Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurologi...

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Bibliographic Details
Published in:Clinical rheumatology 2017-09, Vol.36 (9), p.2159-2162
Main Authors: Aguirre-Valencia, David, Posso-Osorio, Iván, Bravo, Juan-Carlos, Bonilla-Abadía, Fabio, Tobón, Gabriel J., Cañas, Carlos A.
Format: Article
Language:English
Subjects:
Adolescent
Asthma
Asthma - drug therapy
Bronchial Spasm - drug therapy
Bronchospasm
Case Based Review
Churg-Strauss syndrome
Churg-Strauss Syndrome - complications
Churg-Strauss Syndrome - drug therapy
Cyclophosphamide
Eosinophilia
Female
Gastric Mucosa - pathology
Granulomatosis
Heart diseases
Humans
Immunoglobulin E
Immunotherapy
Inflammatory diseases
Kidneys
Leukocytes (eosinophilic)
Medicine
Medicine & Public Health
Monoclonal antibodies
Omalizumab - administration & dosage
Plasmapheresis
Remission
Respiratory tract
Rheumatology
Rhinitis
Rhinitis - drug therapy
Rituximab
Rituximab - administration & dosage
Steroid hormones
Treatment Outcome
Vasculitis
Vein & artery diseases
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Description
Summary:Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms. Here, we report a 16-year-old female patient with severe EGPA (gastrointestinal and cutaneous vasculitis, rhinitis and asthma) refractory to conventional treatment. She was treated with rituximab, which enabled rapid control of the vasculitis component of the disease, but there was no response to rhinitis and asthma. Additionally, she developed severe bronchospasm during rituximab infusion. Sequential rituximab and omalizumab were initiated, leading to remission of all manifestations of vasculitis, rhinitis, and asthma, in addition to bronchospasm related to rituximab infusion.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-017-3780-9