Schnitzler's syndrome associated with pancreatitis: A disease of IL-1 dysregulation

Cecilia A. Larocca, John W. McEvoy, Carla L. Ellis, Jacqueline Junkins-Hopkins, Todd Matthew Kolb, Alan Baer, Brian Thomas Garibaldi

Research output: Contribution to journalArticle

Abstract

Schnitzler's syndrome (SS) is a rare inflammatory disease of unknown origin characterized by chronic urticaria and monoclonal gammopathy (usually IgM) associated with at least two of the following components: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, lymphadenopathy, elevated erythrocyte sedimentation rate, leukocytosis, and/or abnormal findings on bone morphological investigations. To date, about 100 cases have been described with only 4 being reported in the USA. The mean time to diagnosis from the onset of disease is 5.4 years, given the varied symptoms with which patients may present. The pathogenesis of SS remains unknown but likely involves dysregulation of the IL-1 pathway. We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra.

Original languageEnglish (US)
Pages (from-to)169-174
Number of pages6
JournalClinical Rheumatology
Volume31
Issue number1
DOIs
StatePublished - Jan 2012

Fingerprint

Schnitzler Syndrome
Interleukin-1
Pancreatitis
Paraproteinemias
Leukocytosis
Arthritis
Immunoglobulin M
Fever
Interleukin 1 Receptor Antagonist Protein
Bone and Bones
Hepatomegaly
Interleukin-1 Receptors
Blood Sedimentation
Splenomegaly
Urticaria
Chronic Pancreatitis
Arthralgia
Rare Diseases
Skin Diseases
Weight Loss

Keywords

  • IL-1
  • Monoclonal gammopathy
  • Pancreatitis
  • Schniztler's syndrome
  • Urticaria

ASJC Scopus subject areas

  • Rheumatology

Cite this

Schnitzler's syndrome associated with pancreatitis : A disease of IL-1 dysregulation. / Larocca, Cecilia A.; McEvoy, John W.; Ellis, Carla L.; Junkins-Hopkins, Jacqueline; Kolb, Todd Matthew; Baer, Alan; Garibaldi, Brian Thomas.

In: Clinical Rheumatology, Vol. 31, No. 1, 01.2012, p. 169-174.

Research output: Contribution to journalArticle

Larocca, Cecilia A. ; McEvoy, John W. ; Ellis, Carla L. ; Junkins-Hopkins, Jacqueline ; Kolb, Todd Matthew ; Baer, Alan ; Garibaldi, Brian Thomas. / Schnitzler's syndrome associated with pancreatitis : A disease of IL-1 dysregulation. In: Clinical Rheumatology. 2012 ; Vol. 31, No. 1. pp. 169-174.
@article{2d6708a343df4928b289349ce742e9ec,
title = "Schnitzler's syndrome associated with pancreatitis: A disease of IL-1 dysregulation",
abstract = "Schnitzler's syndrome (SS) is a rare inflammatory disease of unknown origin characterized by chronic urticaria and monoclonal gammopathy (usually IgM) associated with at least two of the following components: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, lymphadenopathy, elevated erythrocyte sedimentation rate, leukocytosis, and/or abnormal findings on bone morphological investigations. To date, about 100 cases have been described with only 4 being reported in the USA. The mean time to diagnosis from the onset of disease is 5.4 years, given the varied symptoms with which patients may present. The pathogenesis of SS remains unknown but likely involves dysregulation of the IL-1 pathway. We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra.",
keywords = "IL-1, Monoclonal gammopathy, Pancreatitis, Schniztler's syndrome, Urticaria",
author = "Larocca, {Cecilia A.} and McEvoy, {John W.} and Ellis, {Carla L.} and Jacqueline Junkins-Hopkins and Kolb, {Todd Matthew} and Alan Baer and Garibaldi, {Brian Thomas}",
year = "2012",
month = "1",
doi = "10.1007/s10067-011-1804-4",
language = "English (US)",
volume = "31",
pages = "169--174",
journal = "Clinical Rheumatology",
issn = "0770-3198",
publisher = "Springer London",
number = "1",

}

TY - JOUR

T1 - Schnitzler's syndrome associated with pancreatitis

T2 - A disease of IL-1 dysregulation

AU - Larocca, Cecilia A.

AU - McEvoy, John W.

AU - Ellis, Carla L.

AU - Junkins-Hopkins, Jacqueline

AU - Kolb, Todd Matthew

AU - Baer, Alan

AU - Garibaldi, Brian Thomas

PY - 2012/1

Y1 - 2012/1

N2 - Schnitzler's syndrome (SS) is a rare inflammatory disease of unknown origin characterized by chronic urticaria and monoclonal gammopathy (usually IgM) associated with at least two of the following components: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, lymphadenopathy, elevated erythrocyte sedimentation rate, leukocytosis, and/or abnormal findings on bone morphological investigations. To date, about 100 cases have been described with only 4 being reported in the USA. The mean time to diagnosis from the onset of disease is 5.4 years, given the varied symptoms with which patients may present. The pathogenesis of SS remains unknown but likely involves dysregulation of the IL-1 pathway. We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra.

AB - Schnitzler's syndrome (SS) is a rare inflammatory disease of unknown origin characterized by chronic urticaria and monoclonal gammopathy (usually IgM) associated with at least two of the following components: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, lymphadenopathy, elevated erythrocyte sedimentation rate, leukocytosis, and/or abnormal findings on bone morphological investigations. To date, about 100 cases have been described with only 4 being reported in the USA. The mean time to diagnosis from the onset of disease is 5.4 years, given the varied symptoms with which patients may present. The pathogenesis of SS remains unknown but likely involves dysregulation of the IL-1 pathway. We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra.

KW - IL-1

KW - Monoclonal gammopathy

KW - Pancreatitis

KW - Schniztler's syndrome

KW - Urticaria

UR - http://www.scopus.com/inward/record.url?scp=84856746600&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84856746600&partnerID=8YFLogxK

U2 - 10.1007/s10067-011-1804-4

DO - 10.1007/s10067-011-1804-4

M3 - Article

C2 - 21710158

AN - SCOPUS:84856746600

VL - 31

SP - 169

EP - 174

JO - Clinical Rheumatology

JF - Clinical Rheumatology

SN - 0770-3198

IS - 1

ER -