ANCA-associated renal vasculitis at the end of the twentieth century--a disease of older patients

Rheumatology (Oxford). 2005 Apr;44(4):495-501. doi: 10.1093/rheumatology/keh522. Epub 2004 Dec 21.


Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are increasingly recognized in older patients. However, it is unknown whether disease presentation and response to treatment differs from younger patients. We aimed to examine the presentation, response to treatment and outcome of patients over 65 yr of age compared with a younger cohort.

Methods: This retrospective, single centre, sequential cohort study reports presenting features and outcome of 233 consecutive new patients with ANCA-associated vasculitis between 1990 and 2000.

Results: The median age of all patients was 65 yr (range 16-90 yr). Older patients (>65 yr) presented with more severe renal involvement at presentation (P < 0.001). Older patients were as likely to respond to treatment or undergo relapse as the younger patients. Older patients receiving immunosuppression had an increased risk of infection (P = 0.0027). Survival was worse in the older group (P = 0.016) and death occurred early. Mortality was associated with poor renal function (creatinine >400 micromol/l), infection and low serum albumin. Leucopenia was associated with severe renal impairment (P = 0.0048) and increased risk of infection (P = 0.0006). Multivariate analysis determined that serum creatinine >400 micromol/l and age were independent risk factors for poor prognosis.

Conclusion: ANCA-associated vasculitis occurs frequently in older patients and physicians should maintain a high index of suspicion. Older patients have a poorer prognosis due to more severe renal involvement and increased sensitivity to adverse effects of treatment. This study highlights the importance of careful dosing of cyclophosphamide: in those aged over 65 yr a 25% dose reduction is safe and reduces the risk of leucopenia. This study further highlights the importance of renal function on prognosis and the need for less toxic treatment regimens.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Aged, 80 and over
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / therapy
  • Creatinine / blood
  • Cyclophosphamide / adverse effects
  • Epidemiologic Methods
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Immunosuppressive Agents / adverse effects
  • Kidney / physiopathology
  • Kidney Diseases / diagnosis*
  • Kidney Diseases / drug therapy
  • Kidney Diseases / immunology
  • Kidney Diseases / therapy
  • Kidney Failure, Chronic / etiology
  • Male
  • Middle Aged
  • Opportunistic Infections / etiology
  • Prognosis
  • Vasculitis / diagnosis*
  • Vasculitis / drug therapy
  • Vasculitis / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Creatinine