Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients

Federica Maritati, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Maria Nicastro, Maria L. Urban, Paride Fenaroli, Francesco Peyronel, Giuseppe D. Benigno, Alessandro A. Palumbo, Domenico Corradi, Giacomo Emmi, Nicolò Pipitone, Alessandra Palmisano, Augusto Vaglio

Research output: Contribution to journalArticle

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 languageEnglish
Number of pages1
JournalFrontiers in Immunology
Volume10
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Retroperitoneal Fibrosis
Immunoglobulin G
Serum
Ureteral Diseases
Erdheim-Chester Disease
Ureteral Obstruction
Fluorodeoxyglucose F18
Abdominal Aortic Aneurysm
Vasculitis

Keywords

  • fibro-inflammatory disorder
  • hydronephrosis
  • IgG4
  • IgG4-related disease
  • periaortitis
  • retroperitoneal fibrosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Maritati, F., Rocco, R., Accorsi Buttini, E., Marvisi, C., Nicastro, M., Urban, M. L., ... Vaglio, A. (2019). Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients. Frontiers in Immunology, 10. https://doi.org/10.3389/fimmu.2019.00693

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 journalArticle

Maritati, F, Rocco, R, Accorsi Buttini, E, Marvisi, C, Nicastro, M, Urban, ML, Fenaroli, P, Peyronel, F, Benigno, GD, Palumbo, AA, Corradi, D, Emmi, G, Pipitone, N, Palmisano, A & Vaglio, A 2019, 'Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients', Frontiers in Immunology, vol. 10. https://doi.org/10.3389/fimmu.2019.00693
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. / Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients. In: Frontiers in Immunology. 2019 ; Vol. 10.
@article{393b7a84a0fb48c1bf60ac49b9727a89,
title = "Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients",
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.",
keywords = "fibro-inflammatory disorder, hydronephrosis, IgG4, IgG4-related disease, periaortitis, retroperitoneal fibrosis",
author = "Federica Maritati and Rossana Rocco and {Accorsi Buttini}, Eugenia and Chiara Marvisi and Maria Nicastro and Urban, {Maria L.} and Paride Fenaroli and Francesco Peyronel and Benigno, {Giuseppe D.} and Palumbo, {Alessandro A.} and Domenico Corradi and Giacomo Emmi and Nicol{\`o} Pipitone and Alessandra Palmisano and Augusto Vaglio",
year = "2019",
month = "1",
day = "1",
doi = "10.3389/fimmu.2019.00693",
language = "English",
volume = "10",
journal = "Frontiers in Immunology",
issn = "1664-3224",
publisher = "Frontiers Media S.A.",

}

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

VL - 10

JO - Frontiers in Immunology

JF - Frontiers in Immunology

SN - 1664-3224

ER -