Table 1.
Frequency of NPSLE Syndromes by ACR Nomenclature in Different Cohorts, n (%).
| ACR Nomenclature | Ainiala, 20017 (n=46) | Sanna, 20038 (n=323) | Brey, 20029 (n=128) | Afeltra, 200310 (n=61) | Costallat, 200111 (n=527) | Hanly, 201012 (n=1206) | Mok, 200613 (n=282) | Ferrara University, 201415 (n=228) | Multiple Italian Centers, 201415 (n=221) | Faria, 201416 (n=55) | Steup-Beekman, 201314 (n=102) | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
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| Model A | Model B | |||||||||||
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| Aseptic Meningitis | 1 (2) | 2 (0.4) | 4 (2.7) | 4 (1.6) | 1 (0.4) | 3 (0.7) | 0 | 6 (10.9) | 3 (3) | |||
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| Cerebrovascular Disease | 7 (15) | 47 (14.5) | 2 (2) | 15 (24) | 13 (2.5) | 18 (12.1) | 40 (15.5) | 21 (7.4) | 68 (16.2) | 61 (14.3) | 16 (29) | 44 (43) |
|
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| Demyelinating Syndrome | 1 (2) | 3 (0.9) | 1 (0.2) | 1 (0.7) | 3 (1.2) | 0 | 3 (0.7) | 4 (0.9) | 1 (1.8) | |||
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| Headache | 25 (54) | 78 (24) | 73 (57) | 13 (21) | 25 (62.5) (n=40) | 0 | 0 | 8 (2.8) | 116 (27.7) | 95 (22.2) | 4 (7.2) | 23 (23) |
|
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| Movement Disorder (Chorea) | 1(2) | 4 (1.2) | 1 (1) | 4 (0.76) | 4 (2.7) | 5 (1.9) | 2 (0.7) | 7 (1.7) | 3 (0.7) | 1 (1.8) | 5 (5) | |
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| Myelopathy | 4 (1.2) | 2 (3) | 6 (1) | 5 (3.4) | 10 (3.9) | 6 (2.1) | 1 (0.2) | 4 (0.9) | 2 (3.6) | 6 (6) | ||
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| Seizure Disorders | 4 (9) | 27 (8.3) | 21 (16) | 7 (11) | 39 (7) | 39 (26.2) | 54 (20.9) | 17 (6) | 19 (4.5) | 61 (14.3) | 10 (5.5) | 28 (27) |
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| Acute Confusional State | 3 (7) | 12 (3.7) | 15 (3) | 11 (7.4) | 17 (6.6) | 10 (3.5) | 7 (1.7) | 13 (3) | 7 (7) | |||
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| Anxiety Disorder | 6 (13) | 24 (7.4) | 27 (21) | 4 (6) | 28 (70) (n=40) | 0 | 0 | 3 (1.1) | 15 (3.6) | 28 (6.6) | 1 (1) | |
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| Cognitive Dysfunction | 37/46 (81) | 35 (10.8) | 53/67 (79) | 32/61 (52) | 32 (52) | 8 (5.4) | 22 (8.5) | 10 (3.5) | 61 (14.6) | 56 (13.1) | 6 (10.9) | 27 (26) |
| – Minor | 26/37 (70) | 29/67 (43) | ||||||||||
| – Moderate | 7/37 (19) | 20/67 (30) | ||||||||||
| – Severe | 4/37 (11) | 4/67 (6) | ||||||||||
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| Mood Disorder | 20 (43) | 54 (16.7) | 25 (20) | 17 (27) | 30 (75) (n=40) | 18 (12.1) | 47 (18.1) | 10 (3.5) | 67 (16) | 49 (11.5) | 8 (8) | |
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| Psychosis | 25 (7.7) | 6 (5) | 28 (5) | 8 (5.4) | 13 (5) | 15 (5.3) | 13 (3.1) | 17 (4) | 5 (9.1) | 6 (6) | ||
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| Acute Inflammatory Demyelinating Polyradiculoneuropathy | 2 (0.6) | 2 (1.3) | 2 (0.8) | 0 | 0 | 1 (0.2) | 1 (1) | |||||
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| Autonomic Disorder | 2 (3) | 1 (0.2) | 2 (1.3) | 2 (0.8) | 0 | 0 | 1 (0.2) | - | ||||
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| Mononeuropathy, Single/Multiplex | 9 (7) | 7 (1) | 10 (6.7) | 18 (6.9) | 1 (0.4) | 6 (1.4) | 8 (1.9) | 3 (3) | ||||
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| Myasthenia Gravis | 1 (2) | 1 (0.2) | 0 | 0 | 2 (0.7) | 2 (0.4) | 2 (0.5) | - | ||||
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| Neuropathy, Cranial | 3 (7) | 2 (2) | 3 (4) | 8 (1.5) | 11 (7.4) | 11 (4.3) | 7 (2.4) | 14 (3.4) | 9 (2) | 6 (6) | ||
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| Plexopathy | 0 | 0 | 0 | 0 | 0 | 2 (2) | ||||||
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| Polyneuropathy | 13 (28) | 29 (23) | 8 (13) | 20 (4) | 8 (5.4) | 10 (3.9) | 0 | 17 (4) | 16 (3.7) | 4 (7.2) | 2 (2) | |