Patient demographics and clinical characteristics
| Study (first author) | Liebow | Gaensler | Tubbs | Valdivia | Carrington | Yousem | Hartman | Akira | Travis | Ryu | Craig | Baloira | Kawabata | Individual cases | Total |
| Country of study | USA | USA | USA | USA | USA | USA | Canada | Japan | USA | USA | UK | Spain | Japan | Various | – |
| Year published | 1965 | 1966 | 1977 | 1977 | 1978 | 1989 | 1993 | 1997 | 2000 | 2004 | 2004 | 2007 | 2012 | 1966–2019 | – |
| Patients | 18 | 12 | 26 | 30 | 40 | 36 | 22 | 8 | 16 | 23 | 20 | 12 | 31 | 68 | 362 |
| Male gender | 10 (56) | 7 (58) | 17 (65) | – | 28 (70) | 26 (72) | 6 (22) | 7 (88) | 7 (44) | 11 (48) | 12 (60) | 12 (100) | 29 (96) | 52 (76) | 224 (62) |
| Age years | 42 (16–61) | 39 (17–65) | 52 (24–75) | – | 42±12 | 42 (17–67) | 43 (22–71) | 56±8 | 42 (27–79) | 46±10 | 43 (27–74) | 50±16 | 55±13 | 45±14 | – |
| Non-productive cough | 14 (78) | – | 17 (65) | – | – | – | – | – | – | 10 (43) | – | 9 (75) | – | 46 (68) | 96/147 (65) |
| Exertional dyspnoea | 18 (100) | – | 23 (88) | – | – | – | – | – | – | 20 (87) | – | 7 (58) | – | 58 (85) | 127/147 (86) |
| Chest pain | 9 (50) | – | 1 (4) | – | – | – | – | – | – | 4 (17) | – | 0 (0) | – | 12 (18) | 26/147 (19) |
| Fever | – | – | – | – | – | – | – | – | – | – | – | – | – | 21 (31) | 21/68 (31) |
| Fatigue | – | – | – | – | – | – | – | – | – | – | – | – | – | 15 (22) | 15/68 (22) |
| Anorexia/weight loss | 6 (33) | – | 1 (4) | – | – | – | – | – | – | – | – | – | – | 8 (12) | 15/112 (13) |
| Symptoms prior to presentation months | 17 (2–72) | – | – | – | – | – | – | – | – | – | – | – | – | 14 (0.25–120) | 13 (1–120) |
| No symptoms (incidental finding) | – | – | 1 (4) | – | – | – | – | – | – | 1 (4) | – | 1 (8) | – | 5 (7) | 8/129 (6) |
| Normal physical examination | – | – | – | – | – | – | – | – | – | – | – | – | – | 13 (19) | 13/68 (19) |
| Crackles | – | – | 17 (65) | – | – | – | – | – | – | 13 (57) | – | 11 (92) | – | 41 (72) | 82/129 (64) |
| Clubbing | 5 (28) | – | 12 (46) | – | – | – | – | – | – | 6 (26) | – | 7 (58) | – | 18 (32) | 48/147 (33) |
Data are presented as n, n (%), n/n (%), mean±sd, or mean (range). Available data from case series and individual cases are presented. Totals data are summarised as percentages of available data.