Rituximab for eosinophilic granulomatosis with polyangiitis with severe vasculitic neuropathy: Case report and review of current clinical evidence

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Abstract

Objective

Rituximab is approved for the treatment of granulomatosis with polyangiitis and microscopic polyangiitis. Our objective was to review published clinical evidence on the efficacy of rituximab in the treatment of eosinophilic granulomatosis and polyangiitis (EGPA).

Methods

We describe a case of refractory EGPA with severe vasculitic neuropathy, which responded impressively to B-cell-depleting therapy. A systematic search of the English literature was also performed to capture all available clinical evidence on the use of rituximab in EGPA.

Results

We identified a total of 73 EGPA patients who have been treated with rituximab, all data coming from case series or isolated case reports. The majority of patients (85.1%) were treated for refractory or relapsing disease; a mean (SD) of 2.1 (0.9) different immunosuppressive agents were used prior to rituximab administration. Efficacy of RTX therapy was significant in the majority of cases and in a wide variety of disease manifestations; however, a lack of standardized assessment of disease activity before and after treatment was observed in many reports. Overall, 54.0% of patients were treated with a single cycle of rituximab and only 10.8% experienced relapses of the disease. Few significant side effects were observed during a highly variable period of follow-up (3 months to 5 years), mainly severe infections and allergic reactions.

Conclusions

RTX seems to be effective in cases of severe EGPA refractory to standard of care immunosuppressive treatment, although support comes from case reports and non-controlled studies.

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg–Strauss syndrome) is a small-vessel, antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Although it shares common features with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), EGPA also displays some unique characteristics, including the striking correlation with asthma, the abundance of circulating and tissue eosinophils, and the frequent heart involvement, which confer a distinct clinical phenotype [1]. The lung, skin, and peripheral nervous system (PNS) are commonly involved, but EGPA can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous system (CNS).

Rituximab (RTX), a chimeric monoclonal antibody against the CD20 molecule on B-lymphocytes, has been successfully used and is now officially approved for treatment of patients with GPA and MPA [2], [3]. However, its efficacy in EGPA has been tested in a very limited number of case reports and small case series. Here, we report a case of severe vasculitic neuropathy and glomerulonephritis associated with EGPA, which responded impressively to RTX. We accompany it with a literature review of published EGPA cases treated with B-cell depletion and discuss relevant issues.

Section snippets

Methods

A case of EGPA treated with RTX is presented. We also searched the English language literature using the PubMed database from January 1966 to December 2014, using the following index terms: (“rituximab” OR “B-cell depletion” OR “anti-CD20”) AND (“eosinophilic granulomatosis with polyangiitis” OR “Churg–Strauss syndrome”). Original articles, case series, and case reports were included in the search. Pertinent articles identified by manual search within the reference list of the originally

Case presentation

The patient, a 51-year-old man with a past medical history of recurrent nasal polyposis but no known asthma, presented with a 10-day history of arthralgias and progressive numbness of the upper and lower extremities. Two weeks prior to onset of symptoms, he had been treated for a possible viral respiratory tract infection with oral antibiotics and inhaled β-agonists.

Physical examination on admission revealed tachycardia (120 bpm), widespread bilateral wheezing, and symmetrical polyarthritis of

Discussion

EGPA is the least common among the three ANCA-vasculitides. Left untreated, EGPA has a reported 5-year survival of 25%; however, implementation of aggressive treatment has led to a substantial improvement, with 10-year survival rates reaching 90% in recent cohorts [24], [25]. We herein reported a case of successful use of RTX in a case of EGPA with severe vasculitic neuropathy and glomerulonephritis and reviewed the quality of current clinical evidence to support its use in this rare disease.

We

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