Lupus Myelitis Research Articles

Lupus myelitis in a patient with a first diagnosis of systemic lupus erythematosus

No conflict of interest in this study.

P163 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

Abstract Background/Aims Myelitis is a severe yet rare manifestation of systemic lupus erythematosus (SLE). Literature on lupus myelitis is limited to case reports and series. Birnbaum et al. reported the largest series (n = 22) with two distinct phenotypes based on clinical and imaging findings with mechanistic and therapeutic implications. Further validation of these two subtypes is not reported to date. The objective of the study was to identify distinct phenotypes in lupus myelitis and compare activity and outcomes with the formulation of a prediction model for survival. Methods This is a retrospective study. Data of 35 lupus myelitis patients with follow-up were extracted from the lupus registry (n = 1550) in the last 15 years (2007-2022). Grey matter myelitis (GMM) (i.e., flaccidity, hyporeflexia) and white matter myelitis (WMM) (i.e., spasticity, hyperreflexia) were two subtypes. Disease activity was assessed by SLEDAI-2K and outcome by death/recurrence/disability - modified Rankin score (MRS). MRS at discharge and SLEDAI at admission were used as predictors for survival outcomes among subtypes. Results Median age at presentation was 24.5 years (12.5-36.5); female-male ratio 32:3. 24 patients presented with GMM and 11 with WMM. GMM had more CNS events (p-0.02), high SLEDAI (p-0.021) and extra CNS involvement (p > 0.05). Anticardiolipin antibodies were increased in WMM (p-0.03). Weibull survival probability plot showed that 25%, 50% & 75% of GMM patients had probability of death within 13, 85, and 376 months from the myelitis episode respectively. The same for WMM was 96, 256, 556 months respectively, implying that GMM has a worse survival outcome. Using linear regression, MRS at discharge and SLEDAI can predict survival status in GMM (R = 0.64, p = 0.04) but not in WMM (R = 0.42, p = 0.46). Conclusion Lupus myelitis has two distinct subtypes. GMM is associated with more active disease, poor survival, and significant disability, and its survival status can be predicted by MRS and SLEDAI scores. Disclosure R. Baisya: None. K. Yerram: None. G. Murthy: None. P.K. Devarasetti: None. L. Rajasekhar: None.

Persistent "MRI-negative" lupus myelitis-disease presentation, immunological profile and outcome.

Myelitis is the least common neuropsychiatric manifestation in systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI)-negative myelitis is even rarer. Here, we present the largest cohort of MRI-negative lupus myelitis cases to assess their clinical and immunological profiles and outcome. A single-center, observational study conducted over a period of 5 years (2017-2021) was undertaken to evaluate patients with MRI-negative lupus myelitis for the epidemiological, clinical, immunological, and radiological features at baseline and followed up at monthly intervals for a year, and the outcomes were documented. Among the 22 patients that presented with MRI-negative myelopathy (clinical features suggestive of myelopathy without signal changes on spinal-cord MRI [3Tesla], performed serially at the time of presentation and 7 days, 6 weeks, and 3 months after the onset of symptoms), 8 patients had SLE and were included as the study population. In 8 of 22 patients presenting with MRI-negative myelopathy, the etiology was SLE. MRI-negative lupus myelitis had a female preponderance (male: female ratio, 1:7). Mean age at onset of myelopathy was 30.0 ± 8.93 years, reaching nadir at 4.9 ± 4.39 weeks (Median, 3.0; range, 1.25-9.75). Clinically, cervical cord involvement was observed in 75% of patients, and 62.5% had selective tract involvement. The mean double stranded deoxyribonucleic acid, C3, and C4 titers at onset of myelopathy were 376.0 ± 342.88 IU/ml (median, 247.0), 46.1 ± 17.98 mg/dL (median, 47.5), and 7.3 ± 3.55 mg/dL (median, 9.0), respectively, with high SLE disease activity index 2,000 score of 20.6 ± 5.9. Anti-ribosomal P protein, anti-Smith antibody, and anti-ribonuclear protein positivity was observed in 87.5, 75, and 75% of the patients, respectively. On follow-up, improvement of myelopathic features with no or minimal deficit was observed in 5 of the 8 patients (62.5%). None of the patients had recurrence or new neurological deficit over 1-year follow-up. Persistently "MRI-negative" lupus myelitis presents with white matter dysfunction, often with selective tract involvement, in light of high disease activity, which follows a monophasic course with good responsiveness to immunosuppressive therapy. A meticulous clinical evaluation and a low index of suspicion can greatly aid in the diagnosis of this rare clinical condition in lupus.

