MYELITIS IN

LUPUS
Myelopathy due to SLE is one
of the 19 associated
neuropsychiatric syndromes (12
CNS and 7 PNS) defined by the
American College of
Rheumatology (ACR 1999)

Myelopathy is one of the least

common neuropsychiatric
syndromes (1- 2% of the
patients), which is still more
than 1,000 times greater than the
prevalence of idiopathic myelitis
in the general population.
 Based on pathological and serological findings, it has been proposed
that small vessel vasculitis and thrombosis would be the 2 major
mechanisms most directly responsible for the neuronal and axonal
damage.
Another mechanism could involve the so-called cooperation between
antibodies: ischemia would induce the synthesis of AQP4 with the
subsequent development of lupus myelitis associated with the
neuromyelitis optica (NMO) spectrum mediated by antiAQP4-IgG or
PATHOPHYSI another type of antibody.

OLOGY It has also been proposed that aPL could have a direct cytotoxic
effect, which would correlate with the presence of oligoclonal bands in
aPL-positive patients.

The B cell activation in the initial episode, may lead B cells to

become potent antigen-presenting cells and therefore, cause cellular
immunity activation leading to treatment intractability and relapses
even after functional B cell depletion through Rituximab therapy.
HENCE, CELLULAR IMMUNITY SEEMS TO PLAY A VERY
IMPORTANT ROLE IN SUCCESSIVE EPISODES (RELAPSES)
OF MYELITIS .
 Although SLE-related myelitis is traditionally regarded as a single diagnostic entity, case
reports and cohort studies show clinical and probably physio-pathogenic heterogeneity.

SLE myelitis encapsulates 2 distinct and previously unrecognized syndromes that can be
distinguished clinically and radiologically due to lesions that preferentially affect distinct
neuroanatomical areas

GREY MATTER MYELITIS WHITE MATTER MYELITIS

GREY MATTER
MYELITIS WHITE MATTER
MYELITIS
 CLINICAL
GREY MATTER
MYELITIS
Much more prominent inflammatory
and clinical prodromes
 Fever (100%)
 Nausea/vomiting (58.3%)
Present with persistent flaccidity and
hyporeflexia.
Deteriorated more rapidly to a clinical
nadir (often irreversible, complete
paraplegia)
- Within 6 hours (in 72.7%)
FEATURES
WHITE MATTER
MYELITIS
Less prominent clinical prodrome (9%)
Present with findings of spasticity and
hyperreflexia, after the initial spinal
shock.
Antigravity strength is often
preserved even at clinical nadir. (41%
vs 8.3%)
Progression to clinical nadir is slower
 >72 hours in 100% patients
 CLINICAL FEATURES
GREY MATTER
MYELITIS WHITE MATTER
Often present with urinary retention,
prior to irreversible paraplegia
MYELITIS
Optic neuritis was associated in
54.5%.
(90.9%) (vs 0%)

The presence of an atonic bladder 45.5% of patients with white matter

(with high postvoid residuals) coupled myelitis satisfied NMO criteria (vs 0%)
with a preserved sense of needing to
void is classically associated with gray
matter lesions in the thoracolumbar
cord (i.e., Onuf nuclei).
 SLE DISEASE ACTIVITY
GREY MATTER
MYELITIS WHITE MATTER
Much more likely to be associated with a
MYELITIS
Seen to be associated with lower SLE
high SLE disease activity. disease activity as compared to grey
 Mean SLEDAI (9.8 vs 2.0) matter myelitis flares.
 ESR (78.2 vs 32.7)
 Low mean C3 levels (65.3 vs 84.7)
 Low mean C4 levels (14.3 vs 24.2)
 Hematologic disease (36% vs 18%)
 Oral ulcers (27% vs 18%)
 Neurologic syndromes (36% vs 27%)
 CSF FINDINGS
GREY MATTER
MYELITIS WHITE MATTER
Striking inflammatory features, MYELITIS
CSF studies are usually
resembling the pattern expected for insignificant, except for slightly
bacterial meningitis. raised total protein levels.
 High median WBCs (385.5 vs
10cells/ml)
 High median PMNLs (71% vs 15%)
 High median total protein levels
(254 mg/dl versus 57 mg/dl)
 Low CSF glucose levels (33mg/dl
versus 54mg/dl)
 MRI
GREY MATTER
MYELITIS
 Cord swelling was seen more
commonly (91.7% vs 21.7%).
 However, post-gadolinium
enhancement was seen in only 25% of
MRIs (vs 42.9%).
WHITE MATTER
MYELITIS
Post-gadolinium enhancement was
seen more frequently in patients with
white matter myelitis, despite the
tendency of white matter myelitis to
occur with less severe inflammatory
systemic and CSF findings.

