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

Yasuhiro Kano; Makoto Sugihara

doi:10.2169/internalmedicine.0359-22

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

Intern Med. 2023 May 1;62(9):1361-1364. doi: 10.2169/internalmedicine.0359-22. Epub 2022 Sep 28.

Authors

Yasuhiro Kano^{1

2}, Makoto Sugihara²

Affiliations

¹ Department of General Internal Medicine, Tokyo Metropolitan Tama Medical Center, Japan.
² Department of Rheumatic Diseases, Tokyo Metropolitan Tama-Hokubu Medical Center, Japan.

Abstract

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.

Keywords: Schnitzler syndrome; alkaline phosphatase; diagnostic error; fever of unknown origin; liver biopsy.

Publication types

Case Reports

MeSH terms

Alkaline Phosphatase
Fever of Unknown Origin* / diagnosis
Fever of Unknown Origin* / etiology
Humans
Schnitzler Syndrome* / complications
Schnitzler Syndrome* / diagnosis
Schnitzler Syndrome* / drug therapy

Substances

Alkaline Phosphatase