Sex | Age(years) | ANCA-V | Status | Chronology PF-ANCA-V | Follow-up (months) | Clinical | Type of PF | TLC/TLCO at diagnosis(%) | IAH | MPO-ANCAs | Eosinophils(×109/l) | PF aggra-vation | Relapse of ANCA-V | |
1 | F | 78 | MPA | Dead: CMV pneumonia | Same time | 7 | L K | NSIP | – | 0 | 1 | 13 | – | – |
2 | M | 71 | MPA | Dead: vasculitis | PF preceded ANCA-V | 7 | L K | ND | – | 0 | 1 | ND | – | – |
3 | M | 77 | MPA | Dead: exacerbation of ESRF | Same time | 60 | L N | ND | 81/46 | 0 | 1 | 1.1 | Yes | Yes |
4 | M | 71 | MPA | Dead: exacerbation of ESRF | Same time | 37 | L N S M | UIP | 71/72 | 0 | 1 | 0.8 | Yes | No |
5 | M | 69 | MPA | Dead: exacerbation of ESRF | Same time | 67 | L K N S | UIP | 74/46 | 0 | 1 | 1.2 | Yes | Yes |
6 | F | 64 | MPA | Alive | Same time | 52 | L N | UIP | 101/87 | 0 | 1 | 0.6 | Yes | No |
7 | M | 69 | MPA | Alive | Same time | 92 | L K M | UIP | 78/62 | 0 | 1 | 4.4 | Yes | No |
8 | M | 70 | MPA | Alive | PF preceded ANCA-V | 18 | L K N M | UIP | – | 0 | 1 | 0.5 | No | Yes |
9 | M | 64 | MPA | Alive | PF after ANCA-V | 109 | E L K S O | ND | – | 0 | 1 | 0.2 | No | Yes |
10 | F | 75 | WG | Alive | Same time | 54 | L | ND | – | 0 | 1 | ND | No | No |
11 | M | 67 | MPA | Alive | PF preceded ANCA-V | 116 | L K M N | UIP | 45/51 | 0 | 1 | <0.1 | No | No |
12 | M | 73 | WG | Alive | Same time | 63 | E L K H | ND | – | 0 | 1 | 0.2 | No | No |
ANCA-V, systemic vasculitis related to antineutrophil cytoplasmic antibodies; CMV, cytomegalovirus; E, ear–nose–throat; ESRF, end-stage respiratory failure; IAH, intra-alveolar haemorrhage; K, kidney; L, lung; M, muscles; MPA, microscopic polyangiitis; MPO-ANCAs, anti-myeloperoxidase-ANCAs; N, neuropathy; ND, not determined; NSIP, non-specific interstitial pneumonia; O, ophthalmology; PF, pulmonary fibrosis; S, skin; TLC, total lung capacity at diagnosis (%); TLCO, carbon monoxide transfer factor; UIP, usual interstitial pneumonia; WG, Wegener’s granulomatosis.