Session Information
Date: Sunday, October 21, 2018
Title: Systemic Lupus Erythematosus – Clinical Poster I: Clinical Manifestations and Comorbidity
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose:
Non-infectious myelitis in SLE is caused by heterogenous disease processes including SLE myelitis, comorbid multiple sclerosis (MS), or anti-aquaporin-4 antibody (AQ4) mediated neuromyelitis optica (NMO). These conditions with different treatments and prognosis are difficult to differentiate. We compared the demographic, clinical, laboratory, and radiographic characteristics of these 3 conditions in SLE patients at a large academic institution.
Methods:
We searched the Brigham and Women’s Hospital Lupus Center Registry comprised of 2,297 patients with ≥4 1997 ACR revised criteria for SLE. Neurologic diagnoses within this population were identified by text string searches within electronic medical records for the terms “myelitis”, “NMO”, “neuromyelitis optica”, and “multiple sclerosis” between January 1, 2000 and December 31, 2015. Each subject was then reviewed by an attending neurologist to confirm the diagnosis of myelitis, NMO, or MS. To be classified as NMO, subjects required a positive AQ4 antibody and a neurologic syndrome typical of NMO (myelitis +/- optic neuritis). To be classified as MS, subjects required 2 separate neurologic syndromes typical of MS with characteristic brain lesions. Demographic, clinical, laboratory, and radiographic data were extracted. Characteristics of these 3 groups were compared using Fisher’s exact test for categorical variables and analysis of variance for continuous variables. Wilcoxon rank-sum test was used for SLEDAI-2K score, ESR, and CRP level as these values were not normally distributed.
Results:
Fifteen subjects with SLE (0.7%) met criteria for a spinal cord syndrome: 7 had myelitis, 3 had NMO, and 5 had MS (Table). The median SLEDAI-2K score at time of neurologic syndrome presentation was higher in myelitis subjects (8, IQR 7-16) compared to subjects with NMO (6, IQR 0-14) or MS (2, IQR 0-4), p=0.02. Subjects with myelitis were also more likely to have elevated anti-dsDNA antibodies at presentation (86%) compared to subjects with NMO (33%) or MS (0%), p=0.03. In all 3 groups, 100% of subjects who had a repeat MRI of the spine ≥6 months after spinal disease onset had persistent lesions. One year after spinal disease onset, all subjects were either in American Spinal Injury Association Impairment Scale (AIS) category D (incomplete motor loss, ≥4/5 strength) or category E (normal function).
Conclusion:
Compared to subjects with SLE + NMO and subjects with SLE + MS, subjects with SLE myelitis had higher SLE disease activity at myelitis onset as indicated by SLEDAI-2K scores and elevated anti-dsDNA antibody levels. Studies involving larger populations should be conducted to differentiate these 3 conditions.
Table. Comparison of subjects with SLE myelitis, SLE + NMO, and SLE + MS at neurologic event presentation*
|
|
SLE myelitis (n=7)
|
SLE + NMO (n=3)
|
SLE + MS (n=5)
|
p-value
|
|
Demographic Characteristics
|
|
|
|
|
|
Age, mean ± SD years |
41 ± 10 |
46 ± 14 |
40 ± 13 |
0.80 |
|
Sex |
|
|
|
0.67 |
|
Female
|
5 (71) |
3 (100) |
5 (100) |
|
|
Male
|
2 (29) |
0 (0) |
0 (0) |
|
|
Race |
|
|
|
1.00 |
|
White
|
6 (86) |
3 (100) |
5 (100) |
|
|
Black
|
1 (14) |
0 (0) |
0 (0) |
|
|
Years since SLE onset, mean ± SD |
10 ± 11 |
22 ± 15 |
16 ± 3 |
0.23 |
|
Clinical Characteristics
|
|
|
|
|
|
SLE flare |
3 (43) |
0 (0) |
0 (0) |
0.25 |
|
Median SLEDAI-2K score (IQR) |
8 (7-16) |
6 (0-14) |
2 (0-4) |
0.02 |
|
Sensory loss |
6 (86) |
1 (33) |
3 (60) |
0.39 |
|
Weakness |
4 (57) |
2 (67) |
1 (20)
|
0.41 |
|
Bowel or bladder symptoms |
3 (60) |
1 (33) |
1 (20) |
0.77 |
|
Optic neuritis |
2 (29) |
2 (67) |
0 (0) |
0.13 |
|
Full response to acute treatment |
1 (14) |
0 (0) |
1 (20) |
1.00 |
|
Recurrence of disease |
1 (14) |
1 (33) |
0 (0) |
0.67 |
|
AIS Category E (normal) at 1 year
|
3 (43) |
1 (50) |
3 (60) |
1.00 |
|
Laboratory Characteristics
|
|
|
|
|
|
Elevated anti-dsDNA antibody |
6 (86) |
1 (33) |
0 (0) |
0.03 |
|
Hypocomplementemia |
4 (57) |
1 (33) |
1 (33) |
1.00 |
|
Antiphospholipid antibody positive |
5 (71) |
0 (0) |
1 (20) |
0.15 |
|
Median ESR in mm/hr (IQR) |
19 (12-45) |
22 (13-34) |
11 (5-20) |
0.24
|
|
Median CRP in mg/L (IQR) |
7 (2-13) |
5 (2-8) |
0.6 (0.6-2) |
0.07
|
|
Oligoclonal bands in CSF |
2(67)
|
0 (0)
|
2 (100) |
0.23 |
|
MRI Characteristics
|
|
|
|
|
|
Single spinal lesion |
5 (71)
|
1 (50) |
1 (25) |
0.39 |
|
Contrast-enhancing lesion(s) |
4 (67) |
1 (50) |
1 (25) |
0.48 |
|
Longitudinally extensive myelitis |
2 (29) |
1 (50) |
1 (25) |
1.00 |
|
Persistent lesion(s) after ≥6 months |
6 (100) |
1 (100) |
4 (100) |
N/A |
*Unless indicated otherwise, values are the number (%).
AIS=American Spinal Injury Association Impairment Scale
Note: missing values were excluded from analyses.
To cite this abstract in AMA style:
Williams J, Speyer C, Kreps D, Costenbader K, Bhattacharyya S. Spinal Cord Syndromes Associated with Systemic Lupus Erythematous: Differentiating Lupus Myelitis, Neuromyelitis Optica, and Multiple Sclerosis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/spinal-cord-syndromes-associated-with-systemic-lupus-erythematous-differentiating-lupus-myelitis-neuromyelitis-optica-and-multiple-sclerosis/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/spinal-cord-syndromes-associated-with-systemic-lupus-erythematous-differentiating-lupus-myelitis-neuromyelitis-optica-and-multiple-sclerosis/