sle myelitis

1. Grey matter myelitis is a severe phenotype of myelitis in systemic lupus erythematosus -
Experience from a tertiary care hospital in south India over a decade.
Ritasman Baisya (1), GSR Murthy (2), Phani K Devarasetti (1) , Liza Rajasekhar (1)
1- Nizam’s Institute of Medical Sciences ,Hyderabad
2- Indian Statistical Institute , Hyderabad

2. Introduction
SLE-associated myelopathy, one of the 12 NPSLE syndromes, is defined by neurological deficits resulting from
spinal cord impairment attributable to SLE.
Lupus myelitis ( LM) - Severe but uncommon complication of lupus , prevalence- 0.9% ( pooled data)
Grey matter myelitis (GMM ) - Acute deteriorating flaccid paralysis associated with anterior horn cell injury (LMN )
White matter myelitis (WMM) -spasticity and hyperreflexia with less severity and slower progression of paralysis
(UMN)

3. 22 lupus myelitis patients
11- Flaccidity & hyporeflexia ( GMM ) ,11- spasticity & hyperreflexia ( WMM)
GMM - Irreversible paraplegia but monophasic course , high SLEDAI , CSF mimicking bacterial
meningitis
WMM - More APL positivity , NMO antibody association

4. ● 10/1768 - lupus myelitis ( 1989-2018 )
● Cervicothoracic - common site , 7/10 - Ist presentation
● 4 - relapsing ( Median time- 0.65 years (0.3-7 years )

5. • 45 SLE –TM patients ( retrospective data – 1993-2018)
• 22 met NMOSD criteria , less rash , renal, complement & dsDNA .
• Non-recovered group – young , fever , initial severe myelitis , long segment myelitis , late MPS
pulse ( after 2 weeks of disease onset ) , high CRP

6. • 35 patients (partial, n = 15; complete, n = 20)
• Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role.
• Five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case).

7. Objectives
1. To identify the two clinical phenotypes of LM patients from our centre
2. To compare the disease activity and outcome among the two groups
3. To formulate a model for predicting the survival in LM

8. Method
● Retrospective study
● Data of all LM patients during the past 15 years (2007-2022)was extracted from the lupus
registry (N=1550).
● Clinical data was assessed dividing into GMM & WMM subtypesubtypes.
● Disease activity was assessed by SLEDAI-2K
● Outcome of illness was recorded as death/recurrence/disability (scored by Modified Rankin
score -MRS).
● MRS at discharge & SLEDAI at admission were used as predictors for survival outcome among
the subtypes.

9. ● Cervical segment myelitis in a patient of SLE
– T2 hyperintensity in cervical spine segment

10. Modified Rankin score
Scale for measuring disability /dependence in neurological diseases
Used in clinical trials

11. Baseline characteristics of LM
No of patients - 35
Mean age at presentation 24.86 + 8.29
F: M = 32:3
Disease duration less than five years - 27/35
Diagnosis with myelitis same time - 18/35
Myelitis evolving in less than 1 month = 30/35
Fever = 33/35
Flaccid paralysis- 24/35
Hypotonia - 24/35
Paresthesia- 24/35
Thoracic level of sensory less - 21/35
Sensory loss- 27/35
Bladder involvement - 31/35
Initial bladder involvement - 10/35
CSF pleocytosis – 5/13
CSF elevated protein – 9/13
MRI done – 28/35
Positive MRI findings – 9/28
Mean MRS – 3.47+1.53
Median SLEDAI (IQR) – 14 ( 14)
Hospital death – 6
Total death -11
Relapse - 6

12. Variables GMM WMM p-value
Demography No of the patients (n) 24 11
Age (Median) (IQR) 24 (14-3 4) 27(11-43) 0.69
F: M 22/2 10/1
Clinical presentation Myelitis as the initial presentation 12/23 6/11 0.81
Bladder involvement 23/23 9/11
Other CNS events 17 3 0.02
Other organ
involvement
Nephritis 10 3 0.59
Myocarditis 6 0 -
GI (enteritis, pancreatitis) 5 2 0.83
Thrombocytopenia 11 3 0.25
Investigation Low complement 19 9 0.67
dsDNA positivity 17 6 0.46
ACL (IgG/IgM) positivity 4 6 0.03
Imaging -MRI of the whole spine 6/18 3/10 0.83
Median SLEDAI(IQR) 13 (10) 9(13) 0.021
GMM vs WMM

13. Treatment Methylprednisolone pulse 20/23 8/11
Cyclophosphamide 14/23 9/11 -
Plasma exchange 2/23 0 -
Intravenous immunoglobulin (IVIG) 1/23 0
Outcome Mean MRS at discharge 3.86+1.7 3+1.18 0.08
Death 9/23 2/11 0.60
Death during admission 6/23 0/11 -
Recurrence of myelitis 4/23 2/11 0.95
Mean survival time (months) 103.3 110.8 0.20
Chance of death of 25% of patients (months) from last attack <13m <96
Chance of death of 50 % of patients <85m <256m
Chance of death of 75% of patients <376m <556m
Infections Infections on follow up 11 (sepsis-5, infected ulcer -
4, UTI - 1, zoster 1)
4 (3 UTI,1 -
sepsis)
0.79
Variables GMM WMM p-value

14. Results
• Patients with GMM had more other CNS events (p-0.02), high SLEDAI (p-0.021) & extra-CNS
involvement (p- 0.05).
• Anticardiolipin antibodies were present more in WMM (p-0.03).

15. Survival statistics – Weibull
plot
25%, 50 % & 75% of patients with GMM
had a chance to die within 13, 85, 376
months from the first attack while the same
for WMM is 96,256, 556 months
respectively implying GMM has a worse
survival outcome.

16. Kaplan Meier survival curve
K-M analysis showed that mean survival
time for GMM ( 110 m (95 % CI-76-145
) is longer than WMM (103 m ( 95 % CI -
82-123m ) though statistically not
significant ( ( Log Rank (Mantel-Cox) ,
p= 0.207) )

17. Prediction of survival status
MRS at discharge & SLEDAI can predict survival status in GMM (R=0.64, p= 0.04), not in WMM (R= 0.42, p=
0.46).
Survival status for GMM (ŷ)= 0.177MRS + 0.00033 SLEDAI - 0.29769

18. Discussion - GMM
• More clinical prodrome ( fever , vomiting ) , early recognition prevent irreversible paraplegia
• Flaccidity & hypotonia , early urinary involvement with atonic bladder
• Correlates with high disease activity ,dsDNA positivity , need of extensive IS
• CSF resembles inflammatory meningitis , culture negative
• MRI – extensive cord swelling but less Gd enhancement ( compromised water shed blood flow )
• Less relapse ( monophasic ) but poor survival with more disability

19. Discussion - WMM
• Indolent course , nadir at >72 hrs
• Spasticity and hypertonia
• Association with APL ( LAC , ACL ) , Anti Ro
• Relapsing but less disability
• More NMOSD manifestation - optic neuritis / Anti NMO Ab positivity
• Less association with disease activity
• Less cord edema but more Gd enhancement
• Median survival time varies due to relapsing course

20. Limitations of study
• Retrospective nature
• Missing data
• Contrast imaging not done
• NMO antibody not available for all patients
• Small sample size
• Heterogeneous disease course

21. Strength
● Largest cohort of lupus myelitis till published
● Follow up data well documented
● One of the few studies based on GMM & WMM differentiation

22. Research area
● Detailed imaging with Gd enhancement to differentiate GMM vs WMM
● Histopathological analysis of GMM vs WMM
● Validation of a newer disability score in LM
● Longitudinal study of LM patients

23. References
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