Combined Use of Rituximab and Intravenous Immunoglobulin for Severe Autoimmune Cicatricial Conjunctivitis—An Interventional Case Series

Steger, Bernhard; Madhusudan, Savitha; Kaye, Stephen B.; Stylianides, Amira; Romano, Vito; Maqsood, Sundas; Harper, Jane; Ahmad, Sajjad

doi:10.1097/ico.0000000000001024

Cited by 18 publications

(15 citation statements)

References 19 publications

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“…In order to assess the correlation between the MMP‐involved site and the response to RTX, we conducted a review of the literature. Using the search terms ‘MMP’ and ‘rituximab’ in the PubMed database, we found 30 articles 5–34 (detailed in Table ) covering 167 patients with MMP treated with RTX. Their demographic and therapeutic characteristics are shown in the supplementary material (Tables and , respectively).…”

Section: Reportmentioning

confidence: 99%

Laryngeal mucous membrane pemphigoid serves as a prognostic factor for poor response to treatment with rituximab

et al. 2021

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Summary Mucous membrane pemphigoid (MMP) is an autoimmune blistering, scarring and occasionally mutilating disease that may progress to blindness or airway obstruction. Over the past few years, rituximab (RTX) has emerged as a potential therapeutic solution for MMP; however, the literature regarding its efficacy in the treatment of severe MMP is sparse. We studied four patients with recalcitrant MMP who were treated with RTX. Three of these had recalcitrant laryngeal disease; two were unresponsive to RTX, while the third patient achieved complete remission (CR) but relapsed twice. The fourth patient, who had oral and ocular disease, also achieved CR. In addition, we reviewed 143 cases of MMP treated with RTX reported in the literature to date. Of these, 120 had late observation endpoints, of whom 81 (67.5%) achieved CR, 24 (20%) received partial remission and 15 (12.5%) had no remission. Based on this study, the presence of laryngeal MMP seems to predict refractoriness to RTX treatment. In conclusion, we found that RTX can ameliorate the MMP course and that laryngeal involvement, which is known to be a prognostic factor for severe MMP, may also predict poor response to RTX.

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Section: Reportmentioning

confidence: 99%

Laryngeal mucous membrane pemphigoid serves as a prognostic factor for poor response to treatment with rituximab

et al. 2021

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“…Four articles cite rituximab as an effective additional agent for severe, recalcitrant LAD. Three of the articles used two infusions of 1 g, 2 weeks apart, for two cycles [ 8 , 9 , 12 ]. One used a dosage of 375 mg/m 2 weekly, for a month, with mycophenolate mofetil 500 mg BID (bis in die) after the lack of response to prednisone [ 11 ].…”

Section: Resultsmentioning

confidence: 99%

Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review

2021

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Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the setting of inconclusive preliminary results. Dapsone is a commonly used treatment, but many therapeutic agents have emerged in recent years. The objective of this study is to provide a comprehensive overview of updates on the diagnosis and management of LAD. Materials and Methods: A literature search was conducted from May to June of 2021 for articles published in the last 5 years that were related to the diagnosis and management of LAD. Results: False-negative results in cases of drug-induced LAD and the presence of IgG and IgM antibodies on immunofluorescence studies were reported. Serration pattern analysis has been reported to be useful in distinguishing LAD from sublamina densa-type LAD. Rituximab, omalizumab, etanercept, IVIg, sulfonamides, topical corticosteroids, and others have been used successfully in adult and pediatric patients with varying disease severity. Topical corticosteroids were preferred for pediatric patients while rituximab and IVIg were used in adults with recalcitrant LAD. Sulfonamides were utilized in places without access to dapsone. Conclusion: In cases where preliminary biopsy results are negative and clinical suspicion is high, repeat biopsy and additional diagnostic studies should be used. Patient factors such as age, medical comorbidities, and disease severity play a role in therapeutic selection.

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“…This rather limited sample of studies on autoimmune blistering disease, adds an extremely important dimension to the “Concept,” albeit a little early in the overall scheme. In the two studies ( 45 , 46 ) in which the Ahmed protocol was not followed, the outcomes were not as favorable compared to outcomes where the protocol was followed ( 29 , 41 – 44 ). These limited observations validate its potential to produce long-term sustained clinical and serological remission.…”

Section: Proof Of Conceptmentioning

confidence: 86%

“…A British group in 2016 reported three patients with MMP and one with linear IgA bullous disease, non-responsive to conventional therapy, all four blind in one eye, treated with two cycles of RTX and 2–9 monthly infusions of IVIg ( 46 ). Ahmed protocol ( 41 ) was not used.…”

Section: Proof Of Conceptmentioning

confidence: 99%

Reversing Autoimmunity Combination of Rituximab and Intravenous Immunoglobulin

Ahmed

Kaveri

2018

Front. Immunol.

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In this concept paper, the authors present a unique and novel protocol to treat autoimmune diseases that may have the potential to reverse autoimmunity. It uses a combination of B cell depletion therapy (BDT), specifically rituximab (RTX) and intravenous immunoglobulin (IVIg), based on a specifically designed protocol (Ahmed Protocol). Twelve infusions of RTX are given in 6–14 months. Once the CD20+ B cells are depleted from the peripheral blood, IVIg is given monthly until B cells repopulation occurs. Six additional cycles are given to end the protocol. During the stages of B cell depletion, repopulation and after clinical recovery, IVIg is continued. Along with clinical recovery, significant reduction and eventual disappearance of pathogenic autoantibody occurs. Administration of IVIg in the post-clinical period is a crucial part of this protocol. This combination reduces and may eventually significantly eliminates inflammation in the microenvironment and facilitates restoring immune balance. Consequently, the process of autoimmunity and the phenomenon that lead to autoimmune disease are arrested, and a sustained and prolonged disease and drug-free remission is achieved. Data from seven published studies, in which this combination protocol was used, are presented. It is known that BDT does not affect check points. IVIg has functions that mimic checkpoints. Hence, when inflammation is reduced and the microenvironment is favorable, IVIg may restore tolerance. The authors provide relevant information, molecular mechanism of action of BDT, IVIg, autoimmunity, and autoimmune diseases. The focus of the manuscript is providing an explanation, using the current literature, to demonstrate possible pathways, used by the combination of BDT and IVIg in providing sustained, long-term, drug-free remissions of autoimmune diseases, and thus reversing autoimmunity, albeit for the duration of the observation.

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Combined Use of Rituximab and Intravenous Immunoglobulin for Severe Autoimmune Cicatricial Conjunctivitis—An Interventional Case Series

Abstract: Combination therapy of RTX and IVIg is a potent treatment regimen for recalcitrant autoimmune CC. Further prospective controlled studies on efficacy and safety are warranted before widespread clinical application.

Cited by 18 publications

References 19 publications

Laryngeal mucous membrane pemphigoid serves as a prognostic factor for poor response to treatment with rituximab

Laryngeal mucous membrane pemphigoid serves as a prognostic factor for poor response to treatment with rituximab

Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review

Reversing Autoimmunity Combination of Rituximab and Intravenous Immunoglobulin

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