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Case Reports
. 2015 May 15:2015:bcr2014206421.
doi: 10.1136/bcr-2014-206421.

Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis

Catherine E Najem  1 , Rajwardhan Yadav  2 , Elise Carlson  2
Affiliations
Case Reports

Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis

Catherine E Najem et al. BMJ Case Rep. .

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare eosinophil-rich disorder characterised by necrotising granulomatous inflammation affecting small to medium sized vessels. Extrapulmonary manifestations can be life-threatening when heart, central nervous system (CNS), gastrointestinal tract or kidneys are affected. We describe a case of a 56-year-old woman with a long-standing history of asthma, who presented with an acute sudden painless loss of vision after she had been recently diagnosed with EGPA and induced with pulse steroids and cyclophosphamide. The patient had a complicated hospital course with multisystemic involvement of active vasculitis, involving heart, kidneys, muscles, eyes and CNS. The patient's devastating condition responded remarkably to Rituximab. The role of Rituximab in EGPA is not yet proven. Few cases are reported in the literature about the role of Rituximab in EGPA, of which only one described retinal artery occlusion as a presentation of a recently treated patient with EGPA.

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Figures

Figure 1
Figure 1
Myositis of the muscles. (A) MRI of the thighs demonstrating a focal oedema in the left vastus medialis muscle (arrow) and right vastus lateralis muscle (curved arrow), with oedema of the intermuscular fascia; and (B) cardiac MRI demonstrating anterioseptal punctuate enhancement (arrow) with subendocardial enhancement (curved arrow) of the inferior wall base.
Figure 2
Figure 2
Kidney biopsy-light microscopy revealing (A) a dense eosinophilic infiltrate in the subcapsular area with >70 eosinophils/hpf (high power field); and (B) a glomerulus with segmental necrosis, karyorrhectic debris and fibrinoid necrosis (arrow).
See this image and copyright information in PMC

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