Table 2.
Differences in Imaging, Pulmonary Function Test, Treatment, and Outcomes Among Three Subgroups of Patients with Relapsing Polychondritis (RP)
| All Patients n=73 | Type 1 RP n=10 | Type 2 RP n=21 | Type 3 RP n=42 | p value | |
|---|---|---|---|---|---|
|
| |||||
| Chest Dynamic CT Scan | |||||
|
| |||||
| Airtrapping n (%) | 35 (51) | 8 (89%) | 14 (67%) | 13 (34%) | 0.002 |
| Tracheal wall >3 mm n (%) | 16 (22) | 9 (90%) | 3 (14%) | 4 (10%) | <0.0001 |
|
| |||||
| Pulmonary Function Test | |||||
|
| |||||
| FVC Pre % (median, IQR) | 91 (83–101) | 94 (58–102) | 90 (81–103) | 91 (85–100.5) | 0.92 |
| FVC L/sec (median, IQR) | 3.3 (2.8–3.8) | 2.9 (1.9–3.2) | 3.3 (2.4–3.8) | 3.4 (3–3.9) | 0.12 |
| FEV1 Pre % (median, IQR) | 85 (75–96) | 76 (26–84) | 83 (67–97) | 89 (81–89) | 0.06 |
| FEV1 L/sec (median, IQR) | 2.4 (2–3) | 1.34 (0.7–2.3) | 2.3 (1.9–2.9) | 2.75 (2.3–3.1) | 0.01 |
| FEF 25–75 %Ref (median, IQR) | 80 (51–94) | 38 (15–66) | 88 (47–92) | 82 (65–97) | 0.01 |
| FEF 25–75 L/sec (median, IQR) | 2.32 (1.49–2.85) | 0.9 (0.4–1.7) | 1.98 (1.4–2.5) | 2.58 (2–3) | 0.01 |
| FEV1/FVC % (median, IQR) | 76 (72–80) | 60 (43–71) | 76 (66–80) | 79 (75–81) | 0.0006 |
| VC% Ref (median, IQR) | 95 (86–107) | 91 (44–102) | 94 (86–111) | 96 (87–107) | 0.55 |
| VC L/sec (median, IQR) | 3.34 (2.86–3.99) | 3 (1.42–3.2) | 3.1 (2.7–3.8) | 3.46 (3.1–1.4) | 0.04 |
| TLC % Ref (median, IQR) | 93 (87–101) | 92 (66–97) | 94 (87–105) | 93 (89–99) | 0.78 |
| TLC L/sec (median, IQR) | 4.8 (4.2–5.4) | 4.7 (4.5–5.3) | 4.7 (4–5.7) | 4.9 (4.2–5.4) | 0.7 |
| RV % Ref (median, IQR) | 74 (61–86) | 75 (51–101) | 82 (66–97) | 74 (61–83) | 0.36 |
| RV L/sec (median, IQR) | 1.36 (1.1–1.5) | 1.5 (1.4–2.7) | 1.4 (1.1–1.6) | 1.2 (0.9–1.5) | 0.06 |
| RV/TLC Ref (median, IQR) | 77 (68–88) | 80 (65–104) | 87 (71–95) | 72 (64–85) | 0.12 |
| DLCO Adj %(median, IQR) | 18 (17–22) | 18 (15–19) | 18 (17–22) | 19 (17–21) | 0.25 |
| DLCO Adj (median, IQR) | 68 (64–74) | 66 (61–70) | 67 (64–80) | 69 (61–74) | 0.61 |
|
| |||||
| Physician Global Assessment | 3 (2–4) | 3 (2–4) | 4 (3–5) | 5 (3.75–6) | 0.0003 |
|
| |||||
| Treatment | |||||
|
| |||||
| Current prednisone dose | 7 (0–20) | 10 (5–20) | 7.5 (0–25) | 5 (0–20) | 0.69 |
| Sythentic DMARD’s n (%) | 49 (67) | 8 (80) | 16 (76) | 25 (59) | 0.25 |
| Biologics n (%) | 32 (43) | 7 (70) | 8 (38) | 17 (40) | 0.19 |
|
| |||||
| Complications | |||||
|
| |||||
| Tracheostomy n (%) | 7 (1) | 5 (50) | 0 (0) | 2 (5) | 0.0003 |
| Disability n (%) | 25 34) | 2 (20) | 12 (57) | 11 (26) | 0.10 |
| ICU admission n (%) | 12 (16) | 5 (50) | 2 (9) | 5 (12) | 0.02 |
|
| |||||
| Inflammartoy Markers | |||||
|
| |||||
| CRP (mg/dL) | 2.4 (0.8–8.6) | 4.7 (0.37–12.5) | 3.9 (1.25–8.1) | 2 (0.77–0.52) | 0.568 |
| ESR (mm/hr) | 11 (6–20) | 13.5 (3.5–54.5) | 9 (6.5–20) | 11.5 (6–19.5) | 0.974 |
N = number; IQR = interquartile range; DMARDs = disease modifying antirheumatic drugs