Background/Purpose:  ANCA-associated vasculitis (AAV) usually occurs in elderly patients, but only a few studies regarding clinical features in elderly onset AAV have been reported. So far, there was no report describing clinical features of elderly onset AAV patients in Japan. The purpose of this study is to compare clinical features and outcome between elderly onset (≥75 years old) and younger Japanese AAV patients (<75 years old).

Methods:  AAV patients who met the criteria for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomaosis with polyangiitis (EGPA) were included in this study. AAV patients initially treated at Fukushima Medical University Hospital (Fukushima, Japan) from April 2004 to March 2016 were retrospectively reviewed. These patients were divided into 2 groups: elderly, age ≥ 75 (N = 10) and younger, age < 75 (N = 26). We compared the following clinical features between the 2 groups: age at diagnosis, sex, Birmingham Vasculitis Activity Score (BVAS), clinical symptoms, laboratory data, therapy and outcome.

Results: Thirty six AAV patients (9 GPA, 24 MPA and 3 EGPA) were included in this study. General clinical features showed similar between the two groups except for female rates and body weight in the elderly, compared to younger patients (80% vs. 42.3% and 43.6 vs. 58.9kg, respectively). In clinical symptoms, elderly AAV patients showed significantly higher rates of kidney involvement than younger AAV (90% vs. 50%, p = 0.03). Laboratory data showed significantly lower RBC and ferritin levels in the elderly than younger AAV (241 vs. 438 ng/mL, p = 0.021). As to therapy, significantly lower PSL doses were administered in elderly AAV patients (34 vs. 45.3 mg/day, p = 0.038). Overall survival analysis (Kaplan-Meier curve) showed significantly lower survival in elderly AAV (58.3% vs. 96.2%, p = 0.008). Five MPA patients died; the number of patients and causes of death were as follows: 4 in elderly AAV (alveolar hemorrhage, renal failure, bacterial pneumonia and pneumocystis pneumonia), and 1 in younger AAV (alveolar hemorrhage). We further analyzed correlation between serum ferritin levels and clinical items. Serum ferritin levels positively correlated with serum ALT (rs 0.47, p = 0.013) and C3 levels (rs 0.4, p = 0.045) but not with Hb, RBC, Cr, age at diagnosis, and BVAS scores.

Conclusion:  Elderly AAV patients showed similar clinical features except for increased rate of females, kidney manifestations and lighter body weight. Lower serum ferritin levels in the elderly may show milder inflammatory state than younger AAV at initial manifestation. Serum ferritin, an acute phase protein, are produced in inflammatory state (macrophage activation) and considered as a biomarker of disease activity in several autoimmune diseases. Serum ferritin levels in this study positively correlated with ALT and C3 levels, which can be considered as a possible biomarker of disease activity in AAV patients. The causes of death in elderly AAV can be explained with vasculitis progression itself (especially MPA patients), infection, and treatment using lower PSL doses. Proper management and monitoring of adverse events should be performed in patients with elderly AAV.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-and-outcome-in-patients-with-elderly-onset-anca-associated-vasculitis/