TY - JOUR
T1 - Perivascular fibrosis and igG4-related disease
T2 - A case report
AU - Monti, Sara
AU - Crepaldi, G.
AU - Peri, A.
AU - Pietrabissa, A.
AU - Morbini, P.
AU - Bobbio-Pallavicini, F.
AU - Montecucco, C.
AU - Caporali, R.
PY - 2014
Y1 - 2014
N2 - Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.
AB - Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.
KW - Aortic aneurysm
KW - Immunoglobulin G4-related disease
KW - Perivascular fibrosis
KW - Retroperitoneal fibrosis
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U2 - 10.4081/reumatismo.2014.753
DO - 10.4081/reumatismo.2014.753
M3 - Article
AN - SCOPUS:84908670403
VL - 66
SP - 245
EP - 248
JO - Reumatismo
JF - Reumatismo
SN - 0048-7449
IS - 3
ER -