Anti-neutrophil cytoplasmic autoantibody-associated vasculitis in the very elderly: a 90-year-old iron lady

Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides are characterized by blood vessel inflammation resulting in organ dysfunction or in the patient’s death. The peak of incidence was observed in patients over 75 years of age. We describe an unusual ANCA vasculitis presentation in a 90-year old patient admitted to the emergency room for severe dyspnea, oliguria and a recent onset cutaneous purple rush. Plain chest radiography disclosed pulmonary edema and diffuse bilateral reticulonodular infiltrates. Creatinine was 7.3 mg/dl, azotemia 202 mg/dl, hemoglobin 6.8 g/dl. CPAP (continuous positive airway pressure), furosemide 20 ml/h, nitroglycerin 8γ/kg/min, blood transfusions and i.v. methylprednisolone 60 mg/day were administered. On day 1 a femoral venous catheter was placed and hemodialysis (HD) treatment started for acute renal failure. The patient underwent 13 HD sessions without heparin (EVAL dialyzer). On day 7 renal function had still not recovered and a new plain chest radiogram was unchanged. Proteinase 3(PR3) ANCA were 81 IU/ml (ELISA), C-reactive protein (CRP) was 18 mg/dl, C3 42 mg/dl, C4 5 mg/dl. A high resolution computed tomography considering that the 53% of cases were managed with glucocorticoids alone. Nonetheless, Walsh et al’s metanalysis showed that patients who received a course of glucocorticoid therapy for more than 12 months suffered fewer relapses of ANCA vasculitis. In patients older than 80 years any immunosuppressive therapy is associated with a significantly lower risk of ESRD and death, but the rate of infections is higher than in younger patients. In our patient the CY dose related to the body surface area was reduced to 200 mg in view of age, risk of infection and renal failure, but glucocorticoid therapy was maintained. During the 2 year follow-up no infections were reported, the Birmingham vasculitis score was lower than 10 and reached the 50% reduction of the initial disease activity score recommended. A good outcome was obtained and it seems to be related both to CY and to the long-term maintenance therapy with glucocorticoids.