Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy.

Stefania Leuci,Gianrico Spagnuolo,Massimo Amato,Raffaele Piscopo,Elena Calabria,Michele Davide Mignogna,Fausto Tranfa

doi:10.1155/2018/8372146

Abstract

Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, systemic, autoimmune subepidermal inflammatory disease that affects mucous membranes and the eye. In its most severe forms, this disease needs systemic therapy, usually based on steroids and immunosuppressant agents. In unresponsive cases or in the presence of contraindications or severe side effects due to conventional systemic corticosteroid and/or immunosuppressant therapy, a therapy shift to high-dose intravenous immunoglobulins (IVIg) has been recommended in other reports. This new therapy has proven to be effective in stopping ocular pemphigoid, but the data regarding the long-term effect on the disease activity or reactivation are extremely scarce, so the novel scientific aim of this study was to evaluate the clinical outcomes after a 9-year follow-up in 12 eyes (6 patients) affected by MMP with ocular involvement, successfully treated with IVIg therapy, as previously described in our report published in 2008. The evaluation of ocular and extraocular disease progression was performed at the end of IVIg therapy and at the end of the follow-up period. After 9 years, all the eyes enrolled showed a long-lasting remission of ocular and oral symptoms with a significant steroid-sparing effect. In conclusion, the IVIg has to be considered as a safe and successful alternative therapy in patients with severe ocular mucous membrane pemphigoid; furthermore, this kind of therapy seems to be effective in maintaining the clinical remission by the time.

Highlights

Mucous membrane pemphigoid (MMP) is a severe, systemic, autoimmune bullous disease that affects mucous membranes like ocular conjunctiva (64%), oral mucosa (85%), and occasionally the skin [1], which can have major morbidities and, rarely, deadly consequences [2,3,4].Ocular MMP accounts for 61% of the cases of newly diagnosed cicatricial conjunctivitis between 60 and 80 years of age, with an incidence calculated as 0.8 per million population, and it affects women more often than men with a male-to-female ratio of nearly 2 : 1 [5]
A subset of patients affected by MMP only suffer from ocular involvement: this peculiar MMP is known as ocular cicatricial pemphigoid (OCP) [9]
Corticosteroid doses ranged from 60 mg to 100 mg/day with a cumulative total dose of steroids administered before intravenous immunoglobulins (IVIg) therapy ranging from 16,200 mg to 81,000 mg

Summary

Introduction

Mucous membrane pemphigoid (MMP) is a severe, systemic, autoimmune bullous disease that affects mucous membranes like ocular conjunctiva (64%), oral mucosa (85%), and occasionally the skin [1], which can have major morbidities and, rarely, deadly consequences [2,3,4].Ocular MMP accounts for 61% of the cases of newly diagnosed cicatricial conjunctivitis between 60 and 80 years of age, with an incidence calculated as 0.8 per million population, and it affects women more often than men with a male-to-female ratio of nearly 2 : 1 [5]. Mucous membrane pemphigoid (MMP) is a severe, systemic, autoimmune bullous disease that affects mucous membranes like ocular conjunctiva (64%), oral mucosa (85%), and occasionally the skin [1], which can have major morbidities and, rarely, deadly consequences [2,3,4]. A subset of patients affected by MMP only suffer from ocular involvement: this peculiar MMP is known as ocular cicatricial pemphigoid (OCP) [9]. Both the MMP with ocular involvement and the OCP start with a conjunctival inflammation but in the latter stage the corneal scarring can lead to blindness [2]

Methods

Results

Discussion

Conclusion