Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose:
A retrospective observational study of patients with IgG4 related disease and retroperitoneal fibrosis
Methods:
Retroperitoneal fibrosis (RPF) is a rare chronic inflammatory condition which may be associated with IgG4 disease. Thirty nine patients diagnosed with idiopathic RPF were retrospectively analysed at Guy’s and St Thomas’ Hospitals, London, England. All patients’ data regarding clinical presentation, markers of inflammation, immunoglobulin subsets, autoantibody profiles, imaging and histopathology was collected. Comprehensive Diagnostic Criteria (CDC) for the diagnosis of IgG4 disease was applied to all patients.
Results:
The study included 24 Caucasian, 9 African and 6 Asian patients. There were 19 female and 20 male patients. The median age of the patients was 54 years (range 38 – 80). The majority presented with ureteric obstruction (n=18) and 12 of these patients had renal failure. Clinical features included periaortitis (n=8), periaortic masses (n=6), left kidney atrophy (n=1), atheroma (n=1), lung cavitations (n=1), polymyalgia rheumatica (n=1), 1 patient had RPF diagnosed after a positive PET scan, 1 patient had RPF and a malignancy, and 1 patient developed RPF after methysurgide.
Laboratory markers of inflammation were significantly elevated in IgG4 related RPF. The median ESR was 28 (5 – 91) mm per hour and CRP was 26 (6 – 200) mg/l. The mean ESR=56mm/hr (S.D 28) in IgG4RPF was higher versus non-IgG4 RPF mean ESR=29mm/hr (S.D 27) [p=0.008]. IgG4 RPF, mean CRP=51mg/l (S.D 39) versus non-IgG4 RPF, mean CRP=31 mg/l (S.D 29) [p=0.04]. Immunoglobulin IgG subclass analysis showed higher IgG4 levels in IgG4 RPF, mean IgG4=0.96g/l (S.D 0.8) compared to non-IgG4 RPF, mean IgG4=0.44g/l (S.D 0.3) [p=0.05]. There were no statistically significant differences between IgG3, IgG2 and IgG1 levels in the IgG4 RPF and non-IgG4 RPF patients. Seven patients had elevated IgG4 levels. Thirty seven patients had biopsies, 7 had confirmed diagnosis and 4 had a probable diagnosis of IgG4 RPF based on IgG4 plasma cells and fibrosis in biopsy specimens. None of the patients diagnosed with IgG4 RPF had positive serology for ANA, dsDNA, ENA or ANCA. Seventeen patients had FDG-PET-CT scans of which 12 showed active inflammation, 5 with IgG4 RPF had positive scans. Therapeutic interventions included corticosteroids (n=28), immunosuppressants: azathioprine, methotrexate, ureteric stents (n=12), ureterolysis (n=4), and nephrectomy (n=2). Median corticosteroid dose was 20 mgs (0 – 40) /day. The median ESR 11 (3-63) mm/hour and CRP was 6 (5–70) mg/l improved after treatment. The median IgG4 levels dropped from 7.44 to 0.37 gm/l. Four patients had post therapy FDG-PET-CT scans and 3 (1 with IgG4 RPF) had reduced activity. Clinical improvement was reported in 72% of the patients (n=28), 1 patient deteriorated but declined Rituximab. Fifteen patients had a confirmed diagnosis of IgG4 disease based on CDC criteria.
Conclusion:
IgG4 disease may be considered in patients with retroperitoneal fibrosis and periaortic lesions. FDG-CT-PET imaging is useful diagnostically and response to corticosteroids and immunosuppressant therapy is favourable.
Disclosure:
S. Sangle,
None;
P. Lutalo,
None;
L. Nel,
None;
J. Pattison,
None;
T. OBrien,
None;
D. P. D’Cruz,
Investigator,
5.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/retroperitoneal-fibrosis-and-igg4-disease-response-to-immunosuppressive-therapy-a-single-centre-retrospective-study/