Abstract
IgG4-related disease (IgG4-RD) is a recently recognized syndrome characterized by frequent elevation of serum Ig4 levels and typical pathology of the affected organs. Biopsy shows lymphoplasmacytic inflammation, frequent eosinophils, storiform fibrosis, obliterative phlebitis and abundant IgG4-positive staining plasma cells. Disease manifestations often respond dramatically to steroid therapy.
Original language | English (US) |
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Title of host publication | Challenging Cases in Rheumatology and Diseases of the Immune System |
Publisher | Springer New York |
Pages | 73-88 |
Number of pages | 16 |
ISBN (Electronic) | 9781461450887 |
ISBN (Print) | 146145087X, 9781461450870 |
DOIs | |
State | Published - Mar 1 2014 |
Keywords
- Aortitis
- Autoimmune pancreatitis
- IgG4
- Orbital pseudolymphoma
ASJC Scopus subject areas
- Medicine(all)