Clinical features of the 19 patients with IPF and MPA
| Case | Sex | Age at the time of MPA development | Time from IPF diagnosis to MPA development (years) | MPO-ANCA at the time of IPF diagnosis | MPO-ANCA at the time of MPA development | Diffuse alveolar haemorrhage | Acute respiratory failure | Rapidly progressive glomerulonephritis | Mononeuritis multiplex | Gastrointestinal bleeding | Purpuric rash | Fever | Duration after MPA development (years) | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 67 | 0 | Positive | Positive | + | − | − | + | − | + | + | 9.04 | Dead |
| 2 | M | 67 | 0 | Positive | Positive | + | − | − | + | − | − | − | 2.41 | Dead |
| 3 | F | 72 | 0 | Positive | Positive | + | − | + | − | − | − | + | 0.27 | Alive |
| 4 | F | 66 | 0 | Positive | Positive | + | − | − | + | − | + | − | 0.6 | Dead |
| 5 | F | 73 | 0 | Positive | Positive | + | − | + | − | − | − | − | 0.16 | Alive |
| 6 | M | 62 | 0 | Positive | Positive | + | − | + | − | − | − | − | 9.99 | Alive |
| 7 | M | 69 | 0 | Positive | Positive | + | + | + | − | − | − | + | 3.69 | Alive |
| 8 | F | 79 | 0 | Positive | Positive | + | − | + | + | − | − | − | 1.53 | Alive |
| 9 | M | 69 | 0 | Positive | Positive | + | + | + | + | − | − | − | 2.47 | Alive |
| 10 | F | 79 | 0 | Positive | Positive | + | + | + | − | − | − | + | 0.08 | Dead |
| 11 | F | 74 | 0.28 | Positive | Positive | − | + | − | − | − | + | + | 1.32 | Dead |
| 12 | M | 75 | 6.06 | Negative | Positive | + | − | + | − | − | − | + | 0.06 | Dead |
| 13 | M | 76 | 5.5 | Negative | Positive | + | − | + | − | − | − | + | 0.08 | Dead |
| 14 | M | 76 | 5.12 | Negative | Positive | + | − | + | − | − | − | + | 1.04 | Dead |
| 15 | F | 73 | 8.25 | Negative | Positive | − | + | − | + | − | − | + | 1.87 | Dead |
| 16 | M | 69 | 0.5 | Positive | Positive | − | − | + | − | − | − | + | 9.76 | Alive |
| 17 | F | 69 | 6.93 | Positive | Positive | − | − | + | − | − | − | − | 0.08 | Alive |
| 18 | F | 60 | 0.42 | Negative | Positive | − | − | + | − | − | − | + | 6.31 | Alive |
| 19 | M | 62 | 5.33 | Negative | Positive | − | + | + | − | + | − | + | 0.08 | Alive |
ANCA, antineutrophil cytoplasmic antibody; IPF, idiopathic pulmonary fibrosis; MPA, microscopic polyangiitis.