These Factors May Predict Bullous Pemphigoid Relapse
These Factors May Predict Bullous Pemphigoid Relapse
Fichel F, Barbe C, Joly P, et al
JAMA Dermatol. 2014;150:25-33
Bullous pemphigoid (BP) is a potentially debilitating autoimmune blistering skin disease featuring subepidermal bullae. It is characterized by the presence of autoantibodies against 2 hemidesmosomal proteins (BP180 and BP230) found in the basement membrane zone. Known risk factors for BP include advanced age; triggering medications, such as furosemide, neuroleptic agents, and spironolactone; cancer; and neurologic diseases (eg, Parkinson disease and dementia).
For patients presenting with BP for the first time, predicting their disease course can be challenging. Some experience rapid clearance followed by prolonged remission, whereas others experience recurrent flares necessitating long-term immunosuppressive therapy, with its attendant risks. Is there a way to predict which patients will experience mild and transient vs severe and refractory BP?
To address this important question, Fichel and colleagues looked for risk factors predictive of BP relapse during the first year of treatment in a French cohort of 120 patients with newly diagnosed BP (female-to-male ratio, 1.35; mean age, 81.1 years; 54% with severe disease at presentation; 57% with associated neurologic disorders). All study patients met standard clinical, histopathologic, and direct immunofluorescence criteria for BP. Of note, most of these patients (100 of 120) were treated with ultrapotent topical corticosteroids; relatively few supplemented this with systemic agents, such as oral methotrexate (n = 14), leflunomide (n = 3), or oral corticosteroids (n = 1).
During 1 year of follow-up, Fichel and colleagues noted the following important characteristics of their BP cohort:
• Thirty-five patients (29.2%) experienced disease relapse, and the mean time to relapse was approximately 5 months.
• Baseline autoantibody levels (anti-BP180 and anti-BP230) did not correlate with relapse risk.
• In contrast, mean anti-BP180 and anti-BP230 levels during the first 60 days decreased less in patients who had relapse than in those who maintained remission.
• A higher serum level of anti-BP180 (cut-off on enzyme-linked immunosorbent assay, 23 U/mL) at day 150 predicted an increased risk for relapse.
• Topical corticosteroid use was correlated with a significant reduction in both serum anti-BP180 and anti-BP230 levels.
• Extensive disease at study inclusion showed the strongest correlation with risk for disease relapse (hazard ratio, 2.37).
• Dementia was the second largest risk factor for recurrence (hazard ratio, 2.09).
Clinical and Immunologic Factors Associated With Bullous Pemphigoid Relapse During the First Year of Treatment: A Multicenter, Prospective Study
Fichel F, Barbe C, Joly P, et al
JAMA Dermatol. 2014;150:25-33
Study Summary
Bullous pemphigoid (BP) is a potentially debilitating autoimmune blistering skin disease featuring subepidermal bullae. It is characterized by the presence of autoantibodies against 2 hemidesmosomal proteins (BP180 and BP230) found in the basement membrane zone. Known risk factors for BP include advanced age; triggering medications, such as furosemide, neuroleptic agents, and spironolactone; cancer; and neurologic diseases (eg, Parkinson disease and dementia).
For patients presenting with BP for the first time, predicting their disease course can be challenging. Some experience rapid clearance followed by prolonged remission, whereas others experience recurrent flares necessitating long-term immunosuppressive therapy, with its attendant risks. Is there a way to predict which patients will experience mild and transient vs severe and refractory BP?
To address this important question, Fichel and colleagues looked for risk factors predictive of BP relapse during the first year of treatment in a French cohort of 120 patients with newly diagnosed BP (female-to-male ratio, 1.35; mean age, 81.1 years; 54% with severe disease at presentation; 57% with associated neurologic disorders). All study patients met standard clinical, histopathologic, and direct immunofluorescence criteria for BP. Of note, most of these patients (100 of 120) were treated with ultrapotent topical corticosteroids; relatively few supplemented this with systemic agents, such as oral methotrexate (n = 14), leflunomide (n = 3), or oral corticosteroids (n = 1).
During 1 year of follow-up, Fichel and colleagues noted the following important characteristics of their BP cohort:
• Thirty-five patients (29.2%) experienced disease relapse, and the mean time to relapse was approximately 5 months.
• Baseline autoantibody levels (anti-BP180 and anti-BP230) did not correlate with relapse risk.
• In contrast, mean anti-BP180 and anti-BP230 levels during the first 60 days decreased less in patients who had relapse than in those who maintained remission.
• A higher serum level of anti-BP180 (cut-off on enzyme-linked immunosorbent assay, 23 U/mL) at day 150 predicted an increased risk for relapse.
• Topical corticosteroid use was correlated with a significant reduction in both serum anti-BP180 and anti-BP230 levels.
• Extensive disease at study inclusion showed the strongest correlation with risk for disease relapse (hazard ratio, 2.37).
• Dementia was the second largest risk factor for recurrence (hazard ratio, 2.09).
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