Table 2. Main Characteristics Associated With Overall and Relapse-Free Survival and Neurologic Relapsesa.
| Characteristic | Overall Survival | Relapse-Free Survival | Neurologic Relapse | ||||||
|---|---|---|---|---|---|---|---|---|---|
| No. of Deaths/Patients | HR (95% CI) | P Value | No. of Relapses/Patients | HR (95% CI) | P Value | No. of Relapses/Patients | HR (95% CI)b | P Value | |
| General | |||||||||
| EDSS score at baseline per point | NA | 1.22 (1.07-1.40) | .003 | NA | 0.99 (0.93-1.05) | .69 | NA | 0.99 (0.93-1.07) | .84 |
| Age at NS diagnosis per 10 y | NA | 1.43 (1.06-1.93) | .02 | NA | 0.91 (0.81-1.04) | .16 | NA | 0.82 (0.70-0.97) | .02 |
| Male sex | 12/117 | 1.19 (0.49-2.89) | .69 | 64/97 | 0.90 (0.64-1.28) | .56 | 43/97 | 1.04 (0.68-1.59) | .86 |
| Ethnic background | |||||||||
| White | 13/125 | 1 [Reference] | NA | 76/102 | 1 [Reference] | NA | 52/102 | 1 [Reference] | NA |
| African or Caribbean | 6/58 | 1.05 (0.40-2.79) | .92 | 32/48 | 0.68 (0.44-1.05) | .08 | 20/48 | 0.72 (0.43 to 1.20) | .21 |
| North African and other | 2/51 | 0.49 (0.11-2.18) | .35 | 28/44 | 1.02 (0.66-1.58) | .93 | 16/44 | 0.82 (0.47 to 1.44) | .49 |
| Smoking | 5/54 | 0.90 (0.33-2.50) | .85 | 39/49 | 1.18 (0.80-1.72) | .40 | 24/49 | 1.06 (0.66-1.70) | .81 |
| Sarcoidosis classificationc | |||||||||
| Definite | 5/33 | 1 [Reference] | NA | 23/31 | 1 [Reference] | NA | 14/31 | 1 [Reference] | NA |
| Probable or possible | 16/201 | 0.55 (0.20-1.51) | .24 | 113/163 | 0.86 (0.54-1.35) | .51 | 74/163 | 0.90 (0.50 to 1.59) | .71 |
| Biopsy-proven sarcoidosis | 16/201 | 0.55 (0.20-1.51) | .24 | 99/141 | 0.75 (0.51-1.10) | .14 | 61/141 | 0.70 (0.44-1.11) | .13 |
| Extraneurologic Involvement | |||||||||
| >3 Nonneurologic sites involved | 3/55 | 0.49 (0.14-1.70) | .26 | 35/46 | 1.06 (0.72-1.57) | .76 | 22/46 | 1.02 (0.63-1.67) | .92 |
| Abnormal chest radiographic finding | 17/162 | 1.75 (0.58-5.23) | .32 | 100/137 | 1.00 (0.68-1.47) | >.99 | 63/137 | 0.91 (0.57-1.46) | .70 |
| Heart | 5/84 | 0.53 (0.18-1.54) | .24 | 52/72 | 1.19 (0.81-1.73) | .37 | 36/72 | 1.13 (0.72-1.77) | .61 |
| General symptoms | 7/82 | 0.82 (0.33-2.03) | .66 | 51/67 | 1.15 (0.81-1.63) | .45 | 39/67 | 1.43 (0.94-2.19) | .098 |
| Eye | 4/67 | 0.55 (0.18-1.64) | .28 | 42/53 | 1.16 (0.80-1.69) | .42 | 25/53 | 0.97 (0.61-1.55) | .91 |
| Lymph nodes | 9/67 | 2.00 (0.83-4.84) | .12 | 47/59 | 1.50 (1.05-2.16) | .03 | 29/59 | 1.28 (0.82-2.01) | .28 |
| Cutaneous and mucosal | 3/49 | 0.57 (0.17-1.97) | .37 | 27/40 | 0.77 (0.50-1.18) | .22 | 16/40 | 0.71 (0.41-1.23) | .22 |
| Liver or spleen | 4/41 | 0.87 (0.29-2.63) | .80 | 28/37 | 1.07 (0.70-1.63) | .76 | 15/37 | 0.89 (0.51-1.55) | .67 |
| Joints | 1/36 | 0.27 (0.035-1.99) | .20 | 23/29 | 0.96 (0.61-1.51) | .86 | 15/29 | 1.04 (0.59-1.82) | .89 |
