NSIP (- NSIP) (Non-)
NSIP (Kinder) Retrospective () (Non-)
(Kinder) 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 1. 2. RA 3. Scl-70 4. SS-ASS-B 5. Jo-1 6. (>), CRP
NSIP 62 6PM/DM* 9 47 *PM/DM: Polymyositis/dermatomyositis
47 22 / 25 58 (24-83)* (Current / Ex./ Never) 13 / 13 / 21 47 (5-206) (%) 81 (%) 51 (%) 83 % 11 %VC 66 (22-108) %DLco 66 (25-133) PaO2 (Torr) 80 (50-95) KL-6 (U/ml) 1290 (284-4890) SP-D (ng/ml) 229 (33-739) *. median (range)
NSIP Non- 25 (53%) 22 (47%)
(%)
22 (%) 0 20 40 60 80 100 n=22 RA n=21 Scl-70 n=19 SS-A n=20 SS-B n=20 Jo-1 n=19 n=19 CRP n=22
Non- Non- 22 25 p value 8 / 14 14 / 11 n.s. 57 (24-77)* 58 (38-83) n.s. (Current / Ex./ Never) 3 / 7 / 12 10 / 6 / 9 n.s. 46 (5-206) 49 (5-166) n.s. (%) 73 60 n.s. (%) 15 20 n.s. (%) 77 76 n.s. % 9 5 n.s. *: median (range) n.s.: not significant
Non- (%)
Non- %) Non-
Non- Non- p value WBC (µl) 7,400 ± 2,300* 6,700 ± 2,200 n.s. LDH (IU/L) 333 ± 149 293 ± 104 n.s. CRP (mg/dl) 0.87 ± 1.00 0.60 ± 1.55 n.s. ESR (mm/hr) 38 ± 7 22 ± 16 n.s. CPK (IU/L) 252 ± 415 94 ± 50 n.s. KL-6 (U/mL) 1,690 ± 1,194 1,792 ± 1,410 n.s. SP-D (ng/ml) 261 ± 203 263 ± 132 n.s. *: Mean ± SD
Non- Non- ( ) 80 (40-320)* 120 (20-1280) SS-A ( )** 4 (4-128) 4 Jo-1 ( )** 1 DNA (IU/mL)*** 7.5 (7.0-7.9) RNP (U/mL) # 208 PR3-ANCA (E.U.) # 55 *: Median (range), **:, ***: RIA, # : ELISA,
Non- %VC FEV1% %DLco PaO2 % % % Torr *: Mean ± SD
BAL Non- CD4/CD8 100 % 100 % p<0.05 30 % 30 % 15 p<0.05 80 80 60 60 20 20 10 40 40 10 10 5 20 20 0 Non- 0 Non- 0 Non- 0 Non- 0 Non-
HRCT Non- Non- p value // 0 / 83 / 17* 0 / 65 / 35 n.s. 100 100 n.s. 67 40 n.s. 78 75 n.s. 62 35 n.s. 22 45 n.s. 94 95 n.s. 0 10 n.s. 6 10 n.s. 17 15 n.s. 0 0 n.s. *: %
Non- Non- p value Cellular NSIP / Fibrotic NSIP 2 / 20 3 / 22 n.s. Alveolar wall inflammation 2.0 ± 0.4* 1.9 ± 0.6 n.s. Intra-alveolar macropahges 0.8 ± 0.4 0.8 ± 0.8 n.s. Organizing pneumonia 0.8 ± 0.8 0.6 ± 0.7 n.s. Germinal center 0.7 ± 1.2 0.3 ± 0.5 n.s. Fibrosis 2.2 ± 0.6 2.2 ± 0.8 n.s. Fibroblastic foci 0.3 ± 0.5 0.5 ± 0.7 n.s. Honey combing 0.3 ± 0.8 0.8 ± 0.3 n.s. Pleural lesion 0.3 ± 0.9 0.3 ± 0.9 n.s. *: Scores 1-3, Mean ± SD
Non- 23% 77% + CPA 6% + CsA 29% + AZA 6% PSL 59% 0.5% 99.5% Non- 20% 80% + AZA 10% + CsA 25% + CPA 5% PSL 65% 32% 68% CsA: cyclosporin, AZA: azathioprine, CPA: cyclophosphamide
Non- 1.0 (n=22) 0.8 0.6 0.4 Non- (n=25) Log rank: p<0.0085 0.2 0.0 0 5 10
NSIP Cox p value ( ) 0.614 0.279-1.209 0.164 ( ) 0.979 0.909-1.054 0.583 (Non-) 3.225 1.357-13.98 0.0053 (Current) 1.569 0.539-1.001 0.556 LDH (IU/L) 0.996 0.990 - ± 50 0.220 KL-6 (U/mL) 0.999 0.998-1.000 0.460 SP-D (ng/ml) 0.989 0.958-1.005 0.274 %VC (%) 1.002 0.973-1.032 0.841 %DLco (%) 1.030 0.991-1.072 0.123 BAL (%) 0.917 0.786-0.987 0.0107 BAL (%) 0.941 0.692-1.088 0.531 BAL (%) 0.982 0.722-1.202 0.880 BAL CD4/CD8 0.962 0.668-1.200 0.774
NSIP Cox p value ( ) 0.839 0.213-3.19 0.793 ( ) 0.970 0.843-1.098 0.636 (Non-) 3.189 1.092-15.64 0.032 BAL (%) 0.868 0.692-0.71 0.040 NSIP
NSIP47 (Kinder) 22 (47%) 22 () 25 (Non-) Non- Never smoker %VC %DLco BAL CD4/CD8 HRCT Germinal centerhoney combing NSIP
Kinder NSIP Mosca NSIP (Kinder) NSIP NSIP NSIP NSIP