Session Information
Date: Monday, November 9, 2015
Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster Session II
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Comparison of IgG4-related and Non-IgG4-related
Retroperitoneal fibrosis; a 12 year Retrospective Study
Background/Purpose: Retroperitoneal fibrosis (RPF) is a rare disease
characterized by a progressive proliferation of fibro-inflammatory tissue in
the periaortic region leading to abdominal pain, back pain, renal failure, etc.
Over 2/3rd cases of RPF are idiopathic. Prior studies, using various
criteria to define IgG4 RPF, have suggested an association of idiopathic RPF
(IRPF) with IgG4-related disease (IgG4RD) with a frequency between 28-58%,
while little is known about the clinical outcome of these patients.
We were interested to
determine the prevalence of IgG4-related RPF amongst IRPF and their clinical
characteristics and outcomes compared to non-IgG4-related RPF.
Methods: Retrospective chart review of all biopsy proven cases
of IRPF between 1/1/2003 to 4/3/2015 presenting at Cleveland Clinic. Data including
demographics, history, labs, imaging, treatment and outcomes were collected.
IgG4RD was defined as compatible clinical presentation along with
characteristic HP – dense lymphoplasmacytic (LP) infiltrate; fibrosis, usually storiform and obliterative phlebitis
(OP); with immunohistochemistry (IHC) showing ≥30 IgG4+ plasma cells/ HPF
and IgG4+/IgG >40%. Statistical analysis was done using t-test and Fischer’s
exact test.
Results: 72 cases were identified and their charts were
reviewed – 33 patients were found to have IRPF based on no identifiable
secondary cause. Of these 6 (18%) met the criteria for IgG4RD. There was no statistical
difference found in demographics, clinical features, labs, imaging, treatment modalities
and outcomes in the 2 groups (see table 1 and 2). 30% needed surgical and 56%
needed both surgical and medical management in non-IgG4-related RPF vs. 50% and
33.3%, respectively in the IgG4-related RPF. Two patients were on prednisone at
the time of biopsy-one was IgG4-related and other was non-IgG4-related RPF. IgG4RD
patients were statistically more likely to have OP, eosinophilia and lymphoid
follicles than non-IgG4-related RPF; though none of these histologic features
are sufficiently sensitive/specific for the diagnosis in the absence of
immunostains for IgG4 to establish the diagnosis.
Conclusion: This is the largest reported cohort of biopsy proven
IRPF cases presenting at one tertiary center. Only 18% cases of IRPF were
IgG4RD compared to 28-58% reported in other series. There were no difference
found in the treatment outcomes between IgG4-related RPF and non-IgG4-related
RPF. OP, arteritis, eosinophilia and lymphoid follicles are suggestive of
IgG4RD and should prompt additional testing
|
Table 1. |
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|
|
