Abstract
Methods The 32 patients identified for investigation presented with cases of chronic urticaria along with some of the following symptoms: joint and bone pain, chills, conjunctivitis, periodic fever, fatigue, weight loss, and hearing loss. 11 patients (34%) presented with Muckle-Wells syndrome. Seven patients (22%) presented with Familial Cold Autoinflammatory Syndrome and a history of treatment with rilanocept. Three patients (9.5%) presented with FMF. Three patients (9.5%) presented with Schnitzler’s syndrome. Eight patients (25%) presented with undiagnosed forms of CAPS. All patients showed elevated inflammatory markers, including Serum Amyloid A (SAA) and C-reactive protein (CRP). Nine patients with Muckle-Wells syndrome and a confirmed R260W mutation at the NLRP3 gene, and one patient with an undiagnosed form of CAPS began treatment with canakinumab. Patients received a 150 mg treatment via sub-cutaneous injection every eight weeks. Total injections to date for individual patients range from two to seven. The remaining 22 patients are currently being investigated for treatment with canakinumab.
Highlights
Rationale Patients seeking clinical treatment for urticaria, while presenting with elevated inflammatory markers, were found to be suffering from auto-inflammatory disorders, such as Cryopyrin Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF) and Schnitzler’s syndrome
The 32 patients identified for investigation presented with cases
22 patients are currently being investigated for treatment with canakinumab
Summary
Clinical experiences with canakinumab as a treatment for autoinflammatory disorders. From 8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases Dresden, Germany. Rationale Patients seeking clinical treatment for urticaria, while presenting with elevated inflammatory markers, were found to be suffering from auto-inflammatory disorders, such as Cryopyrin Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF) and Schnitzler’s syndrome. Patients were screened for, and/or began treatment with canakinumab
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