|Year : 2018 | Volume
| Issue : 3 | Page : 116-119
Transverse myelitis as presentation of systemic lupus erythematosus
Hamdi Ehsouna1, Khaled Alsaeiti2
1 Department of Internal Medicine, Faculty of Medicine, University of Benghazi, Benghazi, Libya
2 Department of Internal Medicine, Rheumatology Unit, Aljumhoriya Hospital, Benghazi, Libya
|Date of Web Publication||4-Oct-2018|
Dr. Khaled Alsaeiti
Rheumatology Unit, Aljumhoriya Hospital, Benghazi
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease. The clinical course of the disease is variable and is characterized by periods of relapses and remissions. The most common disease pattern is a mixture of nonspecific constitutional symptoms accompanied by various internal organ involvements. Peripheral neurologic syndrome and central nervous system manifestations are common in lupus patients but are not always attributable to lupus itself. In the present report, we present a case of transverse myelitis, a rare but serious condition reported in patients with SLE.
Keywords: CNS lupus, Systemic lupus erythematosus, transverse myelitis
|How to cite this article:|
Ehsouna H, Alsaeiti K. Transverse myelitis as presentation of systemic lupus erythematosus. Libyan J Med Sci 2018;2:116-9
| Introduction|| |
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease. Because of variable presentations and involvement of all the components of the immune system, the disease has been of interest to clinicians and immunologists. The clinical course of the disease is variable and is characterized by periods of relapses and remissions. The most common disease pattern is a mixture of nonspecific constitutional symptoms accompanied by various internal organ involvements. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. The wide range in reported prevalence reflects in part the use of different criteria for neuropsychiatric disease. Similarly, the reported frequencies of individual neurologic and psychiatric manifestations also vary widely, depending in part on the population studied, the case definition used, and methods used for screening.
The American College of Rheumatology (ACR) has formulated case definitions, reporting standards, and diagnostic testing recommendations for 19 neuropsychiatric SLE syndromes. The frequency of neuropsychiatric symptoms appears to be increasing because of both better testing and increased clinician awareness. The neuropsychiatric manifestations of SLE are varied and may be classified as primary neurologic and psychiatric disease (e.g., related to direct involvement of the neuropsychiatric system) or secondary disease (e.g., related to complications of the disease and its treatment). The latter are generally more common causes of neuropsychiatric symptoms and can be produced by a variety of mechanisms. We present a case of transverse myelitis (TM), a rare but serious condition reported in 1%–2% of patients with SLE. Although it is usually a late manifestation of SLE, it can rarely occur at presentation.
| Clinical Features|| |
A 42-year-old female presented to the neurology clinic as she was recently diagnosed as a case of neuromyelitis optica (NMO) (Devic&39;s disease); in review of her history, her current condition started 9 months back with vomiting, fever, skin rash, and edema of both lower limbs, and these symptoms lasted for 1 month and then resolved completely. Five months later, the patient developed difficulty in walking, paresthesia, and significant reduction of strength and sensitivity of pelvic limbs, which progressively involved the upper limbs. Throughout the course of her illness, she experienced constipation, urinary incontinence, dysphagia, dry mouth with recurrent mouth ulcers, diplopia, and burning sensation in the eyes with blurred vision. While on admission, the patient developed abnormal movements in the upper and lower limbs, which were diagnosed by the neurologist as dystonia.
The most relevant findings at her physical examination were as follows: blood pressure 100/60 mmHg, heart rate 85/min, temperature 36°C, respiratory rate (RR) 18/min, and body mass index 20 kg. She was conscious and oriented and cranial nerves appeared unaltered. Eye fundus examination revealed bilateral pale optic disc. Muscle strength and tendon reflex findings were as follows: in the upper limb: muscle strength was 2–3\5, biceps, ulnar and triceps reflexes were positive, negative Hoffman sign, with decreased fine and pin-prick sensation in the hands at glove distribution. Pelvic limbs showed muscle strength of 0–1\5. Patellar and Achilles reflexes were positive. The Babinski reflex was extensor. Superficial and deep sensitivity was abolished in the pelvic limbs. Other physical examination abnormalities were erythematous rash; focal alopecia; scaled scarred skin rash in the face; red ulcerated tongue; mild telangiectasia over the fingers; and tender joints of the hands, elbows, and knee with swollen metacarpophalangeal joints of the right hand.
The most relevant laboratory abnormalities were the presence of leukocytes in 21,300/mm hemoglobin at 11.8 g/dl with a mean corpuscular volume of 90, and a platelet count in 191,000/mm3. Serum electrolytes and liver function test were within normal levels. The erythrocyte sedimentation rate was 28 mm/h. The immunological laboratory resulted in positive antinuclear antibody (ANA) (1/80), positive anti-Ro52, positive scl-70, and positive anti-RNP\Sm. The cervical and thoracolumbar magnetic resonance imaging (MRI) in T2 sagittal sequence showed an abnormal longitudinal medullary signal predominant with lower cervical and less pronounced at the level of the dorsal bone marrow and at the height of D4/D5 with confluent focal cervical and dorsal hyperintensities, medullary hypertrophy, and a poorly limited contrast. It is associated with a probable left optical neuritis [Figure 1].
