Transverse Myelitis Affecting More Than 4 Spinal Segments Associated with Systemic Lupus Erythematosus: Clinical, Immunological, and Radiological Characteristics of 22 Patients

doi:10.1016/j.semarthrit.2008.09.002

Seminars in Arthritis and Rheumatism

Volume 39, Issue 4, February 2010, Pages 246-256

https://doi.org/10.1016/j.semarthrit.2008.09.002 Get rights and content

Objective

To analyze the clinical and laboratory characteristics and outcomes of patients with transverse myelitis affecting more than 4 spinal segments secondary to systemic lupus erythematosus (SLE).

Methods

A computer-assisted (PubMed) search of the literature was performed to identify all cases of transverse myelitis affecting more than 4 spinal segments secondary to SLE from 1966 to April 2008. In addition, we present 2 previously unreported cases of SLE patients with transverse myelitis affecting more than 4 spinal segments.

Results

Twenty-two SLE patients with transverse myelitis affecting more than 4 spinal segments were finally reviewed. There were 17 (77%) females and the mean age at the diagnosis of myelitis was 29.3 ± 9.4 years (range, 12-53 years). It was the first manifestation of SLE in 5 (23%) patients. The most frequent clinical manifestations were sensory deficit in 20 (91%) patients, variable motor deficit in 19 (86%), and urinary sphincter dysfunction in 15 (83%) patients. On magnetic resonance imaging, all patients showed increased T2 signal intensity of the spinal cord, most frequently in the cervical to mid-lower thoracic spinal segments. Most patients received a combination of therapies; corticosteroids and cyclophosphamide was the most common (45%). Three patients (14%) had complete resolution of symptoms and 14 (59%) had partial recovery.

Conclusions

Transverse myelitis affecting more than 4 spinal segments is a rare complication in patients with SLE but may be the first clinical manifestation of the disease in some patients. A high proportion of affected patients have variable degrees of disability after treatment.

Section snippets

Methods

We systematically searched MEDLINE for English, French, and Spanish language articles published between 1966 and April 2008 using the terms Lupus, Longitudinal Myelitis, and the medical subject heading terms Myelitis and Systemic Lupus Erythematosus (singly and in combination) and scanned the bibliographies of all articles for references not identified in the initial search. Duplicate publications were excluded. Only cases with well-documented clinical summaries and relevant information were

Case 1

The patient was a girl diagnosed with SLE at 13 years of age based on photosensitivity, malar rash, hemolytic anemia, diffuse proliferative glomerulonephritis, and positive antinuclear and anti-dsDNA antibodies. She was treated with high-dose prednisolone and pulses of intravenous cyclophosphamide (Cyclo) with resolution of symptoms. Maintenance treatment was prednisone (10 mg/d), hydroxychloroquine (200 mg/d), and sodium mycophenolate (360 mg twice a day).

At 27 years of age, the patient was

Results

Twenty-two SLE patients with longitudinal myelitis were initially identified in the literature search. Two (9, 19) were discarded: 1 because the authors published the evolution of the same patient 2 years later (9, 20) and the other because the authors defined the neurological involvement as longitudinal myelitis but the patient presented with myelitis affecting less than 4 spinal segments (19). Therefore, 22 SLE patients (20 from the literature (5, 6, 7, 8, 10, 11, 12, 13, 14, 15, 18, 20) and

Discussion

Continuous transverse myelitis affecting more than 4 spinal segments is a rare, but serious, complication of SLE, leading to a variable degree of disability in more than 80% of patients. In general, 1/3 of patients with idiopathic acute transverse myelitis recover with little or no sequelae, 1/3 are left with a moderate degree of permanent disability, and 1/3 have severe disabilities (21). It seems that SLE patients with contiguous transverse myelitis affecting more than 4 spinal segments have

