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Dysregulated mature IL-1β production in familial Mediterranean fever

Kiyoshi Migita¹, Yasumori Izumi², Keita Fujikawa²

¹Department of Rheumatology and Clinical Research Center, Nagasaki Medical Center, Omura, Department of Rheumatology, Isahaya Health Insurance General Hospital, Nagasaki, Department of Infection and Host Defense, Graduate School of Medicine, Shinshu University, Matsumoto, Department of Pathology, Ehime University Graduate School of Medicine and Proteo-Science Center, Ehime, Department of Rheumatology, Sasebo City General Hospital, Sasebo, Department of Rheumatology, Shinto Kumamoto Hospital, Kumamoto, Clinical Research Center, Sagamihara National Hospital, National Hospital Organization, Sagamihara, Institute of Tropical Medicine (NEKKEN), Nagasaki University and Department of Rheumatology, Nagasaki University Hospital, Nagasaki, Japan. migita@nagasaki-mc.com.

Rheumatology (Oxford, England)

|September 22, 2014

Abstract

Objective:

The aim of this study was to analyse the role of circulating cleaved IL-1β in patients with FMF.

Methods:

We enrolled 20 patients with FMF (5 males and 15 females), 22 patients with RA (4 males and 18 females) and 22 healthy controls (6 males and 16 females). Serum levels of serum amyloid A (SAA) were measured by ELISA. We also determined whether IL-1β was present as the cleaved form (p17) in the sera of FMF patients by immunoblotting using anti-cleaved IL-1β antibody.

Results:

Although SAA concentrations were elevated in the sera, there was no significant difference in these concentrations between FMF patients and RA patients. Immunoblot analysis demonstrated that the cleaved form of IL-1β (p17) was present in sera from FMF patients during febrile attack periods, but not in healthy controls. Bands representing the cleaved form of IL-1β were not detected in serum from FMF patients at non-febrile attack periods or remission periods under colchicine treatment. The amounts of cleaved IL-1β (p17) were significantly higher in patients with FMF compared with those in patients with RA in the inflammatory phase.

Conclusion:

The cleaved form of IL-1β is a valuable biomarker for monitoring disease activity and response to colchicine treatment in patients with FMF. It might be useful to discriminate FMF from other non-IL-1β-mediated inflammatory disorders.

Insights

The cleaved form of interleukin-1β (IL-1β) is elevated in Familial Mediterranean Fever (FMF) patients during attacks. This biomarker helps monitor FMF activity and colchicine treatment response.

Area of Science:

Immunology
Rheumatology
Genetics

Background:

Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder characterized by recurrent febrile episodes.
Interleukin-1β (IL-1β) plays a crucial role in FMF pathogenesis.
The presence and role of cleaved IL-1β in FMF patients require further investigation.

Purpose of the Study:

To analyze the role of circulating cleaved IL-1β in patients diagnosed with FMF.
To assess cleaved IL-1β as a potential biomarker for FMF disease activity and treatment response.

Main Methods:

Serum samples were collected from 20 FMF patients, 22 Rheumatoid Arthritis (RA) patients, and 22 healthy controls.
Serum amyloid A (SAA) levels were measured using ELISA.
Cleaved IL-1β (p17) presence in serum was determined by immunoblotting.

Main Results:

SAA levels were elevated in FMF and RA patients but did not differ significantly between the groups.
Cleaved IL-1β (p17) was detected in FMF patients' serum during febrile attacks but not in healthy controls or FMF patients in remission.
Significantly higher levels of cleaved IL-1β (p17) were observed in FMF patients compared to RA patients during the inflammatory phase.

Conclusions:

Circulating cleaved IL-1β (p17) serves as a valuable biomarker for monitoring FMF disease activity.
Cleaved IL-1β can indicate response to colchicine treatment in FMF patients.
This biomarker may aid in differentiating FMF from other inflammatory conditions not mediated by IL-1β.

Read full analysis

Keywords:

IL-1β autoinflammatory disease biomarker familial Mediterranean fever serum amyloid A

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