Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Autoimmune Phenomena in Muckle-Wells Syndrome (MWS).

Gramlich1,  Katharina, Klein4,  Reinhild, Hansmann3,  Sandra, Benseler5,  Susanne M., Kuemmerle-Deschner2,  Jasmin B.

Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tuebingen, Germany
Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tuebingen, Germany
Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Germany
Division of Rheumatology, Department of Internal Medicine, University Hospital Tübingen
The Hospital for Sick Children, Toronto, ON, Canada


Muckle-Wells Syndrome (MWS) is a rare inherited autoinflammatory disease which is driven by excessive interleukin-1b (IL-1b) production. IL-1 blocking therapies including anakinra and canakinumab have been shown to be safe and effective. Absence of autoantibodies is the hallmark of autoinflammatory diseases, discriminating them from autoimmune diseases. The aim of this study was to determine the frequency of autoantibodies in MWS-patients prior to therapy and during anakinra and canakinumab therapy.


A single center observational study was performed. Patients with confirmed MWS were tested for autoantibodies at baseline prior to IL-1 blockade, within 6 months of treatment with anakinra or canakinumab respectively, and at 6–12 months of treatment. Monitoring included antinuclear antibodies (ANA), smooth muscle antibodies (SMA), anti-endothelial antibodies (AEA), anti-sarcolemmal antibodies (ASA) by immunofluorescence test, and antibodies against serotonin, gangliosides and CNS-tissue of the IgG and IgM type by ELISA.


We included 20 patients, 8 males, 12 females, median age at diagnosis, 34 years (3,5–72). All patients were screened for the presence of autoantibodies before starting anti-IL-1 therapy. Positive ANA were detected in 3 patients (15%); SMA, AEA or ASA were found in 6 patients (30%). 2/4 tested were positive for anti-serotonin antibodies and 2/15 for anti-ganglioside antibodies. Antibodies against CNS-tissue were detected in 5/15 tested.

During anakinra therapy no formation of ANA in initially ANA-negative patients was observed, ANA newly emerged in 4/14 during canakinumab therapy. New antibody formation against gangliosides occurred in 2 patients during anakinra and in 1 patient during canakinumab therapy. Antibodies against serotonin and CNS-tissue were frequently detected during both anakinra and canakinumab therapy; however there was no evidence of new formation of these antibodies. SMA, AEA and ASA were unchanged over the whole study period. During the overall study period, 15/20 patients (75%) were positive for antibodies against serotonin, gangliosides and/ or CNS-tissue at least at one time point.


Patients with MWS can have ANAs and antibodies against serotonin, gangliosides and CNS-tissue. IL-1 blockade may increase the formation of autoantibodies in previously antibody-negative patients. The clinical relevance of the presence of autoantibodies in patients with MWS is still unclear.

To cite this abstract, please use the following information:
Gramlich, Katharina, Klein, Reinhild, Hansmann, Sandra, Benseler, Susanne M., Kuemmerle-Deschner, Jasmin B.; Autoimmune Phenomena in Muckle-Wells Syndrome (MWS). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :894
DOI: 10.1002/art.28662

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