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Demyelinating and inflammatory myelopathies represent a group of diseases with characteristic patterns in neuroimaging and several differential diagnoses. The main imaging patterns of demyelinating myelopathies (multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, and myelin oligodendrocyte glycoprotein antibody-related disorder) and inflammatory myelopathies (systemic lupus erythematosus-myelitis, sarcoidosis-myelitis, Sjögren-myelitis, and Behçet's-myelitis) will be discussed in this article, highlighting key points to the differential diagnosis.
Transverse myelitis in systemic lupus erythematosus: A case report and systematic literature review
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Acute transverse myelitis (TM) is a rare complication secondary to systemic lupus erythematosus (SLE) that can cause patients' extensive and severe neuropsychiatric disorders. Due to the rarity of the onset of acute TM, there is still no standard treatment protocol. This study was to summarize the clinical features of SLE-TM through a case report and systematic review.
We report a case of acute TM with the initial symptoms of headache and fever on admission to hospital, with lesions in medulla oblongata, cervical medulla, and thoracic medulla. Furthermore, all cases of SLE combined with acute TM from January 1975 to February 2022 were concluded and reviewed to compare the disease's current treatment strategies and prognosis.
Patients with SLE-TM are mainly female (97.65%), with an average age of 36.89, a TM incidence of 24.51% and a longitudinal myelitis (LM) incidence of 67.76%. In addition, 68.63% of patients present an increased albumin, and only 16.50% of patients could recover. 32.35% of patients showed positive anti-cardiolipin antibody. Moreover, the patients who could recover are generally younger than those in the improved and paraparesis groups. After classifying the statistical results twice according to magnetic resonance imaging results and prognosis respectively, the erythrocyte sedimentation rate (ESR) in LM group was significantly higher than that in the other two groups. The positive rate of anti-DNA and anti-cardiolipin antibody (ANCL) in TM group was significantly higher than that of the other groups. According to the prognostic grouping, ESR in the recovery group was significantly higher than those in the other two groups. The positive ANCL in the poor prognosis group was slightly higher than that in the other two groups.
We offer a novel insight for this rare disease and hope to bring some inspiration the basic research for SLE-TM.
Relapse rates and risk factors for unfavorable neurological prognosis of transverse myelitis in systemic lupus erythematosus: A systematic review and meta-analysis
2022, Autoimmunity Reviews
Background: Transverse myelitis (TM) is a rare but severe systemic lupus erythematosus (SLE) manifestation.
To date, the prognostic factors for SLE-associated TM have been far less well-studied. There are also controversial data on the association of antiphospholipid antibodies (aPLs), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score, longitudinal extensive transverse myelitis (LETM), and decreased complement levels with the outcome of TM. We aimed to review the potential prognostic factors and integrate relapse rates of observational studies for SLE-associated TM.
Method: To review the prognosis for SLE-associated TM, relevant articles published up to July 30, 2021, were comprehensively and systematically identified from PubMed, EMBASE and Web of Science databases. Five studies encompassing 283 patients with SLE-related TM were included in this meta-analysis; raw data were obtained from three studies.
Results: The risk factors for unfavorable neurological outcome included demographic features, clinical characteristics, laboratory data, among which a grade of A, B or C on the American Spinal Injury Association Impairment Scale (AIS) at the onset of TM was associated with poor prognosis (OR: 56.05, 95% CI: 6.29–499.25, P < 0.001). The presence of hypoglycorrhachia was also correlated with a worse prognosis (OR: 10.78, 95% CI: 3.74–31.07, P < 0.001). No noticeable correlation was revealed between a poor outcome and positive aPLs and different aPLs profiles (anticardiolipin antibody [aCL], anti–β2-glycoprotein I (anti-β2GPI], lupus anticoagulant [LA]). The pooled 1-, 3- and 5-year relapse rates were 22% (95% CI: 0.13–0.31), 34% (95% CI: 0.22–0.47) and 36% (95% CI: 0.14–0.58), respectively. No significant publication bias was found.
Conclusion: A grade of A, B, or C on the AIS at initial TM and the presence of hypoglycorrhachia were found to be related to a worse prognosis in patients with SLE-associated TM. Notably, aPLs and different aPLs profiles may not suggest poor neurological outcome. The long-term relapse rate of patients with SLE-associated TM was relatively high. We recommend that treatment be stratified based on the initial severity of myelitis. For patients with severe myelitis, early intensive therapy may be initiated as soon as possible.
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Establishing differential diagnosis between different inflammatory causes of acute transverse myelitis (ATM) can be difficult. The objective of this study was to see which clinical, imaging or laboratory findings best contribute to confirm ATM etiology.
We reviewed clinical history, MRI images, CSF and serum laboratory tests in a retrospective study of patients presenting ATM. Univariate and multivariate multinomial logistic regression analysis was performed for each of the items listed above.
One hundred and seventy-two patients were analyzed in the study: 68 with multiple sclerosis (MS), 67 presenting idiopathic myelitis (IM; 23 of which were recurrent), 21 who developed positive systemic-antibodies associated myelitis (SAb-M) and 16 with neuromyelitis optica spectrum disorders (NMOSD). The following factors were associated with increased risk of developing MS: lower values in the modified Rankin scale at admission; positive oligoclonal bands (OCB); higher spinal cord lesion load; presence of brain demyelinating lesions; and disease recurrence. Longitudinally extended (LE) lesions, brain demyelinating lesions, and recurrences also contributed to final diagnosis of NMOSD. Multivariate multinomial logistic regression analysis showed presence of LE lesions increased risk of NMOSD and recurrence of ATM. Whereas, brain demyelinating lesions, and presence of OCB increased risk of MS.
ATM etiology may be clarified on the basis of spinal cord and brain MRI findings, together with CSF biochemistry and serum laboratory test results, allowing more timely and exact diagnosis as well as specific therapy for cases of uncertain origin.
Autoimmune and autoantibody-associated encephalomyelopathies
2019, The Autoimmune Diseases
Autoimmune central nervous system (CNS) affection comprises an expanding group of potentially treatable disorders that should be included in the differential diagnosis of any type of encephalitis or myelitis.
The extent of CNS involvement in systemic immunopathic disorders such as lupus erythematosus, rheumatoid arthritis (RA), or sarcoidosis has been recognized since long. However, the identification of underlying pathogenic mechanisms has led to the development of revolutionary antibody (Ab)-based therapies improving the prognosis of this group of patients.
Further advances in autoimmune involvement of the nervous system have led to the identification of new clinical syndromes, associated with antineuronal Abs that have transformed the diagnostic and therapeutic approach to these disorders. Starting with auto-Abs to the acetylcholine receptor for myasthenia gravis and against intracellular antineuronal nuclear Ab 1 (ANNA-1) (Hu) antigen, there is still a continuous expansion of the number of cell surface, synaptic, and intracellular molecules, which expose antigenic epitopes for autoimmune neurological disorders. Numerous of these Abs were associated with an extraneural malignancy, causing “paraneoplastic neurological syndromes,” while others occur as primary autoimmune diseases. Furthermore, identification of several Abs targeting glial antigens, such as aquaporin-4 (AQP4), has enabled the classification of these disorders as distinct clinical entities.
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: The authors have no conflicts of interest to disclose.

