dermatology, Chalons en Champagne Hospital, Chalons en Champagne, France.
Juliette
J
Jegou
Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France.
Florent
F
Grange
Laboratory of Dermatology, Faculty of Medicine of Reims, University of Champagne-Ardenne, Reims, France.
Frank
F
Antonicelli
Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France.
Philippe
P
Bernard
D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000276:immunology*; D015999:Multivariate Analysis; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012008:Recurrence; D012307:Risk Factors; D012680:Sensitivity and Specificity; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D005938:Glucocorticoids; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human