Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 245-248 |
| Number of pages | 4 |
| Journal | Reumatismo |
| Volume | 66 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - 2014 |
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Keywords
- Aortic aneurysm
- Immunoglobulin G4-related disease
- Perivascular fibrosis
- Retroperitoneal fibrosis
ASJC Scopus subject areas
- Rheumatology
Cite this
Perivascular fibrosis and igG4-related disease : A case report. / Monti, Sara; Crepaldi, G.; Peri, A.; Pietrabissa, A.; Morbini, P.; Bobbio-Pallavicini, F.; Montecucco, C.; Caporali, R.
In: Reumatismo, Vol. 66, No. 3, 2014, p. 245-248.Research output: Contribution to journal › Article
}
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
UR - http://www.scopus.com/inward/record.url?scp=84908670403&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84908670403&partnerID=8YFLogxK
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 -