Immunoglobulin G4-related retroperitoneal fibrosis (IgG4-RPF) - understanding a new clinical entity
Author(s):
Ms Archie Fernando
,
Ms Archie Fernando
Affiliations:
Dr Catherine Horsfield
,
Dr Catherine Horsfield
Affiliations:
Dr James Pattison
,
Dr James Pattison
Affiliations:
Mr Tim O'Brien
Mr Tim O'Brien
Affiliations:
BAUS ePoster online library. Fernando A. 06/26/17; 177348; P2-2
Ms. Archie Fernando
Ms. Archie Fernando
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Abstract
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INTRODUCTION
IgG4-related retroperitoneal fibrosis (IgG4-RPF) has emerged as a distinct subtype of RPF. Uncertainty surrounds its incidence; diagnosis; presentation; treatment; and clinical course compared to other RPF sub-types.

METHODS
Prospective study of 153 RPF patients managed in a multi-disciplinary RPF service since February 2012. Comparative analysis of IgG4-RPF and other RPF-subtypes. IgG4 diagnosis based on histological +/- serological confirmation.

RESULTS

153 patients

42/153(27%) IgG4-RPF

20/42(48%) had raised serum IgG4 levels at presentation.
14/20(70%) had only slight elevation in IgG4 above the upper limit of normal (1.11-2.0g/L); 6/20 (30%) had much higher levels (>2.0g/L) - all 6 patients had involvement of other organs with IgG4-related disease.

107 (70%) with non-IgG4-RPF – 44 (41%) idiopathic; 35 (33%) atheromatous; 23 (21%) aneurysmal; 1 (1%) paraneoplastic; 1 (1%) amyloid; 2 (2%) peri-aortitis; 1 (1%) methysergide

4 (3%) with IgG4-RPF diagnosed on clinical features alone (excluded)

SEE TABLE

CONCLUSIONS

Distinctive clinical features of IgG4-related RPF have been identified.

IgG4-RPF:
-Causes more severe ureteric obstruction leading to renal loss / nephrostomy dependency
-Is more likely to be steroid responsive than other types but often requires additional medical treatment
-Commonly affects multiple organ systems particularly if serum IgG4 levels >2.0g/L
-Has a much higher incidence of relapse (24% vs 2%) and so requires long term monitoring
Theses insights into IgG4-RPF have helped in counselling patients with this rare disease. The severity of renal obstruction and higher chance of relapse have led us to recommend early ureterolysis in patients with IgG4 related RPF.
INTRODUCTION
IgG4-related retroperitoneal fibrosis (IgG4-RPF) has emerged as a distinct subtype of RPF. Uncertainty surrounds its incidence; diagnosis; presentation; treatment; and clinical course compared to other RPF sub-types.

METHODS
Prospective study of 153 RPF patients managed in a multi-disciplinary RPF service since February 2012. Comparative analysis of IgG4-RPF and other RPF-subtypes. IgG4 diagnosis based on histological +/- serological confirmation.

RESULTS

153 patients

42/153(27%) IgG4-RPF

20/42(48%) had raised serum IgG4 levels at presentation.
14/20(70%) had only slight elevation in IgG4 above the upper limit of normal (1.11-2.0g/L); 6/20 (30%) had much higher levels (>2.0g/L) - all 6 patients had involvement of other organs with IgG4-related disease.

107 (70%) with non-IgG4-RPF – 44 (41%) idiopathic; 35 (33%) atheromatous; 23 (21%) aneurysmal; 1 (1%) paraneoplastic; 1 (1%) amyloid; 2 (2%) peri-aortitis; 1 (1%) methysergide

4 (3%) with IgG4-RPF diagnosed on clinical features alone (excluded)

SEE TABLE

CONCLUSIONS

Distinctive clinical features of IgG4-related RPF have been identified.

IgG4-RPF:
-Causes more severe ureteric obstruction leading to renal loss / nephrostomy dependency
-Is more likely to be steroid responsive than other types but often requires additional medical treatment
-Commonly affects multiple organ systems particularly if serum IgG4 levels >2.0g/L
-Has a much higher incidence of relapse (24% vs 2%) and so requires long term monitoring
Theses insights into IgG4-RPF have helped in counselling patients with this rare disease. The severity of renal obstruction and higher chance of relapse have led us to recommend early ureterolysis in patients with IgG4 related RPF.
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