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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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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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container_title	Internal Medicine
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creator	Kano, Yasuhiro Sugihara, Makoto
description	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.
doi_str_mv	10.2169/internalmedicine.0359-22
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We herein report a case of SchS presenting with a FUO and highly elevated ALP concentration, which led to repeated, unnecessary liver biopsies.</description><subject>Alkaline Phosphatase</subject><subject>Biopsy</subject><subject>Bone remodeling</subject><subject>Case Report</subject><subject>Case reports</subject><subject>diagnostic error</subject><subject>Fever</subject><subject>fever of unknown origin</subject><subject>Fever of Unknown Origin - diagnosis</subject><subject>Fever of Unknown Origin - etiology</subject><subject>Humans</subject><subject>Inflammatory diseases</subject><subject>Internal medicine</subject><subject>liver biopsy</subject><subject>Schnitzler syndrome</subject><subject>Schnitzler Syndrome - complications</subject><subject>Schnitzler Syndrome - diagnosis</subject><subject>Schnitzler Syndrome - drug therapy</subject><issn>0918-2918</issn><issn>1349-7235</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><recordid>eNplkVtvEzEQhS0EoqHwF5AlXnjZ1re9-AlVVQtIEa1U-mw53tmsU8cOtpOq_Hq8ShpBkaXxw3zneMYHIUzJGaONPLc-Q_TaraG3xno4I7yWFWOv0IxyIauW8fo1mhFJu4qVcoLepbQihHetZG_RCW9oSynjMwR3ZvQ2_3YQ8d2T72NYA76NkMBn65dYJ6zxNexKOwz43j_48OjxTbRL6_GjzSO-crDTGXp84R60K8Pg2zGkzaizToDnRerSe_Rm0C7Bh8N9iu6vr35efqvmN1-_X17MK9NQkiutSQ98YcrhbU3FYgF0EF2viZZ9b8QgJK1rs-gHQeqOCNobTg0jHWe1kMLwU_Rl77vZLsrfmLJE1E5tol3r-KSCturfjrejWoadoqS4tE1THD4fHGL4tYWU1domA85pD2GbFGupFIzUkhT00wt0FbZTKoXqSgxdRwgrVLenTAwpRRiO01CipjDVyzDVFKZik_Tj39schc_pFeDHHlilrJdwBHTM1jj437lhSk7l8MIRNKOOCjz_AzCIv3I</recordid><startdate>20230501</startdate><enddate>20230501</enddate><creator>Kano, Yasuhiro</creator><creator>Sugihara, Makoto</creator><general>The Japanese Society of Internal Medicine</general><general>Japan Science and Technology Agency</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20230501</creationdate><title>Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels</title><author>Kano, Yasuhiro ; Sugihara, Makoto</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c610t-aa0de3bcbcb37514bbe1f48da0a9ddc4f49155cbdf4058041dc31c208325494c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Alkaline Phosphatase</topic><topic>Biopsy</topic><topic>Bone remodeling</topic><topic>Case Report</topic><topic>Case reports</topic><topic>diagnostic error</topic><topic>Fever</topic><topic>fever of unknown origin</topic><topic>Fever of Unknown Origin - diagnosis</topic><topic>Fever of Unknown Origin - etiology</topic><topic>Humans</topic><topic>Inflammatory diseases</topic><topic>Internal medicine</topic><topic>liver biopsy</topic><topic>Schnitzler syndrome</topic><topic>Schnitzler Syndrome - complications</topic><topic>Schnitzler Syndrome - diagnosis</topic><topic>Schnitzler Syndrome - drug therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kano, Yasuhiro</creatorcontrib><creatorcontrib>Sugihara, Makoto</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kano, Yasuhiro</au><au>Sugihara, Makoto</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. 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source	PubMed Central
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
title	Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels
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