Clinical and immunological features of pemphigus relapse

Abstract

Background

More than half of pemphigus patients experience relapse during the disease course. However, the risk factors and clinical and immunological characteristics of relapse remain largely unclear.

Objective

To elucidate risk factors and clinical features of pemphigus relapse.

Methods

Retrospective review of the clinical records of 42 pemphigus cases in a single center.

Results

61·9% of cases experienced relapse, usually when oral prednisolone was tapered to around 0·1mg/kg. In mucocutaneous pemphigus vulgaris (mcPV), the initial doses of prednisolone were significantly lower in cases with relapse (0·78 ± 0·24 mg/kg) than without relapse (1·01 ± 0·01 mg/kg). At relapse, mcPV shifted to mucosal dominant PV (mPV) (40%), pemphigus foliaceus (PF) (20%) or others (20%). In contrast, the relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti‐Dsg1 and anti‐Dsg3 antibodies at onset had recurrence with anti‐Dsg3 antibodies alone (40%), with both anti‐Dsg1 and Dsg3 antibodies (30%) or with anti‐Dsg1 antibody alone (20%), or were subthreshold (10%).

Conclusion

mcPV shows transitions in clinical phenotype and autoantibody profile at relapse. At least 1mg/kg/day of prednisolone, especially for mcPV cases, and prudent tapering around 0·1mg/kg may lead to better outcomes.

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