Abstract
Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.
| Original language | English |
|---|---|
| Number of pages | 1 |
| Journal | Frontiers in Immunology |
| Volume | 10 |
| DOIs | |
| Publication status | Published - Jan 1 2019 |
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Keywords
- fibro-inflammatory disorder
- hydronephrosis
- IgG4
- IgG4-related disease
- periaortitis
- retroperitoneal fibrosis
ASJC Scopus subject areas
- Immunology and Allergy
- Immunology
Cite this
Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients. / Maritati, Federica; Rocco, Rossana; Accorsi Buttini, Eugenia; Marvisi, Chiara; Nicastro, Maria; Urban, Maria L.; Fenaroli, Paride; Peyronel, Francesco; Benigno, Giuseppe D.; Palumbo, Alessandro A.; Corradi, Domenico; Emmi, Giacomo; Pipitone, Nicolò; Palmisano, Alessandra; Vaglio, Augusto.
In: Frontiers in Immunology, Vol. 10, 01.01.2019.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients
AU - Maritati, Federica
AU - Rocco, Rossana
AU - Accorsi Buttini, Eugenia
AU - Marvisi, Chiara
AU - Nicastro, Maria
AU - Urban, Maria L.
AU - Fenaroli, Paride
AU - Peyronel, Francesco
AU - Benigno, Giuseppe D.
AU - Palumbo, Alessandro A.
AU - Corradi, Domenico
AU - Emmi, Giacomo
AU - Pipitone, Nicolò
AU - Palmisano, Alessandra
AU - Vaglio, Augusto
PY - 2019/1/1
Y1 - 2019/1/1
N2 - Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.
AB - Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.
KW - fibro-inflammatory disorder
KW - hydronephrosis
KW - IgG4
KW - IgG4-related disease
KW - periaortitis
KW - retroperitoneal fibrosis
UR - http://www.scopus.com/inward/record.url?scp=85065298868&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85065298868&partnerID=8YFLogxK
U2 - 10.3389/fimmu.2019.00693
DO - 10.3389/fimmu.2019.00693
M3 - Article
C2 - 31019511
AN - SCOPUS:85065298868
VL - 10
JO - Frontiers in Immunology
JF - Frontiers in Immunology
SN - 1664-3224
ER -