Monoclonal Gammopathy, Arthralgias, and Recurrent Fever Syndrome: A New Autoinflammatory Syndrome?

Alexandre Terré; Alexis Talbot; Camille Louvrier; Jean Baptiste Picque; Matthieu Mahévas; David Boutboul; Serge Amselem; Irina Giurgea; Gilles Grateau; Sophie Georgin-Lavialle; French Network of Dysimmune Disorders Associated with Hemopathies

doi:10.3899/jrheum.181204

Monoclonal Gammopathy, Arthralgias, and Recurrent Fever Syndrome: A New Autoinflammatory Syndrome?

J Rheumatol. 2019 Nov;46(11):1535-1539. doi: 10.3899/jrheum.181204. Epub 2019 Mar 15.

Authors

Alexandre Terré^{1

2}, Alexis Talbot^{1

2}, Camille Louvrier^{1

2}, Jean Baptiste Picque^{1

2}, Matthieu Mahévas^{1

2}, David Boutboul^{1

2}, Serge Amselem^{1

2}, Irina Giurgea^{1

2}, Gilles Grateau^{1

2}, Sophie Georgin-Lavialle; French Network of Dysimmune Disorders Associated with Hemopathies

Affiliations

¹ From the Sorbonne Université, AP-HP, Hôpital Tenon, Service de Médecine Interne, Centre de Référence des Maladies Auto-inflammatoires et des Amyloses d'Origine Inflammatoire (CEREMAIA); Service d'Immuno-hélatologie, Hôpital St. Louis; INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université, Paris; Service de Médecine Interne, Centre Hospitalier d'Auxerre, Auxerre; Service de Médecine Interne, Centre Hospitalier Universitaire (CHU) Henri Mondor; and Service d'Immunologie Clinique, Hôpital St. Louis, Paris, France.
² A. Terré, MD, Sorbonne Université, AP-HP, Hôpital Tenon, Service de Médecine Interne, CEREMAIA; A. Talbot, MD, Service d'Immunohélatologie, Hôpital St. Louis; C. Louvrier, PharmD, INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université; J.B. Picque, MD, Service de Médecine Interne, Centre Hospitalier d'Auxerre; M. Mahévas, MD, Service de Médecine Interne, CHU Henri Mondor; D. Boutboul, MD, Service d'Immunologie Clinique, Hôpital St. Louis; S. Amselem, MD, PhD, INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université; I. Giurgea, MD, PhD, INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université; G. Grateau, MD, PhD, Sorbonne Université, AP-HP, Hôpital Tenon, Service de Médecine Interne, CEREMAIA, and INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université; S. Georgin-Lavialle, MD, PhD, Sorbonne Université, AP-HP, Hôpital Tenon, Service de Médecine Interne, CEREMAIA, and INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université.

PMID: 30877203
DOI: 10.3899/jrheum.181204

Abstract

Objective: To describe a new autoinflammatory syndrome with recurrent fever and monoclonal gammopathy that differs from Schnitzler syndrome.

Methods: We conducted a retrospective study of patients with monoclonal gammopathy and recurrent fever of unknown origin.

Results: Five patients were studied; median age at onset of symptoms was 44 years. Median frequency of fever attacks was 6 episodes per year. In the absence of treatment, the median duration of fevers was 3 days.

Conclusion: This new autoinflammatory syndrome is defined by an association among monoclonal gammopathy, arthralgias, and recurrent fever.

Keywords: INNATE IMMUNE RESPONSE; PARAPROTEINEMIAS; SCHNITZLER SYNDROME.

Publication types

Multicenter Study

MeSH terms

Adult
Aged
Antibodies, Monoclonal / blood
Arthralgia / complications*
Arthralgia / drug therapy
C-Reactive Protein / analysis
Female
Fever / complications*
Fever / drug therapy
Follow-Up Studies
Humans
Immunoglobulin G / blood
Immunoglobulin M / blood
Interleukin 1 Receptor Antagonist Protein / therapeutic use
Male
Middle Aged
Recurrence
Retrospective Studies
Schnitzler Syndrome / complications*
Schnitzler Syndrome / drug therapy
Syndrome
Treatment Outcome

Substances

Antibodies, Monoclonal
Immunoglobulin G
Immunoglobulin M
Interleukin 1 Receptor Antagonist Protein
C-Reactive Protein