Myeloradiculitis with lupus-like autoimmunity following Sinovac COVID-19 vaccination

We report a 67 year old man with features in keeping with transverse myelitis, lumbar root and cauda equina involvement suggestive of delayed autoimmune pathogenesis after the Sinovac COVID-19 vaccination previously observed in vaccines containing viral antigens. Grossly elevated antinuclear antibody and anti-double-stranded DNA are in line with observations in lupus myelitis despite the lack of systemic manifestations.

Spinal cord syndromes in patients with systemic lupus erythematosus: differentiating lupus myelitis, neuromyelitis optica, and multiple sclerosis.

Non-infectious myelitis in systemic lupus erythematosus (SLE) may be due to SLE myelitis, comorbid multiple sclerosis (MS), or neuromyelitis optica (NMO). We compared characteristics of these three conditions in SLE patients at a large academic institution. We searched for neurologic diagnoses of SLE myelitis, NMO myelitis, and MS myelitis among 2297 patients with at least four 1997 American College of Rheumatology revised criteria for SLE between 2000 and 2015. Each subject was reviewed by a neurologist to confirm the underlying neurologic diagnosis. Demographic, clinical, laboratory, and radiographic data were extracted and compared using Fisher's exact test, analysis of variance, and Wilcoxon rank-sum test. Fifteen of the 2297 subjects with SLE (0.7%) met criteria for a spinal cord syndrome: seven had SLE myelitis, three had AQP4 seropositive NMO, and five had MS. The median SLE Disease Activity Index 2000 score at time of neurologic syndrome presentation was higher in SLE myelitis subjects (8, interquartile range (IQR) 7-16) compared with subjects with NMO (6, IQR 0-14) or MS (2, IQR 0-4), p = 0.02. Subjects with SLE myelitis were also more likely to have elevated anti-dsDNA antibodies at presentation (86%) compared with subjects with NMO (33%) or MS (0%), p = 0.03. Myelitis occurs rarely among patients with SLE. Compared with subjects with SLE + NMO and subjects with SLE + MS, subjects with SLE myelitis had higher SLE disease activity at presentation.

Longitudinally Extensive Transverse Myelitis Associated With Systemic Lupus Erythematosus: A Case Report and Literature Review.

BackgroundLupus myelitis is a rare but disastrous complication of systemic lupus erythematosus (SLE). The transverse myelitis (TM) may involve three or more contiguous spinal cord segments and as such is designated longitudinally extensive transverse myelitis (LETM). The neurological presentation may vary based on the location of the pathology and may consist of a combination of sensory and motor deficits. TM could be the presenting feature of SLE or present after 10 years of disease, while SLE was considered to be in remission.Case presentation26-year-old Black man with history of biopsy proven-lupus nephritis that had progressed to ESRD, presented with sudden onset quadriplegia that resolved upon arrival to the hospital. On exam, the temperature was 101.8°F and the neurological exam was consistent with residual weakness on the left sided-upper and lower extremities. Leukopenia, lymphopenia and thrombocytopenia, along with low complements were noted. Brain MRI was normal however, the spine MRI was suspicious for an epidural process (C2-T4) and intravenous antibiotics were commenced. After five days, neurological improvement was nil and new spine MRI revealed spinal cord edema secondary to myelitis at several spinal cord levels (C2-T4). Laboratory data was consistent with a SLE flare complicated with longitudinal extensive transverse myelitis. Pulse steroids and plasma exchange were initiated. Two weeks after admission, MRI demonstrated resolution of the epidural spinal lesion and marked improvement in spinal cord edema.ConclusionTM can be the presenting feature of SLE or appear later on during the course of their disease. LETM is the most frequently type of TM found among SLE patients. Given the grave nature of the disease, it is of paramount importance that clinical features of TM be promptly recognized among SLE patients, to prevent catastrophic or even life-threatening outcomes.