| Exocrine glands | 4/35 | 1.34 (0.44-4.07) | .60 | 22/28 | 0.89 (0.56-1.42) | .62 | 10/28 | 0.64 (0.33-1.24) | .18 |
| Ear, nose, and throat | 0/16 | NA | .22d | 8/11 | 1.90 (0.91-3.95) | .09 | 6/11 | 1.92 (0.82-4.47) | .13 |
| Kidney | 0/7 | NA | .38d | 6/7 | 1.50 (0.63-3.62) | .36 | 3/7 | 1.60 (0.50-5.15) | .43 |
| Digestive tract | 1/7 | 1.35 (0.18-10.1) | .77 | 5/7 | 0.89 (0.36-2.22) | .81 | 2/7 | 0.63 (0.15-2.55) | .51 |
| Bones | 0/6 | NA | .47d | 3/4 | 0.89 (0.28-2.82) | .85 | 2/4 | 0.90 (0.22-3.68) | .88 |
| Neurologic Involvement | |||||||||
| CNS localization | 17/170 | 1.51 (0.50-4.53) | .46 | 103/140 | 1.59 (1.05-2.39) | .03 | 70/140 | 1.80 (1.06-3.06) | .03 |
| Encephalon | 14/143 | 1.18 (0.47-2.96) | .72 | 90/117 | 1.88 (1.30-2.74) | <.001 | 64/117 | 2.35 (1.44-3.83) | <.001 |
| Myelopathy | 6/62 | 0.96 (0.37-2.49) | .93 | 33/48 | 0.83 (0.56-1.24) | .37 | 21/48 | 0.82 (0.50-1.35) | .43 |
| Cranial nerve | 6/86 | 0.70 (0.27-1.82) | .46 | 57/72 | 1.03 (0.72-1.47) | .87 | 38/72 | 1.20 (0.78-1.85) | .40 |
| PNS | 7/24 | 4.38 (1.69-11.3) | .002 | 19/22 | 1.40 (0.85-2.32) | .19 | 11/22 | 1.20 (0.63-2.30) | .58 |
| Myopathy | 3/19 | 2.22 (0.65-7.60) | .20 | 13/18 | 1.54 (0.86-2.74) | .15 | 9/18 | 1.47 (0.73-2.94) | .28 |
| Abnormal MRI finding of the brain or spinal cord | 13/154 | 1.05 (0.14-8.11) | .96 | 91/129 | 1.20 (0.55-2.63) | .65 | 65/129 | 1.37 (0.50-3.77) | .54 |
| CSF protein level >50 mg/dL | 14/117 | 4.57 (1.03-20.3) | .046 | 76/99 | 1.16 (0.78-1.73) | .47 | 54/99 | 1.36 (0.84-2.21) | .21 |
| CSF white blood cells >5/μL | 11/119 | 1.53 (0.53-4.40) | .43 | 72/96 | 1.32 (0.90-1.96) | .16 | 52/96 | 1.45 (0.90-2.32) | .13 |
| Elevated serum ACE level | 11/85 | 1.96 (0.72-5.31) | .18 | 48/73 | 0.87 (0.58-1.30) | .49 | 28/73 | 0.73 (0.45-1.19) | .21 |
Abbreviations: ACE, angiotensin-converting enzyme; CSF, cerebrospinal fluid; CNS, central nervous system; EDSS, Expanded Disability Status Scale; HR, hazard ratio; MRI, magnetic resonance imaging; NA, not applicable; NS, neurosarcoidosis; PNS, peripheral nervous system.
SI conversion factors: To convert protein to grams per liter, multiply by 0.01; white blood cells to ×109 per liter, multiply by 0.001.
All estimations stratified on period of NS diagnosis (1990-1999 vs 2000-2015).
Uses the Cox proportional hazards regresssion model for cause-specific HRs (neurologic relapse, nonneurologic relapse, relapse with unavailable localization, and death as mutually competing risks).
Classified according to Zajicek et al.
Calculated using the log-rank test, stratified on period (1990-1999 vs 2000-2015); estimation of HR using a Cox proportional hazards regression model was not performed owing to the absence of events in the subgroups with kidney (n = 7) or bone (n = 5) involvement vs 23 of 227 and 23 of 229 in the subgroups without kidney or bone involvement, respectively.