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Evidence summaries

Interventions for Pemphigus

There is insufficient information to conclude which is the most effective and safest treatment for pemphigus, although some interventions may be superior for certain outcomes. Level of evidence: "D"

A Cochrane review [Abstract] 1 included 11 studies with a total of 404 subjects. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor and traditional Chinese medicine. Ten studies included participants with newly diagnosed or newly active recurrent disease, and one trial included participants in maintenance phase.

Some interventions were found to be superior for certain outcomes, although it was not possible to conclude which treatments are superior overall. Mycophenolate was more effective in achieving disease control than azathioprine (1 study; n=40; RR 0.72; 95% CI 0.52 to 0.99, NNT 3.7). There was evidence of a steroid-sparing benefit of azathioprine (1 study; n=57; MWD -3 919 mg prednisolone; 95% CI -6 712 to -1 126 mg) and cyclophosphamide (1 study; n=54; MWD -3 355 mg prednisolone; 95% CI -6 144 to -566) compared to glucocorticoids alone. Topical epidermal growth factor decreased time to control (1 study; n=20; HR 2.35; 95% CI 1.62 to 3.41).

Comment: The quality of evidence is downgraded by study quality (inadequate or unclear allocation concealment), inconsistency (heterogeneity in interventions and outcomes) and imprecise results (few patients and wide confidence intervals).

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Primary/Secondary Keywords