Long‐term evaluation of pemphigus vulgaris: A retrospective consideration of 98 patients treated in an oral medicine unit in north‐west Italy

Abstract

Background Despite the frequency of oral involvement, there are unexpectedly few studies of either on the oral manifestations of pemphigus or their long‐term management, and diagnostic delay in Dentistry is frequent. Methods We have examined outcome of patients presenting with predominantly oral pemphigus vulgaris (PV). Ninety‐eight subjects were followed up for 85.12 months and treated with systemic steroids: 48 of them received adjunctive therapy with azathioprine, 16 with rituximab, 13 with mycophenolate mofetil, three with immunoglobulin and one with dapsone. Results Clinical remission was achieved in 80 patients (84.21%); 39 of them were off therapy and 41 on therapy. Fifteen patients were not in remission, having been under systemic therapy for 72.16 months. Sixty‐nine patients developed detectable adverse effects. Two fatal outcomes were recorded. Each additional year of steroid therapy ensured 47% chance of developing 1 or 2 side effects, and 64% chance of developing more than 3 (ORs 1.47, CI 1.162‐1.903; ORs 1.64, CI 1.107‐2.130, respectively). Conclusion In one of the largest available cohort with the longest follow‐up ever reported, we observed that the management remains need‐based and patient‐specific, still relying on systemic corticosteroids.

The prompt use of rituximab could decrease adverse effects in patient with pemphigus vulgaris: A preliminary evaluation

“…Pemphigus vulgaris (PV) usually affects first the oral mucosa, and its management aims to induce and maintain clinical remission with as few as possible adverse effects. Because of the lack of international agreed recommendations, both the dosage of corticosteroids and choice of adjuvant agents usually reflect the experience of clinicians at various centers . Recently, we confirmed that systemic corticosteroid should be considered the mainstay medication for predominantly oral involvement …”

“…The control group comprised 95 unrelated Caucasian patients (60 female and 35 male), together with two female and one Afro‐Caribbean male (F: M; 1.65:1), mean age 54.56 (±14.95) …”

“…The patient group received treatment with systemic corticosteroids (typical starting dose of 1.5 mg/kg per day of prednisone, for variable lengths of time usually of 2‐4 months, and then tapered in other 8 months approximately), together with RTX (1 g IV, two infusions two week apart within 6 months from diagnosis) provided at the Center of Research of Immunopathology and Rare Diseases (S. Giovanni Bosco Hospital). We described therapeutic response and adverse effects previously …”

“…Qualitative variables were described via frequencies and percentages, quantitative ones via median and interquartile range (IQR), due to the low sample size in the new group and the following lack of the normality assumption. As control group, we decided to select all PV patients reported recently by our group and treated with systemic medication . Sixteen patients of this group received intravenous RTX, after standard treatment failure.…”

“…RTX has been used as first‐line intervention together with short‐term prednisone in patients with mucosal‐cutaneous PV and it was suggested to be more effective than prednisone alone, with fewer adverse events . Few data are on RTX effectiveness are available for predominantly oral PV, and most of the studies report different regimens; moreover, it has been not yet used as a first‐line intervention in oral cases …”