A rare cause for lower back pain: a case of an IgG4-related periaortitis

IgG4-related disease (IgG4-RD) are a group of autoinflammatory diseases often presenting as tumor-like lesions because of their infiltrative or mass forming behavior. They are characterized by a typical histology consisting of storiform fibrosis, high numbers of infiltrating IgG4-positive plasma cel...

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Hauptverfasser: Löffler, Christian (VerfasserIn) , Hoffend, Johannes (VerfasserIn) , Rebel, Martin (VerfasserIn) , Waldherr, Rüdiger (VerfasserIn) , Bergner, Raoul (VerfasserIn)
Weitere Verfasser: Uppenkamp, Michael (BerichterstatterIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 2016
In: Clinical rheumatology
Year: 2016, Jahrgang: 35, Heft: 1, Pages: 265-270
ISSN:1434-9949
DOI:10.1007/s10067-014-2535-0
Online-Zugang:Verlag, Volltext: https://doi.org/10.1007/s10067-014-2535-0
Volltext
Verfasserangaben:Christian Löffler, Johannes Hoffend, Martin Rebel, Rüdiger Waldherr, Michael Uppenkamp, Raoul Bergner

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520 |a IgG4-related disease (IgG4-RD) are a group of autoinflammatory diseases often presenting as tumor-like lesions because of their infiltrative or mass forming behavior. They are characterized by a typical histology consisting of storiform fibrosis, high numbers of infiltrating IgG4-positive plasma cells, obliterative phlebitis, and a moderate presence of eosinophilic cells. Serum IgG4 levels can be elevated. We present a case of a 57 year-old male patient with immobilizing lower back pain, fever, and night sweats. We diagnosed IgG4-related periaortitis using serum IgG4 levels, abdominal ultrasound, PET/CT, and histology. We successfully treated the patient with glucocorticoids (GC) and azathioprine. Periaortitis is a rare presentation of IgG4-RD and therefore noteworthy. It has to be considered in patients with a retroperitoneal mass. 
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