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