| Literature DB >> 24562718 |
Christian Löffler1, Johannes Hoffend2, Martin Rebel3, Rüdiger Waldherr4, Michael Uppenkamp5, Raoul Bergner5.
Abstract
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.Entities:
Keywords: Aortitis; Azathioprine; IgG4; PET/CT; Steroids; Therapy
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Year: 2014 PMID: 24562718 DOI: 10.1007/s10067-014-2535-0
Source DB: PubMed Journal: Clin Rheumatol ISSN: 0770-3198 Impact factor: 2.980