Refractory urticaria and the importance of diagnosing Schnitzler's syndrome.
A 52-year-old man presented with chronic urticaria that was refractory to standard chronic spontaneous urticaria (CSU) treatment. Over time, he developed systemic symptoms including fatigue, weight loss, arthralgia and bone pain. His laboratory investigations also became significant for microcytic anaemia, neutrophilia and elevated C reactive protein, erythrocyte sedimentation rate and IgE levels, in addition to an IgM monoclonal protein. He achieved only partial remission with typical medications for CSU including omalizumab, cyclosporine and cetirizine. After 6 years, his worsening symptoms and abnormal investigations led to a rare diagnosis of Schnitzler's syndrome and a trial of the interleukin 1 receptor antagonist, anakinra, which caused a rapid and complete resolution of his symptoms.
['Nham|Tina|T|', 'Saleh|Carol|C|', 'Chu|Derek|D|', 'Vakaljan|Stephanie L|SL|', 'Ohayon|Jason A|JA|', 'Siegal|Deborah M|DM|']
D018501:Antirheumatic Agents D002908:Chronic Disease D003937:Diagnosis, Differential D006801:Humans D007075:Immunoglobulin M D007279:Injections, Subcutaneous D053590:Interleukin 1 Receptor Antagonist Protein D008297:Male D008875:Middle Aged D035583:Rare Diseases D019873:Schnitzler Syndrome D016896:Treatment Outcome D014581:Urticaria
biological agents immunology