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Systemic lupus erythematosusTransverse Myelitis Affecting More Than 4 Spinal Segments Associated with Systemic Lupus Erythematosus: Clinical, Immunological, and Radiological Characteristics of 22 Patients

Objective

Methods

Results

Conclusions

Section snippets

Methods

Case 1

Results

Discussion

Am J Med Sci

Am J Med

Transfus Apher Sci

Kidney Int

Acute transverse myelitis: incidence and etiologic considerations

Neurology

Acute myelopathies: clinical, laboratory and outcome profiles in 79 cases

Brain

Proposed diagnostic criteria and nosology of acute transverse myelitis

Neurology

Lupus-related myelitis: serial MR findings

Am J Neuroradiol

Longitudinal involvement of the spinal cord in a patient with lupus related transverse myelitis

J Rheumatol

Systemic lupus erythematosus related transverse myelitis presenting longitudinal involvement of the spinal cord

Intern Med (Tokyo, Japan)

Longitudinal myelitis associated with systemic lupus erythematosus: clinical features and magnetic resonance imaging of six cases

Lupus

Development of severe longitudinal atrophy of thoracic spinal cord following lupus-related myelitis

Intern Med (Tokyo, Japan)

Recurrent longitudinal myelitis as primary manifestation of SLE

Neurology

Involvement of the entire spinal cord and medulla oblongata in acute catastrophic-onset transverse myelitis in SLE

Clin Rheumatol

A catastrophic-onset longitudinal myelitis accompanied by bilateral internuclear ophthalmoplegia in a patient with systemic lupus erythematosus

J Korean Med Sci

Longitudinal myelitis, aseptic meningitis, and conus medullaris infarction as presenting manifestations of pediatric systemic lupus erythematosus

Lupus

Marked inflammation in catastrophic longitudinal myelitis associated with systemic lupus erythematosus

Lupus

Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature

Ann Rheum Dis

Systemic lupus erythematosus
Transverse Myelitis Affecting More Than 4 Spinal Segments Associated with Systemic Lupus Erythematosus: Clinical, Immunological, and Radiological Characteristics of 22 Patients