טיפול ב-mucous membrane pemphigoid עם ריטוקסימאב – סדרת מקרים וסקירת ספרות.

Background:

Mucous membrane pemphigoid is an autoimmune blistering, scarring and occasionally mutilating disease that may progress to blindness or airway obstruction. Therefore, prompt and adequate therapy is needed. Over the last few years, rituximab has emerged as a potential therapeutic solution for MMP. The literature regarding the efficiency of RTX in treatment of severe MMP is sparse.

Patients and methods:
We report four recalcitrant patients treated with rituximab and a review of the literature.

Results:

We studied a series of 4 patients with recalcitrant laryngeal disease in three patients. Two of these patients were unresponsive in to RTX. The third patient with laryngeal MMP achieved complete remission (CR), but relapsed twice. The fourth patient, with oral and ocular disease, achieved complete remission under RTX. In a review of the literature, 143 MMP patients treated rituximab were found. Late observation end points were noted for 120 patients, of whom 81 (67.5%) achieved complete remission, 24 (20%) partial remission and 15 (12.5%) no remission. Relapses occurred in 35 (25%) patients with mean time to relapse of 16.9 months. Laryngeal involvement seems to predict refractoriness to treatment. Thus laryngeal involvement which is known to be a prognostic factor for severe disease, also predicts poor response to RTX.

Conclusion:

RTX can ameliorate MMP course.