Rituximab for Patients With Refractory Mucous Membrane Pemphigoid

Abstract

Background: Mucous membrane pemphigoid (MMP) still represents a potentially life-and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated.Observations: Twenty-five patients with severe refractory MMP, including 5 with mucous membranedominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (375 mg/m 2 weekly for 4 weeks). Twenty-one of the patients were receiving concomitant therapy with dapsone and/or sulfasalazine therapy, which was maintained during rituximab cycles. Complete responses in all affected sites (ocular and/or extraocular) were obtained in 17 patients (68%) by a median time of 12 weeks after the first cycle, and 5 additional patients responded completely after a second cycle, yielding an 88% complete response rate. In all but 1 of the 10 patients with ocular lesions, their eyes became noninflammatory within a mean of 10 weeks. Among the 3 patients (12%) who developed severe infectious complications, 2 (8%) died; they had been receiving concomitant conventional immunosuppressants and high-dose corticosteroids and were hypogammaglobulinemic. Treatment with immunosuppressants was discontinued for all other patients, and no other infection was observed. Ten patients experienced relapse after a mean of 4 (range, 1-16) months after achieving complete responses.Conclusions: Rituximab appears to have rapid and dramatic efficacy in patients with severe, refractory MMP. The occurrence of severe infections in patients receiving concomitant conventional immunosuppressants supports using rituximab without other immunosuppressants. Controlled prospective studies are warranted to define an optimal treatment protocol.

Prevalence and Clinical Significance of Anti–Laminin 332 Autoantibodies Detected by a Novel Enzyme-Linked Immunosorbent Assay in Mucous Membrane Pemphigoid

“…Other antigens targeted predominantly by IgG autoantibodies in MMP included BP230. 22,27,34,43 However, autoantibodies against BP230 are detected only occasionally in MMP serum, as shown in the present study. Interestingly, serum autoantibodies directed against BP230 are more frequently detected in the serum of patients with MMP when anti-Lam332 autoantibodies are also present.…”

Dermatolojide Rituksimab Kullanımı

“…LeRoux ve ark 25 dirençli mukoz membran pemfigoidli olguda rituksimabla % 68 tam remisyon elde edebilmiştir. Ayrıca 5 akkiz epidermolizis büllozalı olguda ise % 88 oranında remisyon elde edebilmişlerdir (105).…”

Therapieresistentes Anti-Laminin-332-Schleimhautpemphigoid

“…223 Augen zeigte die beste klinische Wirkung für Cyclophosphamid, das günstigste RisikoWirkungs-Profil wurde für Mykophenolat-Mofetil ermittelt [14]. Über den Einsatz des anti-CD20-Antikörpers Rituximab wurde bisher bei 36 SHP-Patienten berichtet [4, 8,10,15]. Hierbei zeigte sich in den retrospektiven Studien eine komplette klinische Remission (Abheilung aller Läsionen) in 26 von 32 Fällen und eine partielle Remission in weiteren 3 Fällen.…”

“…Hierbei zeigte sich in den retrospektiven Studien eine komplette klinische Remission (Abheilung aller Läsionen) in 26 von 32 Fällen und eine partielle Remission in weiteren 3 Fällen. Zu schweren unerwünschten Ereignissen kam es in 3 Fällen, davon 2 mit letalem Ausgang [8,10,15]. Eine Cyclophosphamid-Dexamethason-Pulstherapie in Kombination mit täglichem oralem Cyclophosphamid über 8 Wochen hatte bei unserem Patienten zu keiner klinischen Besserung geführt.…”