Background ANCA associated vasculitis (AAV) usually occurs in elderly patients, but only a few studies regarding clinical features and treatment of elderly onset AAV have been reported so far1,2).
Objectives To compare clinical features and treatment of AAV between elderly patients (≥75) and younger patients (<75) in Japanese patients.
Methods Patients who met the criteria for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS) were included in this study. AAV patients (N=31) who were initially treated at Fukushima Medical University Hospital (Fukushima, Japan) from 2004 to 2015 were retrospectively reviewed. These patients were divided into 2 groups (elderly, N=9 and younger, N=22) and compared for the following clinical features, laboratory data and treatment: age at diagnosis, sex, Birmingham Vasculitis Activity Score (BVAS), symptoms, liver and renal function, hemoglobin, serum ferritin levels, IgG, ANCA (MPO/PR3) positivity, and therapy (prednisolone and immunosuppressive therapy).
Results Following 31 AAV patients were identified: 8 cases of GPA, 20 cases of MPA, and 3 cases of CSS. Elderly group showed significantly lower frequency of skin manifestation and significantly higher frequency of kidney symptoms. In laboratory data, elderly group showed significantly lower serum AST, LDH and Ferritin levels. As to therapy, elderly patients received significantly lower prednisolone doses and frequency of cyclophosphamide (CYP) administration. Mortality rates (1 year) were significantly higher in elderly group (33.3%) than younger group (4.6%). Four patients died: all patients were MPA and the main causes of death were vasculitis progression (alveolar hemorrhage) and infection.
Conclusions This study shows that the clinical features of elderly AAV patients are comparable to those of younger patients in Japan, except for lower skin manifestations and higher kidney symptoms. Lower AST, LDH, and ferritin levels in elderly AAV may indicate that elderly patients show milder organ damage on admission. Higher mortality rates in elderly AAV may be associated with lower PSL doses and frequency of CYP administration. Proper immunosuppressive therapy should be considered to reduce mortality rates for vasculitis progression or severe infection in elderly AAV.
Hoganson et al. J Clin Rheumatol. 2008.
Weiner et al. Clin J Am Soc Nephrol. 2015.
Disclosure of Interest None declared