Outcome of clinical characteristics, pulmonary function tests, and CT scan patterns after autologous hematopoietic stem cell transplant. Results are given as median (range).
| Clinical Characteristics | Baseline | 6 Months | 1 Year | 2 Years | 3 Years | 4 Years | 5 Years |
|---|---|---|---|---|---|---|---|
| No. of patients | 9 | 9 | 9 | 8 | 7 | 7 | 7 |
| NYHA class of dyspnea I/II/III/IV | 3/4/2/0 | 4/4/0/1 | 5/3/1/0 | 4/4/0/0 | 5/2/0/0 | 3/4/0/0 | 5/2/0/0 |
| Respiration rate at rest | 14 (14–20) | 14 (12–24) | 14 (12–15) | 12 (12–25) | 16 (12–16) | 16 (14–18) | 16 (16–20) |
| mRSS | 36 (9–51)* | 24 (4–37)* | 25 (3–44)* | 11 (0–48)* | 11 (3–43)* | 6 (2–35)* | 6 (3–23)* |
| SHAQ | 2.12 (0–2.7) | 0.87 (0–2.12) | 1 (0–3.25) | 0.37 (0–2.3) | 0.62 (0–2.1) | 0.75 (0–2.2) | 1 (0–2.25) |
| Pulmonary function tests, % predicted | |||||||
| VC | 72 (37–108) | 74 (32–114) | 76 (58–113) | 83 (62–111)† | 74 (58–112) | 68 (44–115) | 60 (34–122) |
| TLC | 82 (42–114) | 80 (43–100) | 74 (63–109) | 86 (76–118) | 77 (67–115) | 75 (64–116) | 73 (54–114) |
| FEV1 | 69 (44–112) | 83 (38–122) | 83 (61–128) | 79 (67–113) | 78 (54–115) | 73 (50–114) | 67 (42–121) |
| DLCO | 46 (28–61) | 51 (12–76) | 42 (36–53) | 44 (38–74) | 40 (31–60) | 41 (33–85) | 40 (16–74) |
| KCO | 61 (36–86) | 56 (16–80) | 47 (32–74) | 60 (56–84) | 68 (53–71) | 62 (52–84) | 54 (31–78) |
| FEF75 | 86 (63–123) | 93 (63–139) | 93 (59–139) | 104 (86–28) | 99 (61–117) | 86 (52–122) | 72 (48–128) |
| FEF50 | 79 (52–182) | 98 (69–131) | 95 (73–141) | 90 (76–139) | 96 (78–119) | 80 (52–126) | 87 (48–119) |
| FEF25 | 82 (40–134) | 113 (64–197) | 82 (54–146) | 78 (32–143) | 76 (53–99) | 68 (28–97) | 70 (47–113) |
| Overall CT scan patterns | |||||||
| Disease extent | 10 (0–45) | 4 (0–36)* | 6 (0–39) | 12 (0–32.5) | 16 (1–38.8) | 13 (1–32.5) | 15 (1–33) |
| Ground-glass opacification, % | 50 (12–90) | 30.5 (0–90) | 42 (0–67) | 37 (0–60) | 20 (0–45) | 17 (0–50) | 20 (3–55) |
| Coarseness score | 8.1 (4–15) | 10 (5–15) | 8.3 (3.3–15) | 10 (5–15) | 10 (5–15) | 10 (5–15) | 10 (5–15) |
| Grade 1/2/3, n | 2/2/2 | 1/2/3 | 1/5/2 | 0/5/2 | 0/3/4 | 0/4/3 | 0/3/4 |
| Improvement/stability/worsening on serial HRCT | — | 5/4/0 | 1/3/5 | 2/2/4 | 2/2/3 | 4/2/1 | 2/2/3 |
NYHA: New York Heart Association; mRSS: modified Rodnan skin score; SHAQ: Self Health Assessment Questionnaire; VC: slow inspiratory vital capacity; TLC: total lung capacity; DLCO: diffusing capacity for carbon monoxide; KCO: DLCO adjusted for alveolar volume; FEV1: forced expiratory volume in 1 second; FEF: forced expiratory flow rate; PF-SSc: fibrosing alveolitis (pulmonary function) associated with systemic sclerosis.
↵* p < 0.05 compared to baseline.
↵† p < 0.1 compared to baseline.