Table 1.

Demographic and clinical characteristics of patients with AAV with active severe renal disease

CharacteristicActive Severe Renal Disease in AAV n=251
Age at diagnosis of severe renal involvement, median (IQR) yra66 (55–74)
Male, n (%)128 (51.0)
Disease presentation, n (%)
 AAV new diagnosis194 (77.3)
 AAV relapse57 (22.7)
AAV, n (%)
 MPA140 (55.8)
 GPA111 (44.2)
ANCA specificity (ELISA), n (%)
 MPO156 (62.2)
 PR395 (37.8)
BVAS/WG at renal involvement diagnosis, median (IQR)8 (7–10)
 Renal limited disease, n (%)84 (33.5)
 Alveolar hemorrhage, n (%)40 (15.9)
 Pulmonary-renal syndrome, n (%)33 (13.1)
Cardiovascular risk factors, n (%)
 Arterial hypertension187 (74.5)
 Diabetes mellitus56 (22.3)
 Dyslipidemia91 (36.3)
 BMI>30 kg/m287 (34.7)
Laboratory findings
 Hemoglobin, mean (SD) g/dl9.7 (8.7–11.0)
 ESR>22 mm/h, n (%)145 (57.8)
 SCr at diagnosis, median (IQR) mg/dl3.1 (2.5–4.2)
 eGFR at diagnosis, mean (SD) ml/min per 1.73 m216.3 (10.2–21.9)
Biopsy proven, n (%)222 (88.4)
Intervention
 Remission-induction treatment, n (%)
  CYC161 (64.1)
  RTX64 (25.5)
  Mycophenolate mofetil14 (5.6)
  Prednisone11 (4.8)
  Methotrexate1 (0.4)
 Corticosteroids, n (%)
  iv methylprednisolone followed by oral prednisone157 (62.5)
  Oral prednisone only94 (37.5)
 PLEX therapy, n (%)51 (20.3)
  PLEX number, median (IQR) sessions7 (5–9)
  Indications for PLEX, n (%)
   Renal48 (94.1)
   Renal and pulmonary19 (37.3)
  SCr at the time of PLEX, median (IQR) mg/dl5.1 (3.2–6.3)
  eGFR at the time of PLEX, mean (SD), ml/min per 1.73 m213.4 (1.14)
  eGFR<15 ml/min per 1.73 m2 at the time of PLEX, n (%)31 (14.7)
 Maintenance treatment, n (%)
  Azathioprine78 (31.1)
  Mycophenolate mofetil60 (23.9)
  RTX28 (11.2)
  CYC16 (6.4)
  Prednisone19 (7.6)
  Methotrexate5 (2.0)
  Impossible to determine the start of maintenance therapy45 (17.9)
  • EGFR is estimated using the Chronic Kidney Disease Epidemiology collaboration method. IQR, interquartile range; GPA, granulomatosis with polyangiitis; ESR, erythrocyte sedimentation rate.

  • a Treatment was started on average within 16 h of the diagnosis of severe renal involvement.