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

Research output: Contribution to journalArticle

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-1α 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 number of patients.

Original languageEnglish
Pages (from-to)633-636
Number of pages4
JournalInternational Journal of Immunopathology and Pharmacology
Volume23
Issue number2
Publication statusPublished - Apr 2010

Fingerprint

Schnitzler Syndrome
Interleukin 1 Receptor Antagonist Protein
Exanthema
Paraproteins
Skin
Paraproteinemias
Histamine Antagonists
Urticaria
Arthralgia
Interleukin-1
Epidermis
Fatigue
Immunoglobulin M
Adrenal Cortex Hormones
Fever
Thorax
Therapeutics
Biopsy
Pharmaceutical Preparations
Rituximab

Keywords

  • Anakinra
  • Schnitzler's syndrome

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Pharmacology

Cite this

@article{f426bf99cb9d46e7b263199931fa49da,
title = "Successful treatment of Schnitzler's syndrome with anakinra after failure of rituximab trial",
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-1α 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 number of patients.",
keywords = "Anakinra, Schnitzler's syndrome",
author = "Nicola Cascavilla and M. Bisceglia and G. D'Arena",
year = "2010",
month = "4",
language = "English",
volume = "23",
pages = "633--636",
journal = "International Journal of Immunopathology and Pharmacology",
issn = "0394-6320",
publisher = "Biomedical Research Press s.a.s.",
number = "2",

}

TY - JOUR

T1 - Successful treatment of Schnitzler's syndrome with anakinra after failure of rituximab trial

AU - Cascavilla, Nicola

AU - Bisceglia, M.

AU - D'Arena, G.

PY - 2010/4

Y1 - 2010/4

N2 - 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-1α 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 number of patients.

AB - 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-1α 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 number of patients.

KW - Anakinra

KW - Schnitzler's syndrome

UR - http://www.scopus.com/inward/record.url?scp=77955349737&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77955349737&partnerID=8YFLogxK

M3 - Article

C2 - 20646359

AN - SCOPUS:77955349737

VL - 23

SP - 633

EP - 636

JO - International Journal of Immunopathology and Pharmacology

JF - International Journal of Immunopathology and Pharmacology

SN - 0394-6320

IS - 2

ER -