Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome.
BACKGROUND
Treatments for Churg-Strauss syndrome (CSS), a rare eosinophilic vasculitis characterized by asthma, sinusitis, peripheral eosinophilia, pulmonary infiltrates, and tissue infiltration, are limited by toxicity or poor efficacy. Levels of IL-5, a cytokine regulating eosinophils, can be increased in patients with CSS. Mepolizumab, a humanized monoclonal anti-IL-5 antibody, decreases steroid requirements in patients with non-CSS hypereosinophilic syndromes.


OBJECTIVE
The purpose of this study was to assess whether mepolizumab would safely allow corticosteroid tapering in patients with steroid-dependent CSS while decreasing serum markers of disease activity.


METHODS
This open-label pilot study treated 7 patients with 4 monthly doses of mepolizumab to assess whether it safely decreased CSS disease activity and permitted tapering of systemic corticosteroids.


RESULTS
Mepolizumab was safe and well tolerated in patients with CSS. Mepolizumab reduced eosinophil counts and allowed for safe corticosteroid reduction in all 7 subjects. On cessation of mepolizumab, CSS manifestations recurred, necessitating corticosteroid bursts.


CONCLUSIONS
Mepolizumab is a safe and well-tolerated therapy in patients with CSS, offering clinical benefit by enabling corticosteroid tapering while maintaining clinical stability.
Department of Pediatrics, Children's Hospital Boston, Boston, Mass., USA.
Sophia
S
Kim
Gautham
G
Marigowda
Eyal
E
Oren
Elliot
E
Israel
Michael E
ME
Wechsler
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage* / Q000009:adverse effects; D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage* / Q000009:adverse effects; D061067:Antibodies, Monoclonal, Humanized; D002452:Cell Count; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D004804:Eosinophils / Q000187:drug effects* / Q000473:pathology; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D012852:Sinusitis; D014657:Vasculitis; D028761:Withholding Treatment
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C434107:mepolizumab