Successful treatment of Schnitzler's syndrome with anakinra after failure of rituximab trial

International Journal of Immunopathology and Pharmacology
N CascavillaG D'Arena

Abstract

We report a 50-year-old man who presented with a 5-year history of an intermittent widespread pruritic urticarioid rash and fever, fatigue, arthralgia and a monoclonal immunoglobulin-M paraprotein. The patient was initially treated with antihistamines and corticosteroids without the disappearance of symptoms. A skin biopsy from the urticarial rash on the thorax was performed, revealing dermal mononuclear and polymorphonuclear cell infiltrate and normal epidermis. A diagnosis of Schnitzler's syndrome (SS), a rare disorder in which the simultaneous occurrence of monoclonal gammopathy and chronic urticaria is usually observed, was made. After an unsuccessful trial with rituximab at a dosage of 375 mg/sqm weekly for 4 consecutive weeks, the patient was treated with anakinra, an inhibitor of interleukin-1alpha that is thought to be involved in the pathogenesis of the disease, at a dose of 100 mg daily given subcutaneously. He showed a prompt response to the drug and he is still well and symptom-free after 12 months of follow-up. On the basis of both this experience and the review of the literature we conclude that anakinra may be a promising option for the treatment of SS. However, these results need to be confirmed on a larger numb...Continue Reading

References

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Citations

Dec 31, 2014·Molecular Medicine·Charles Anthony Dinarello
Dec 23, 2017·Clinical and Experimental Dermatology·A SalamD H McGibbon
May 1, 2019·BMJ Case Reports·Tina NhamDeborah M Siegal
Apr 14, 2020·The Journal of Dermatological Treatment·Kyle TegtmeyerPeter A Lio
Dec 15, 2010·Orphanet Journal of Rare Diseases·Dan Lipsker
Nov 26, 2010·Current Allergy and Asthma Reports·Justin R Yu, Kieron S Leslie
Jul 11, 2013·Case Reports in Medicine·Gabriel TinocoDeepthi Moola
Jan 24, 2014·Clinical Science·Ilja StrizAlena Sekerkova
Mar 14, 2013·Allergy·A SimonD Lipsker
Jan 1, 2014·Clinical and Translational Allergy·Heleen D de Koning

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