Oropharyngeal pemphigus vulgaris and clinical remission: a long-term, longitudinal study

Abstract

Background: The pattern of clinical remission in pemphigus vulgaris patients still remains a controversial issue because of the limited data reported in the literature.

Objective: To evaluate the time to clinical remission in patients with exclusive oropharyngeal pemphigus vulgaris.

Methods: We conducted a long-term, longitudinal study in a university hospital. We treated 37 patients with oropharyngeal pemphigus vulgaris, who underwent a periodic follow-up for an average of 5.3 years, and evaluated their outcome in terms of clinical remission. The main outcome measure was the clinical outcome (assessed by objective measures of severity, extent of disease, intensity of therapy, and remission) before and after conventional immunosuppressive therapy.

Results: Complete and long-lasting clinical remission was achieved in 35 patients (94.6%) with oropharyngeal lesions, of whom 13 (35.1%) were off therapy and 21 (56.8%) were on therapy at the last evaluation. One patient (2.7%) died following a stroke 3 years after complete remission on therapy. Partial remission was achieved in two patients (5.4%). The mean time to achieve complete clinical remission was 4.7 +/- 2.57 months after commencement of therapy. In all patients the mean disease severity score decreased from 7.81 +/- 1.35 at time of diagnosis to 1.0 +/- 0.9 at time of clinical remission (p < 0.0001 vs baseline), while the extent of the disease decreased from 2.9 +/- 1.0 to 0.27 +/- 0.45 (p < 0.0019 vs baseline) and the intensity of therapy from 4.91 +/- 0.64 to 0.70 +/- 0.57 (p < 0.0001 vs baseline). The mean duration of complete remission was 63.53 +/- 44.9 months.

Conclusions: In almost all patients with oropharyngeal pemphigus vulgaris it was possible to schedule a safe tapering of the conventional immunosuppressive therapy very shortly after the disease was controlled. Thus, we may conclude that: (i) the percentage of patients with oropharyngeal pemphigus vulgaris who achieved complete long-lasting clinical remission was very high; (ii) transient lesions that healed within a week were very frequent and had to be actively controlled; (iii) if treated early, most patients had a good clinical response and could achieve a disease- and drug-free clinical remission; (iv) early treatment may prevent extension or progression of disease; (v) there is a possible role for immunosuppressive agents; and (vi) a more favorable course of the disease, in terms of attainment and duration of clinical remission and a better prognosis, seemed to be related to a rapid response to therapy rather than to the initial severity and extent of the disease.