Long-Term Outcome of Patients with Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Renal Involvement

Abstract

Background: Despite treatment, renal involvement in antineutrophil cytoplasmic autoantibody (ANCA)-positive vasculitis is still associated with significant long-term mortality and remains an important cause of end-stage renal failure. Methods: We retrospectively analyzed a series of 61 consecutive patients with newly diagnosed ANCA-associated renal vasculitis (54.1% Wegener’s granulomatosis, 23% renal-limited vasculitis, 16.4% microscopic polyangiitis, 4.9% Churg-Strauss syndrome) diagnosed between 1986 and 1997. Results: The median creatinine level at diagnosis was 221.5 (63–762) µmol/l, i.e. 2.5 (0.7–8.6) mg/dl, 32.8% were dialysis-dependent. All patients were treated with cyclophosphamide. Remission was achieved in 87% of patients. Relapses occurred in 44.7%. The median renal disease-free interval was 62.5 (0–138) months. The estimated patient survival at 5 and 10 years was 78.3 and 62.2%, respectively. Mortality was associated with age (p = 0.04 when age limit 50 years) and advanced renal failure (p = 0.038 when compared dialysis-dependent and independent patients). Estimated renal survival time at 5 and 10 years was 69.2 and 55.8%, respectively. At the end of follow-up, 50.8% of patients were in complete remission, 31% had died. The median serum creatinine level was 137.5 (77–469) µmol/l, i.e. 1.56 (0.87–5.3) mg/dl, 24.6% of patients were on regular dialysis treatment. Conclusion: Patient survival, relapse rate and mortality were comparable to similar reports. In view of the severity of the renal disease and the length of follow-up, renal survival was very good. Despite effective treatment, the long-term outcome of patients with ANCA-associated renal vasculitis remains unsatisfactory.

Outcome of Thirty Patients with ANCA-Associated Renal Vasculitis Admitted to the Intensive Care Unit

“…[16] In patients with AAV, renal insufficiency is also connected with poor prognosis. [17,18] Because our ICU forms an integral part of a nephrology unit, where most of our patients were recruited from, renal insufficiency was present in all our patients, without exception. Acute renal failure or insufficiency was registered in 13 patients, 11 of whom required renal replacement therapy.…”

Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis

“…Data on this matter remain, however, inconclusive. 92,[96][97][98] In addition to renal fibrosis, an association between lung fibrosis and MPO-ANCA has been observed. [99][100][101] Pulmonary fibrosis in PR3-AAV seems to occur less often.…”

“…96,[132][133][134][135] A systematic review demonstrated worse patient survival in patients with MPA compared with patients with GPA. 136 In our patients with AAV who had renal involvement, patient survival was worse in patients with MPO-AAV (n=92) compared with patients with PR3-AAV (n=89), whereas renal survival was similar (P50.28).…”

“…Various studies, however, could not find a clear difference in patient survival between PR3-AAV and MPO-AAV patients, 98,137 whereas one study reported a worse patient survival in patients with PR3-AAV. 138 Three studies found no difference in renal survival between ANCA serotypes, 92,96,139 whereas several recent studies reported a worse renal survival in patients with MPO-AAV. 98,[140][141][142] Survival in patients with PR3-AAV may be largely determined by diffuse alveolar hemorrhage at presentation and renal relapses in followup, 98,143 whereas renal and/or patient survival in MPO-AAV is probably related to smoldering disease 94 causing end-stage renal disease.…”

Renal Survival in Proteinase 3 and Myeloperoxidase ANCA-Associated Systemic Vasculitis

“…It is recognized that there are meaningful clinical differences between patients with PR3-AAV and MPO-AAV and it has been suggested that PR3-AAV and MPO-AAV are distinct diseases because PR3-ANCA-positive and MPO-ANCA-positive patients demonstrate a different disease spectrum (2,6,7). In general, patients with PR3-ANCA have more widespread extrarenal organ involvement and more active renal lesions at the time of diagnosis compared with MPO-ANCA-positive patients who have more chronic lesions (2,8,9). Another important difference is the higher relapse rate found in PR3-ANCA-positive AAV patients, which may be a major prognostic factor for renal survival during long-term follow-up (3).…”