 Longitudinally extensive pattern of

inflammation (spanning at least 3
vertebral segments) was seen in 91.7%
(vs 73.9%).
AUTOANTIBODY PROFILE
 PROGNOSIS
GREY MATTER
MYELITIS WHITE MATTER
Despite more intensive immunosuppressive
treatment, patients with grey matter myelitis
MYELITIS
The median EDSS was 6.0
had more disability. (range 2.0–10.0) in patients with
The median EDSS (Expanded Disability white matter myelitis.
Status Scale) at the end of follow-up was
Wheelchair dependence was 0%
8.0 (range 8.0- 10.0) in patients with grey
Catheter dependence at recovery
matter myelitis
was 30%
Wheelchair dependence was 100%
Catheter dependence at recovery was
*An EDSS score of 6.0 or greater
100% corresponds to functional dependence on
*An EDSS score of 8.0 is indicative of ambulatory assistive devices to walk
dependence on a wheelchair minimal distances
TREATMENT IN GREY MATTER MYELITIS
 Because of the presence of fevers, all of the patients were unfortunately and
erroneously diagnosed as having urinary tract infections. Therefore, unexplained
urinary retention in a patient with SLE, especially when accompanied by signs
of fever and active SLE, should be regarded as an ominous sign.
 It is proposed that urinary retention in grey matter myelitis represents the
potentially treatable period of ischemia to grey matter micturition tracts.

 IT IS IMPERATIVE THAT THIS EARLY AND POTENTIALLY

SALVAGEABLE CLINICAL PRESENTATION OF GREY MATTER
MYELITIS BE RECOGNISED WITHOUT DELAY FOR EARLY AND
AGGRESSIVE IMMUNOSUPPRESSION, SINCE IT INEVITABLY
PROGRESSES TO COMPLETE PARAPLEGIA WITHIN 24HOURS AND
HAS A VERY HIGH PERCENTAGE OF WHEELCHAIR AND
CATHETHER DEPENDENCE IF UNTREATED.
 RECURRENCE
GREY MATTER
MYELITIS WHITE MATTER
Only a single patient with grey matter MYELITIS
72.7% of patients with white matter
myelitis had a relapse (9.1%) myelitis had at least 2 relapses.

 Studies have noted that anti-Ro/SSA autoantibodies are seen more frequently in
patients with a relapsing pattern of myelitis.

 Given that white matter myelitis was associated with a relapsing course, it was
investigated whether anti-Ro/SSA autoantibodies would be associated with white
matter versus grey matter myelitis. Anti-Ro/SSA autoantibodies were seen more
frequently in patients with white matter myelitis (60.0%) than in patients with grey
matter myelitis (18.2%)
 TAKE HOME
MESSAGE
 2 major types – Grey Matter & White Matter myelitis.
 Grey Matter myelitis has a much more rapid progression,
grave prognosis and a very high percentage of residual
paralysis leading to morbidity.
 Grey matter myelitis often masquerades as a UTI with
acute urinary retention, which needs to be recognized for
prompt and aggressive immunosuppression.
 The only available effective drugs for treatment of
Myelitis in SLE are Steroids and Cyclophosphamide.
There is still a huge dearth of research, novel therapies
and other treatment options for myelitis and other
neuropsychiatric manifestations of SLE.
 REFERENCES
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Subtypes of Myelitis in Systemic Lupus Erythematosus. Arthritis and
Rheumatism 2009 Nov;60(11):3378-3387
2. Edson Hernán Chiganer, Javier Pablo Hryb, Edgar Carnero Contentti.
Myelitis and Lupus: Clinical Manifestations, Diagnosis and Treatment,
Review. Reumatol Clin. 2017;13(6):344-348
3. Alessandra Bortoluzzi, Antonis Fanouriakis, Simone Appenzeller,
Lilian Costallat, Carlo Alberto Scirè et al. Validity of the Italian
algorithm for the attribution of neuropsychiatric events in systemic lupus
erythematosus: a retrospective multicentre international diagnostic
cohort study. BMJ Open 2017;7:e015546.doi:10.1136/bmjopen-2016-
015546