Clinical value of autoantibodies for lupus myelitis and its subtypes: A systematic review.

We conducted a systematic review to investigate the clinical value of clinical characteristics and autoantibodies, especially lupus-specific antibodies, for lupus myelitis and its subtypes. We searched PubMed, EMBASE, and ICHUSHI without language restrictions for case reports or series of lupus myelitis. We focused on cases reported since 1997, when the revised classification criteria for systemic lupus erythematosus were published. Associations between patient characteristics including autoantibodies and functional outcome, survival, subtypes of myelitis (grey and white matter myelitis), and treatment were examined. We attempted to contact authors to supplement missing information for analysis. Our search identified 224 cases from 105 articles. White matter myelitis predicted favorable function (odds ratio = 15.18; 99% confidence interval, 3.09-151.31; p < 0.0001). Grey matter myelitis was associated with longitudinally extensive transverse myelitis (p < 0.001) and anti-double-stranded DNA (p = 0.003), and tended to be associated with anti-β2-glycoprotein I (p = 0.011). White matter myelitis tended to be associated with optic neuritis and anti-neuromyelitis optica antibodies. Although our study might be susceptible to under-reporting of original cases and selection bias, we aimed to provide a conservative interpretation by setting the statistical significance threshold at p < 0.01. This systematic review confirmed that grey matter myelitis predicted poor functional outcome and was associated with longitudinally extensive transverse myelitis and anti-double-stranded DNA antibodies. White matter myelitis was associated with favorable functional outcomes and may partially represent a complication of neuromyelitis optica.

A report of three cases with lupus myelitis.

Lupus myelitis (LM) is a rare but serious complication of systemic lupus erythematosus (SLE). In 2009, Birnbaum et al. suggested that LM could be classified into two subtypes, namely gray and white matter myelitis, based on neurological examination findings. Here we describe three cases of this disorder, one with signs of white matter dysfunction and two with signs of gray matter dysfunction. We discuss the potential role of autoantibodies in the development of LM.

Myelitis in systemic lupus erythematosus: clinical features, immunological profile and magnetic resonance imaging of five cases.

Myelopathy is one of the neuropsychiatric lupus syndromes. In this article, an original series of related lupus myelitis is reported and analyzed. We employed a retrospective chart review and identified all patients who were admitted to a general hospital in Buenos Aires, Argentina, with SLE and myelitis during the period 2007-2014. Five patients were observed, all women. The mean age was 25.4 years (19-39). In three of five cases, myelitis was one of the initial SLE manifestations. The SLE Disease Activity Index was variable (3/5 with high activity). Time to nadir ranged from 6 to 72 h. All had severe impairment, with motor deficit, sensory level and urinary retention. Magnetic resonance imaging was abnormal in all cases, 3/5 presented a longitudinally extensive myelitis. Serum analysis revealed positive antinuclear antibodies at a high titer in all patients, 4/5 had low complement levels and 3/5 had anti-phospholipids positive. The treatment (methylprednisolone and, in some cases, cyclophosphamide, anticoagulation and/or plasmapheresis) produced partial improvement or no benefits. One patient died due to sepsis. The others showed significant disability at 6 months (European Database for Multiple Sclerosis grading scale=6-8). In view of these results, myelitis associated with lupus shows heterogeneity of the clinical, radiological and serological features. In our experience, the cases were severe and with poor response to treatment. Further studies are required to understand this disease and establish a more efficient treatment.

Bulbar myelitis – As an initial presentation of NMO associated with SLE

Neuromyelitis optica (NMO) is a central nervous system inflammatory demyelinating autoimmune disease. It can be an isolated entity or can coexist other autoimmune diseases like systemic lupus erythematous. We report a 33-year-old female who presented with symptoms of bulbar myelitis. On evaluation, she was found to have leukopenia, positivity for antinuclear factor, anti-double-stranded DNA, Ro-52, and SS-A/SS-B antibodies. She did not respond to methylprednisolone, but improved after IVIg and cyclophosphamide. NMO antibody was also positive. There is a debate about the relationship of NMO with autoimmune disorders. In patients with uncharacterized lupus myelitis, doing NMO antibodies routinely will help in detecting more of NMO and increase our familiarity of the disorder and hence its association with lupus.

