Background/Purpose: Incident cases of pauci-immune glomerulonephritis affect adults at the older end of the age spectrum though relevant published data are limited. We sought to determine whether the clinical expression of disease differed in adults older than 70 years of age. 

Methods: Between January 1, 1995 and June 22, 2012, a total of 55 patients (pts) ≥ age 60 years with histologic evidence of pauci-immune glomerulonephritis were evaluated at a single university center. The association of demographic and clinical parameters with age category was assessed using student’s tand chi-square tests for continuous and categorical variables, respectively. The association of age category (vasculitis onset age 60-69 vs ≥70 yrs) with several outcome measures was examined using logistic regression in univariate analyses. 

Results: 33 pts were age 60-69 yrs at presentation; 22 were ≥70 yrs. This cohort was 87% Caucasian, 44% female, and 89% ANCA-positive; the proportion pANCA and cANCA positive did not differ by age group. There were no differences in mean BVAS/WG scores at diagnosis between the 2 groups (8.2 ± 4.4 vs 9.5 ± 3.4, p=0.25). Peak serum creatinine at the time of renal biopsy was 4.6 mg/dl ± 2.4 among the older vs 3.4 ± 2.3 (p=0.085) among the younger age group with a mean GFR 15.9 ± 8.4 vs 26.8 ± 21.6 (p=0.035), respectively. Combination therapy with steroids and cyclophosphamide was the most frequently employed first vasculitis treatment regardless of age group (n=15; 68% older vs. n=26; 79% younger; p=0.53). There were 4 total deaths in the cohort, 2 in each age group. Ultimately, 5 renal transplants were performed among the younger vs. none in the older patient group (p=0.056). Further associations of key clinical features of disease differed between the two age groups, as follows: 

Outcome                                                                                       Univariate Odds Ratio (95%CI) (older vs younger)     

Hospitalization at Presentation                                                         5.0 (1.0, 25.4)                

Hemodialysis-dependent at diagnosis                                             3.1 (1.0, 9.9)

Severe infection (hospitalization) during initial therapy                    5.7  (1.3 – 24.9)

Leukopenia during initial therapy                                                      1.1 (0.4 – 3.5)

Conclusion: There is relatively little information on the clinical features and outcomes regarding pauci-immune glomerulonephritis in the elderly. This single center experience, limited by retrospective design and small sample size, suggests that patients ≥70 years of age have worse renal outcomes associated with a higher serum creatinine and lower GFR at time of diagnosis, and an increased risk of progression to hemodialysis (borderline statistically significant) despite similar BVAS/WG scores. Elderly patients may be diagnosed later in their disease course, which may reflect a delay in initiating effective therapy. Our experience also suggests that elderly patients are more likely to experience treatment complications such as severe infection; although this did not correlate with an increased rate of leukopenia. These data imply that older pates may require a different treatment paradigm with more aggressive surveillance for both incipient renal disease and treatment complications.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pauci-immune-glomerulonephritis-in-the-elderly-disease-severity-and-outcomes/