Clinical and radiological characteristics based on pleuroparenchymal fibroelastosis (PPFE) presence
| Characteristic | Patients with PPFE (n=59) | Patients without PPFE (n=165) | p-value |
| Age years | 70.3±11.6 | 65.8±11.8 | 0.013 |
| Male/female n | 10/49 | 40/125 | 0.279 |
| BMI kg·m−2 | 17.9±2.3 | 19.8±2.6 | <0.001 |
| Smoker | 13 (22.0) | 51 (30.9) | 0.241 |
| Smoking pack-years | 3.8±9.4 | 10.2±22.2 | 0.032 |
| Previous history of pulmonary TB | 10 (16.9) | 18 (10.9) | 0.254 |
| Presence of comorbidities | 12 (20.3) | 44 (26.7) | 0.384 |
| Use of immunosuppressants | 2 (3.4) | 6 (3.6) | >0.999 |
| Symptoms | 46 (78.0) | 124 (75.2) | 0.726 |
| Cough | 40 (67.8) | 101 (61.2) | 0.433 |
| Sputum | 31 (52.5) | 77 (46.7) | 0.452 |
| Dyspnoea | 14 (23.7) | 24 (14.5) | 0.111 |
| Haemoptysis | 18 (30.5) | 31 (18.8) | 0.069 |
| Fever | 9 (15.3) | 14 (8.5) | 0.209 |
| Fatigue | 8 (13.6) | 7 (4.2) | 0.028 |
| Weight loss | 20 (33.9) | 27 (16.4) | 0.008 |
| Positive sputum AFB smear | 20 (33.9) | 54 (32.7) | 0.873 |
| Causative organisms n | |||
| Mycobacterium avium | 35 (59.3) | 104 (63.0) | 0.641 |
| Mycobacterium intracellulare | 26 (44.1) | 75 (45.5) | 0.880 |
| Co-infections# | 2 (3.4) | 14 (8.5) | 0.249 |
| Radiological types | |||
| Nodular bronchiectatic | 43 (72.9) | 130 (78.8) | 0.369 |
| Fibrocavitary | 16 (27.1) | 32 (19.4) | 0.267 |
| Unclassifiable | 0 (0.0) | 3 (1.8) | 0.568 |
| MAC-PD score | 10.8±4.6 | 8.4±4.4 | <0.001 |
| Bronchiectasis | 3.9±1.8 | 2.7±1.8 | <0.001 |
| Cellular bronchiolitis | 3.7±1.4 | 3.3±1.7 | 0.084 |
| Cavity | 1.6±2.6 | 1.2±2.3 | 0.270 |
| Nodule | 0.6±0.6 | 0.5±0.6 | 0.609 |
| Consolidation | 1.0±0.7 | 0.6±0.7 | 0.002 |
| MAC-PD status | |||
| Progressive disease¶ | 31 (52.5) | 72 (43.6) | 0.287 |
| Stationary disease+ | 28 (47.5) | 93 (56.4) | |
| All-cause mortality within 5 years | 8 (13.6) | 1 (0.6) | <0.001 |
Data are presented as mean±sd or n (%), unless otherwise stated. BMI: body mass index; TB: tuberculosis; AFB: acid-fast bacilli; MAC-PD: M. avium complex pulmonary disease. #: co-infections of M. avium and M. intracellulare; ¶: a case requiring multidrug treatment within 3 years of diagnosis due to clinical or radiological deterioration; +: a case stable for ≥3 years after diagnosis without clinical or radiological deterioration.