Refractory urticaria and the importance of diagnosing Schnitzler’s syndrome Journal Articles

abstract

• 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.

authors

• Nham, Tina
• Saleh, Carol
• Chu, Derek
• Vakaljan, Stephanie L
• Ohayon, Jason A
• Siegal, Deborah M

status

• published 

publication date

• April 2019

has subject area

• 1103 Clinical Sciences (FoR)
• Antirheumatic Agents (MeSH)
• Chronic Disease (MeSH)
• Diagnosis, Differential (MeSH)
• Humans (MeSH)
• Immunoglobulin M (MeSH)
• Injections, Subcutaneous (MeSH)
• Interleukin 1 Receptor Antagonist Protein (MeSH)
• Male (MeSH)
• Middle Aged (MeSH)
• Rare Diseases (MeSH)
• Schnitzler Syndrome (MeSH)
• Treatment Outcome (MeSH)
• Urticaria (MeSH)

published in

• BMJ Case Reports  Journal

keywords

• Antirheumatic Agents
• Chronic Disease
• Diagnosis, Differential
• Humans
• Immunoglobulin M
• Injections, Subcutaneous
• Interleukin 1 Receptor Antagonist Protein
• Male
• Middle Aged
• Rare Diseases
• Schnitzler Syndrome
• Treatment Outcome
• Urticaria
• biological agents
• immunology

Digital Object Identifier (DOI)

• 10.1136/bcr-2018-228546

start page

• e228546

end page

• e228546

volume

• 12

issue

• 4