Overall (N=33) |
|
Non-IgG4-related RPF (N=27) |
|
IgG4-related RPF (N=6) |
|
|
|||
|
|
N |
Summary |
|
N |
Summary |
|
N |
Summary |
|
p-value |
|
DEMOGRAPHICS: |
|
|
|
|
|
|
|
|
|
|
|
Age (years) |
33 |
57.33±13.1 |
|
27 |
55.6±13.6 |
|
6 |
65.3±6.5 |
|
0.10 |
|
Race . White . Black or African American |
26/33 7/33 |
78.8% 21.2% |
|
20/27 7/27 |
74.1% 25.9% |
|
6/6 0/6 |
100% 0% |
|
0.16 |
|
Gender . Male . Female |
23/33 10/33 |
69.7% 30.3% |
|
18/27 9/27 |
66.7% 33.6% |
|
5/6 1/6 |
83.3% 16.7% |
|
0.69 |
|
Duration of follow-up (months) |
33 |
47.3±46 |
|
27 |
43.4±43.7 |
|
6 |
64.8±56 |
|
0.31 |
|
CLINICAL FEATURES: |
|
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|
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|
|
|
Abdominal pain |
19/28 |
67.86% |
|
15/22 |
68.2% |
|
4/6 |
66.7% |
|
0.94 |
|
Acute kidney injury |
16/29 |
55.17% |
|
13/24 |
54.17% |
|
3/5 |
60% |
|
1.0 |
|
Low back pain |
10/27 |
37.04% |
|
8/22 |
36.4% |
|
2/5 |
40% |
|
0.89 |
|
HISTORY: |
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|
|
|
Smoker . Current . Former . Never |
10/33 11/33 12/33 |
30.3% 33.3% 36.4% |
|
8/27 8/27 11/27 |
29.5% 29.5% 40.7% |
|
2/6 3/6 1/6 |
33.3% 50% 16.7% |
|
0.49 |
|
Other fibrosing conditions |
1/33 |
3% |
|
1/27 |
3.7% |
|
0/6 |
0% |
|
0.62 |
|
LABS: |
|
|
|
|
|
|
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|
|
|
|
Creatinine (mg/dL) |
31 |
3.3±4.6 |
|
25 |
3±4.5 |
|
6 |
4.6±5.4 |
|
0.46 |
|
CRP (mg/dL) |
10 |
4.8±5.1 |
|
9 |
5.3±5.1 |
|
1 |
0 |
|
0.35 |
|
WSR (mm/hr) |
11 |
79.3±47.7 |
|
10 |
73.3±45 |
|
1 |
140 |
|
0.19 |
|
IgG4 serum (mg/dL) |
9 |
48.2±53.8 |
|
7 |
57.5±58.3 |
|
2 |
15.5±2.1 |
|
0.36 |
|
IMAGING: |
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|
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Hydronephrosis |
25/32 |
78.12% |
|
20/26 |
76.9% |
|
5/6 |
83.3% |
|
0.73 |
|
Bilateral Hydronephrosis |
23/32 |
71.9% |
|
18/26 |
69.2% |
|
5/6 |
83.3% |
|
0.49 |
|
Maximal dimension (cm) |
22 |
6.4±4.9 |
|
17 |
6±5.1 |
|
5 |
7.5±4.1 |
|
0.57 |
|
Abdominal aortic aneurysm |
8/29 |
27.59% |
|
6/24 |
25% |
|
2/5 |
40% |
|
0.49 |
|
Location of mass . Periaortic and periureteral . Periaortic only . Periureteral only |
8/33 10/33 5/33 |
24.4% 30.3% 15.2% |
|
6/27 9/27 4/27 |
22.2% 33.3% 14.8% |
|
2/6 1/6 1/6 |
33.3% 16.7% 16.7% |
|
0.93 |
|
Values presented as Mean±SD or percentage p-values: t-test for continuous variables and Fisher’s Exact test for non-continuous variables (ordinal and nominal) |
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|
Table 2. |
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|
|
Overall (N=33) |
|
Non-IgG4-related RPF (N=27) |
|
IgG4-related RPF (N=6) |
|
|
|||
|
|
N |
Summary |
|
N |
Summary |
|
N |
Summary |
|
p-value |
|
Method of biopsy . CT guided . Open |
11/33 22/33 |
33.3% 66.7% |
|
8/27 19/27 |
29.6% 70.4% |
|
3/6 3/6 |
50% 50% |
|
0.34 |
|
HISTOPATHOLOGY: |
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Lymphoplasmacytic infiltrate (0 to 3) . 0 . 1 . 2 . 3 |
2/33 11/33 6/33 14/33 |
6.1% 33.3% 18.2% 42.4% |
|
2/27 10/27 5/27 10/27 |
7.4% 37% 18.5% 37% |
|
0/6 1/6 1/6 4/6 |
0% 16.7% 16.7% 66.7% |
|
0.56 |
|
Storiform fibrosis |
8/33 |
24.2% |
|
6/27 |
22.2% |
|
2/6 |
33.3% |
|
0.46 |
|
Fibrosis |
28/33 |
84.8% |
|
23/27 |
85.2% |
|
5/6 |
83.3% |
|
0.90 |
|
Obliterative phlebitis |
2/33 |
6.1% |
|
0/27 |
0% |
|
2/6 |
33.3% |
|
0.002 |
|
Eosinophilia |
9/33 |
27.3% |
|
5/27 |
18.5% |
|
4/6 |
66.7% |
|
0.02 |
|
Arteritis |
1/33 |
3% |
|
0/27 |
0% |
|
1/6 |
16.7% |
|
0.03 |
|
Lymphoid follicles |
6/33 |
18.2% |
|
3/27 |
11.1% |
|
3/6 |
50% |
|
0.03 |
|
TREATMENTS: |
|
|
|
|
|
|
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Treatment type . Medical and Surgical . None . Surgical . Unknown |
17/33 4/33 11/33 1/33 |
51.5% 12.2% 33.3% 3% |
|
15/27 4/27 8/27 0/27 |
55.6% 14.8% 29.6% 0% |
|
2/6 0/6 3/6 1/6 |
33.3% 0% 50% 16.7% |
|
0.09 |
|
Glucocorticoids |
15/32 |
46.9% |
|
13/27 |
48.1% |
|
2/5 |
40% |
|
0.74 |
|
Medical treatment for at least 6 months |
16/32 |
50.0% |
|
14/27 |
51.9% |
|
2/5 |
40% |
|
0.63 |
|
Treatment stents |
24/32 |
75% |
|
19/27 |
70.4% |
|
5/5 |
100% |
|
0.30 |
|
Treatment recurrent stents |
19/31 |
61.3% |
|
16/26 |
61.53% |
|
3/5 |
60% |
|
1.0 |
|
Treatment ureterolysis |
22/32 |
68.75% |
|
18/27 |
66.7% |
|
4/5 |
80% |
|
0.56 |
|
OUTCOMES: |
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Outcome creatinine change (mg/dL) |
30 |
-0.9±5.2 |
|
24 |
-1.2±5 |
|
6 |
0.4±6.3 |
|
1.0 |
|
Outcome WSR change (mm/hr) |
9 |
-66.7±44.7 |
|
8 |
-59.1±41 |
|
1 |
-128 |
|
0.16 |
|
Outcome imaging change . Improved . Resolution . Stable . Unknown . Worse |
10/33 3/33 9/33 6/33 5/33 |
30.3% 9.1% 27.3% 18.2% 15.2% |
|
8/27 2/27 7/27 5/27 5/27 |
29.6% 7.4% 25.9% 18.5% 18.5% |
|
2/6 1/6 2/6 1/6 0/6 |
33.5% 16.7% 33.3% 16.7% 0% |
|
0.79 |
|
Steroids on last follow-up Off steroids On steroids Unavailable data |
3/16 9/16 4/16 |
18.75% 56.25% 25% |
|
3/13 7/13 3/13 |
23.1% 53.8% 23.1% |
|
0/3 2/3 1/3 |
0% 66.7% 33.3% |
|
1.0 |
|
Stent/ nephrostomy on last follow-up |
9/24 |
37.5% |
|
8/19 |
42.1% |
|
1/5 |
20% |
|
0.36 |
|
Values presented as Mean±SD or percentage p-values: t-test for continuous variables and Fisher’s Exact test for non-continuous variables (ordinal and nominal) |
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To cite this abstract in AMA style:
Poddar P, Billings S, Calabrese LH, Gota CE. Comparison of IgG4-Related and Non-IgG4-Related Retroperitoneal Fibrosis; A 12 Year Retrospective Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/comparison-of-igg4-related-and-non-igg4-related-retroperitoneal-fibrosis-a-12-year-retrospective-study/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-igg4-related-and-non-igg4-related-retroperitoneal-fibrosis-a-12-year-retrospective-study/