Table 1

Characteristics of the patients at the time of the first diagnosis

Case 1Case 2Case 3Case 4Case 5Case 6
Age (y)/sex47/F58/F32/M67/F26/M38/F
Clinical features and diagnosisMild renal failure INCGNMild renal failure INCGNModerate renal failure + PH INCGN + NACSevere renal failure INCGNSevere isolated PH NACModerate renal failure INCGN
ANCA (specificity/titre)PR3/1:1280PR3/1:1280MPO/1:5120PR3/1:2560MPO/1:1280MPO/1:640
Time for remission1 month3 weeks3 months2 weeks2 months1 month
Latent period1-150 36 months20 months72 months42 months48 months36 months
Recrudescence in latent periodNoneNoneNoneNoneNoneNone
Length of time on treatment36 months20 months24 months24 months18 months30 months
Last control1-151 3 weeks1 month3 months2 months2 months6 weeks
Treatment1-152 Low dose cyclophosphamideLow dose corticosteroids + cyclophosphamideNoneNoneNoneNone
  • 1-150 Until development of Wegener’s granulomatosis (WG);

  • 1-151 before development of WG;

  • 1-152 at the time of development of WG. INCGN: idiopathic necrotising and crescentic glomerulonephritis; PH: pulmonary haemorrhage; NAC: necrotising alveolar capillaritis; ANCA: antineutrophil cytoplasmic antibodies; PR3: proteinase 3; MPO: myeloperoxidase.