Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels

Schnitzler syndrome (SchS) is a rare, acquired, autoinflammatory disease that is sometimes associated with a fever of unknown origin (FUO). Elevated alkaline phosphatase (ALP) stemming from abnormal bone remodeling is a characteristic laboratory finding of SchS and is included in the diagnostic crit...

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Bibliographic Details
Published in:Internal Medicine 2023/05/01, Vol.62(9), pp.1361-1364
Main Authors: Kano, Yasuhiro, Sugihara, Makoto
Format: Article
Language:English
Subjects:
Alkaline Phosphatase
Biopsy
Bone remodeling
Case Report
Case reports
diagnostic error
Fever
fever of unknown origin
Fever of Unknown Origin - diagnosis
Fever of Unknown Origin - etiology
Humans
Inflammatory diseases
Internal medicine
liver biopsy
Schnitzler syndrome
Schnitzler Syndrome - complications
Schnitzler Syndrome - diagnosis
Schnitzler Syndrome - drug therapy
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Description
Summary:Schnitzler syndrome (SchS) is a rare, acquired, autoinflammatory disease that is sometimes associated with a fever of unknown origin (FUO). Elevated alkaline phosphatase (ALP) stemming from abnormal bone remodeling is a characteristic laboratory finding of SchS and is included in the diagnostic criteria. However, its utility as a clue to the diagnosis of SchS has been under-emphasized. We herein report a case of SchS presenting with a FUO and highly elevated ALP concentration, which led to repeated, unnecessary liver biopsies.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.0359-22