Work

Schnitzler Syndrome: An Under-Recognised Cause of Pyrexia of Unknown Origin

Year: 2025

Type: article

Abstract: Schnitzler syndrome is a rare adult-onset autoinflammatory disease characterised by urticarial rash, monoclonal gammopathy, and systemic inflammation. We present the first reported case in Hong Kong, involving a 37-year-old female with a prolonged diagnostic journey of nearly a decade after initially presenting with pyrexia of unknown origin. Extensive investigations ruled out other causes, and the diagnosis was made using the Strasbourg diagnostic criteria for Schnitzler syndrome. Treatment with canakinumab, an interleukin (IL)-1[Formula: see text] inhibitor, resulted in rapid resolution of symptoms. This case highlights the importance of early recognition and timely diagnosis to improve patient outcomes in this rare condition.

Source: Journal of Clinical Rheumatology and Immunology

DOI: 10.1142/s2661341725720010

Authors: Dennis Tin Ho Chan, Ling Yin Ho

Institutions: Alice Ho Miu Ling Nethersole Hospital, Tai Po Hospital

Language: English

Topic: Inflammasome and immune disorders

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $800