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Abstract Number: 826

Does Anti-Glomerular Basement (anti-GBM) Antibody Positivity Correlate with Relapse in Patients with Anti-GBM Disease?

Nicole Droz1, Alexis Katz2, John Sedor3 and Rula A Hajj-Ali1,4, 1Rheumatology, Cleveland Clinic Foundation, Cleveland, OH, 2Cleveland Clinic Foundation, Cleveland, OH, 3Nephrology, Cleveland Clinic Foundation, Cleveland, OH, 4Rheumatic and Immunologic Disease, Cleveland Clinic Foundation, Cleveland, OH

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: glomerulonephritis and vasculitis

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Session Information

Date: Sunday, October 21, 2018

Session Title: Vasculitis Poster I: Non-ANCA-Associated and Related Disorders

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Anti-GBM disease is characterized by rapidly progressive glomerular nephritis with or without pulmonary hemorrhage.  It is usually monophasic in nature and disease severity correlates with antibody titer.  The disease is mediated by pathogenic antibodies directed against the non-collagenous region of the α3 chain of type IV collagen.  

Despite the known pathogenicity of anti-GBM antibodies, and the correlation of disease severity with their titers, there is conflicting reports on whether anti-GBM antibody positivity correlates with disease relapse on long term follow up. The objective of this study was to assess for correlation of anti-GBM antibody positivity and disease relapse in patients with anti-GBM disease.

Methods: Patients seen in a single academic center between 1997 and 2017 were initially screened for the presence of anti-GBM disease by ICD 9/10 code for anti-GBM disease or Goodpasture’s syndrome.  435 patients were identified. Patients were then included in the study if the diagnosis was confirmed by a board certified rheumatologist or nephrologist at our institution and had positive anti-GBM antibodies or biopsy results consistent with a diagnosis of anti-GBM disease.  Relapsing disease was defined as recurrence of glomerulonephritis or pulmonary hemorrhage after the initial presentation that necessitated a change in therapy. The primary endpoint of this study was anti-GBM antibody positivity at the time of relapse.  All charts were reviewed for baseline demographics, clinical manifestations, anti-GBM antibody and ANCA positivity at the time of initial presentation; these were compared between those with relapsing and non-relapsing disease.  Results were analyzed using a two tailed standard t-test.  These same characteristics were also examined in the relapsing cohort at the time of relapse.

 

Results: 40 patients were confirmed as having anti-GBM disease at our institution.  Mean follow up from disease onset to the date of last follow up was 56.2 months.  8 patients had relapsing disease and 32 patients had non-relapsing disease.  Baseline characteristics and clinical manifestations were similar between groups (Table 1).  Patients with relapsing disease had a statistically higher incidence of ANCA co-positivity as compared to non-relapsing patients (62.5% vs. 21.7% respectively  p value- 0.03).

In patients with relapsing disease, only 14.7% (1/7 tested patients) had positive anti-GBM antibodies at the time of their relapse.

Conclusion: In this study, anti-GBM positivity did not correlate with disease relapse in patients with anti-GBM disease.  Patients with relapsing disease had a higher incidence of ANCA positivity, consistent with previous investigations.  In patients with newly diagnosed anti-GBM disease, ANCAs should be obtained to assess for the risk of relapse and to help guide long term follow up and treatment. Larger studies are needed to validate our results. 

 

 

 

 

Table 1: Baseline demographics, clinical features and laboratory values at baseline and at the time of relapse of anti-GBM disease

 

 

Non-relapsing anti-GBM disease (n=32)

Relapsing anti-GBM disease (n=8)

p-value

Baseline characteristics

Length of follow up, mos (range)

62.79 (2-228)

26.29 (3-78)

     0.16

 

Time to diagnosis, days (range)

 

30 (1-182)

62 (5-243)

0.33

 

Age, Yr (range)

46.06 (17-78)

41 (14-62)

     0.52

 

Male Gender (%)

 

53.1

37.5

0.44

 

Non-Smoking Status (%)

43.8

50.0

0.61

 

Renal involvement (%)

    

93.8

100

0.48

 

Peak creatinine mg/dL (range)

 

7.24 (0.8-17.6)

5.37 (1.6-13.7)

0.33

 

Pulmonary hemorrhage (%)

 

30.0

37.5

0.69

 

Positive Anti-GBM antibodies (%)

PPosi

73.9 (17/23)a

37.5 (3/8)

0.07

 

Positive ANCA (%)

 

21.7(5/23)a

62.5 (5/8)

0.03

Characteristics at time

 of relapse

 

 

 

 

 

Time to relapse, weeks (range)

 

77.14 (6-480)

 

 

Positive Anti-GBM antibodies (%)

Posit

 

14.3 (1/7)a

 

 

Positive ANCA (%)

 

75 (3/4)a

 

 

Renal involvement (%)

 

 

83.3

 

 

Peak creatinine mg/dL (range)

 

 

3.42 (2.7-4.85)

 

 

Pulmonary hemorrhage (%)

 

 

71.4

 

Values are expressed as mean (range) or percent

aIncomplete data available.  Number of positive patients out of total tested represented in parentheses

 

 



Disclosure: N. Droz, None; A. Katz, None; J. Sedor, None; R. A. Hajj-Ali, None.

To cite this abstract in AMA style:

Droz N, Katz A, Sedor J, Hajj-Ali RA. Does Anti-Glomerular Basement (anti-GBM) Antibody Positivity Correlate with Relapse in Patients with Anti-GBM Disease? [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/does-anti-glomerular-basement-anti-gbm-antibody-positivity-correlate-with-relapse-in-patients-with-anti-gbm-disease/. Accessed May 17, 2022.
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