Clinical research in systemic lupus erythematosus: immediate relevance to clinical practice

Systemic lupus erythematosus remains a challenge because of its diverse presentations, variable natural history, and lack of uniform response to treatment. True remission is very rare. Reliance on corticosteroid treatment leads to unwanted long-term toxicity. Great advances have been made in the early detection of lupus nephritis and in treatment. Greater appreciation of cognitive impairment and of lupus myelitis is now possible. Pregnancy risks are better characterized. However, the greatest unmet challenge remains atherosclerosis.

Myelitis in Patients with Systemic Lupus Erythematosus

Objective: Myelitis is a rare but severe neurologic presentation of patients with systemic lupus erythematosus (SLE). Few large-scale retrospective cohort studies have been conducted, especially in Taiwan; therefore, we designed a retrospective study to investigate the disease parameters, treatment, and prognosis of myelitis in southern Taiwan. Methods: We reviewed medical records of patients with SLE who were evaluated at the Chang Gung Memorial Hospital-Kaohsiung Medical Center between January 1998 and January 2009. A total of 10 patients with myelopathy were included in the study. Neurologic variables and serologic features of SLE were assessed. Magnetic resonance images (MRI) of the spine and cerebrospinal fluid profiles were collected. We also analyzed the treatment and outcome of myelitis. Results: The cohort of 10 patients included 8 females (80%) and 2 males (20%). Three patients (30%) were initially admitted under the tentative impression of urinary tract infection (UTI). In 8 patients diagnosed with myelitis using MRI, 7 patients (88%) had increased T2 MRI signal intensity over the cervical and upper thoracic spinal area. Treatment regimens included conventional high-dose glucocorticoid, pulse methylprednisolone, cyclophosphamide, and plasma exchange. Six patients (60%) had a poor outcome. Additional cyclophosphamide was used in 5 patients and 3 of them (60%) had a good outcome. Conclusion: SLE patients, who contracted myelitis, presented with possible urinary difficulty and were admitted under the tentative impression of UTI. Besides, the most frequent site of lupus myelitis is the cervical to upper thoracic spinal area. Treatment with high-dose glucocorticoid and cyclophosphamide may be related to a better outcome compared to those without using cyclophosphamide.

Acute transverse myelitis and antiphospholipid antibodies in lupus. No evidence for anticoagulation

Current views suggest that prothrombotic properties of antiphospholipid antibodies (aPL) have a role in the development of acute transverse myelitis (ATM) in patients with systemic lupus erythematosus (SLE). Consequently, empiric anticoagulation may be included in these patients' treatment. We performed a systemic review of the literature to explore the clinical value of the presence of aPL in patients with lupus myelitis and the possible effectiveness of anticoagulation. We analyzed clinical and laboratory data extracted from published cases of SLE-associated ATM, fulfilling the Transverse Myelitis Consortium Working Group diagnostic criteria, that provided information on aPL. We report on a total of 70 patients. aPL, detected upon ATM onset in 54% of patients, neither predicted the involvement of the thoracic part of the spine, which has been postulated to reflect a predominantly thrombosis-induced injury, nor correlated with relapsing ATM, additional lupus CNS manifestations, or worse clinical outcome. An unfavorable outcome could be predicted by paralysis (P=0.02) and abnormal CSF findings at presentation (P=0.02). Whilst all patients received major immunosuppressive regimens, severe neurologic impairment (estimated Expanded Disability Status Scale score>7) was found primarily in aPL-negative patients (P=0.03). Anticoagulation was more frequently applied in aPL-positive patients (P=0.04), but any additional therapeutic effect was not evident. Detection of circulating aPL at ATM onset appears unreliable to suggest a thrombotic cause and perhaps not enough to dictate therapeutic anticoagulation. Registry creation of ATM in patients with SLE is needed to obtain more definite answers on the role of aPL in this condition.

