Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | |
---|---|---|---|---|---|---|
Age (y)/sex | 47/F | 58/F | 32/M | 67/F | 26/M | 38/F |
Clinical features and diagnosis | Mild renal failure INCGN | Mild renal failure INCGN | Moderate renal failure + PH INCGN + NAC | Severe renal failure INCGN | Severe isolated PH NAC | Moderate renal failure INCGN |
ANCA (specificity/titre) | PR3/1:1280 | PR3/1:1280 | MPO/1:5120 | PR3/1:2560 | MPO/1:1280 | MPO/1:640 |
Time for remission | 1 month | 3 weeks | 3 months | 2 weeks | 2 months | 1 month |
Latent period1-150 | 36 months | 20 months | 72 months | 42 months | 48 months | 36 months |
Recrudescence in latent period | None | None | None | None | None | None |
Length of time on treatment | 36 months | 20 months | 24 months | 24 months | 18 months | 30 months |
Last control1-151 | 3 weeks | 1 month | 3 months | 2 months | 2 months | 6 weeks |
Treatment1-152 | Low dose cyclophosphamide | Low dose corticosteroids + cyclophosphamide | None | None | None | None |
↵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.