Successful therapy with steroid and cyclophosphamide pulse for CNS lupus and lupus myelitis

The patient was a 33 year female. In 2001, she was diagnosed with systemic lupus erythematosus (SLE) and treated with prednisolone and ciclosporin. In May 2006, she noticed slight muscle weakness in the bilateral lower limbs. In July of the same year, she experienced gait difficulty and was admitted to our hospital because of fatigue, appetite loss, fever and disorientation. Soon afterwards, she had a fit of general convulsion and suffered from urinary retention and fecal incontinence. A brain magnetic resonance image revealed atrophy of the thoracic cord in T2 weighted images, and cerebrospinal fluid examination showed high total protein and interleukin-6 concentration, indicating complication of lupus myelitis as well as cerebral involvement. Steroid pulse and oral prednisolone treatment resulted in ameriolation of cerebral complications such as disorientation and convulsion, but muscle weakness and paresthesia in the lower limbs and urinary retention persisted. Cyclophosphamide pulse therapy was started and resulted in a marked recovery from muscle weakness, paresthesia and urinary retention, and she could discharge. We conclude that steroid and cyclophosphamide pulse therapy for a SLE patient with CNS lupus and lupus myelitisis is effective for ameriolation of symptoms such as disorientation, convulsion, urinary retension, fecal incontinence, muscle weakness and paresthesia in the lower limbs as well as elevated level of serum anti-ribosomal P antibody.

Lupus profond et myélopathie

We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the upper and lower limbs, suggestive of a medullary lesion. MRI disclosed an image of acute myelitis at the level of the second cervical vertebra. High doses of corticosteroids were promptly administered intravenously (methylprednisolone bolus) followed by oral prednisone and hydroxychloroquine. Six months later the patient was free of neurologic or cutaneous symptoms. In the literature, systemic signs are rarely associated with lupus profundus and myelitis has never been reported. Lupus myelitis is a vascular and/or demyelinating and usually segmental lesion of the spinal cord. Neurologic symptoms are those of acute rather than chronic or recurrent transverse myelitis. The prognosis is poor with frequent and severe functional sequelae. Treatment is mainly based on high-dose systemic corticotherapy alone or combined with cyclophosphamide. Our patient responded favourably to early methylprednisolone bolus followed by oral corticosteroids and antimalarial treatment. Lupus profundus is classically of benign course with only cutaneous involvement, but it may sometimes be a sign of systemic lupus and in rare cases may be associated with severe complications.

Successful treatment of intravenous cyclophosphamide pulse therapy for lupus myelitis with urinary disturbance and acute confusional state

A 18-year-old female had low grade fever, butterfly rush, proteinuria, leukocytopenia and hypocomplimentemia in 1988, and she was diagnosed as systemic lupus erythematosus (SLE) with lupus nephritis (WHOIIb). Treatments with prednisolone and mizoribine resulted in the remission for three years. In May 2001, she presented neurosis and polakisuria despite of the increase of prednisolone to 20 mg/day. Finally, she admitted in our hospital because of manic and repressive state and disorientaion. A brain MRI revealed high intensity lesions in bilateral basal ganglia in T2 weighted images, and cerebrospinal fluid showed elevated protein and IFN-alpha (421 IU/ml). In addition, she manifested neurogenic bladder, muscle weakness and hyperactive deep tendon reflex of bilateral lower limbs due to both supranuclear disorder and hypesthesia under the Th 10 level. Spinal MRI revealed marked atrophy and high intensity signals at the middle to lower thoracic spinal cord in T2 weighted images, indicating complication of lupus myelitis as well as cerebral involvement. Although the symptoms of CNS lupus did not respond to prednisolone, twelve monthly cyclophosphamide pulse therapy (IV-CY) has resolved urinary disturbance, muscle weakness and sensory loss, along with the improvement of both cerebral and spinal MRI images. Lupus myelitis and neurogenic bladder are the rare, but very refractory manifestation among CNS involvement of SLE. We here propose IV-CY as an invaluable choice for the treatment of not only active lupus myelitis but also neurogenic bladder resisted for steroid.

Spinal cord swelling and brain lesions in lupus myelitis

Spinal cord swelling and brain lesions in lupus myelitis