|Figure 1: (a) Magnetic resonance imaging in T2 sagittal cervical and thoracolumbar spine sequence showing an abnormal longitudinal signal predominant with lower cervical and less pronounced at the level of the dorsal bone marrow and at the height of D4/D5 with confluent focal cervical and dorsal hyperintensities, medullary hypertrophy, and a poorly limited contrast. It is associated with a probable left optical neuritis. (b) Magnetic resonance imaging|
Click here to view
The case was reviewed with a rheumatologist and a diagnosis of SLE with TM was confirmed, in account of oral ulcer, arthritis, CNS manifestations, positive ANA, and proteinuria, which are 5\11 according to the ACR criteria for diagnosis of SLE. The patient was treated with intravenous steroids and cyclophosphamide with favorable results.
| Discussion|| |
Neuropsychiatric manifestations are not uncommon in SLE and the observed incidence has varied from 13% to 59%., Acute TM is a complication which occurs following an inflammatory process associated with many etiologies. The prevalence in the general population has been reported in up to four or fewer cases per million people per year. Acute TM is not a complication associated only with SLE. It can occur in association with many diseases, including sarcoidosis, multiple sclerosis, and a variety of connective tissue disorders. It can be a paraneoplastic syndrome, and it has been reported in association with infectious diseases such as syphilis, tuberculosis, AIDS, herpes, and Mycoplasma pneumoniae, among others. It has been estimated recently that up to 10% of cases are cases of idiopathic acute TM.
Among the diseases of the connective tissue, SLE is probably the most associated with acute TM. It has been reported also in association with antiphospholipid antibody syndrome and there are some reports of association of TM with other connective tissue diseases but it is sporadic cases. An interesting fact is that patients with acute TM usually present with other neurological symptoms of SLE, such as depression, seizures, and psychosis, among others. There are even reports of acute TM preceded by aseptic meningitis, and a significant percentage of patients are associated with optic neuritis, which is known as Devic&39;s syndrome. While symptoms of TM may be the initial feature of SLE, the onset usually coincides with other signs of active lupus, including optic neuritis. This complication usually affects young women and is an expression that occurs early in the diagnosis of SLE, as in the case of our patient. There are even a high percentage of patients who present with this syndrome as the first manifestation of SLE, and nearly half of the patients have a recurrence of myelitis. There are reports of catastrophic presentations of TM with important involvement of both spinal cord and brain tissue at the level of the brainstem, associated with respiratory depression and poor prognosis.
It is not known with certainty the mechanism by which SLE can lead to affect the spinal cord in acute TM. There are some theories suggesting that this syndrome is due to an arteritis, with resultant ischemic necrosis of the spinal cord. It has been associated with antiphospholipid antibodies in some studies, but other series found no difference in the incidence of antiphospholipid antibodies in patients with or without TM. Myelopathy can also be due to hematomas, tumors, fractures, disc herniations, infections, vascular occlusions, demyelinating conditions, or epidural abscesses. The imaging study of choice for diagnosis of acute TM is MRI. It is reported that up to 30% of patients do not present a normal image of the spinal cord in MRI. MRI should be performed to exclude a compressive lesion from infection or another cause. Patients with TM and SLE may have localized edema along with T2 hyperintense foci on MRI scan. Cerebrospinal fluid should be obtained to exclude infection; in SLE, there is typically an elevated protein level, a moderate lymphocytic pleocytosis, and there are some reports about detection of the characteristic antibodies of SLE and probable evidence of intrathecal production of these antibodies. Recurrence of myelopathy in particular within the 1st year is common, especially during steroid dose reduction.
While NMO spectrum disorders (TM occurring with bilateral optic neuritis) with seropositive findings for NMO-IgG have been described as occurring in SLE and antiphospholipid syndrome, investigations suggest their presence to be an indication of coexisting NMO rather than a vasculopathic or other complication of SLE.,
There are no well-established recommendations regarding the treatment of choice for these patients. The use of steroids and cyclophosphamide is recommended as quickly as possible during the presentation of this complication because the best prognosis has been obtained from earlier therapy. There are reports of cases where the disease is refractory or recurrent and plasmapheresis or intravenous immunoglobulins have been used with controversial results. It is not well established yet, but some recommend the use of antithrombotic therapy in patients with TM and positive antiphospholipid antibodies. There are reports in which rituximab has been used with apparent success. On the other hand, there have been cases involving using other drugs such as mycophenolate mofetil with dexamethasone (cases of myelitis persistent and resistant to treatment) with almost complete relief. Intrathecal therapy with methotrexate and dexamethasone has been used successfully, and, interestingly, there are reports where bone marrow transplantation has been used, especially for recurrent and refractory cases with apparent success.
Among the many complications that can occur in patients with SLE, it is always important to consider the neurological manifestations due to their high prevalence. Some of these complications such as acute TM may not be common, but regained its importance due to damage, disability, and the potential consequences that may occur. Having in mind acute TM as a possibility in any patient with SLE, especially in the early diagnosis, allows us to be ready and able to diagnose and timely treat this complication early and avoid important sequels and poor prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med 2008;358:929-39.
Von Feldt JM. Systemic lupus erythematosus. Recognizing its various presentations. Postgrad Med 1995;97:79, 83, 86.
Lefèvre G, Zéphir H, Warembourg F, Michelin E, Pruvo JP, Hachulla E, et al.
Neuropsychiatric systemic lupus erythematosus (1st
part). Cases definitions and diagnosis and treatment of central nervous system and psychiatric manifestations of systemic lupus erythematosus. Rev Med Interne 2012;33:491-502.
Hanly JG. ACR classification criteria for systemic lupus erythematosus: Limitations and revisions to neuropsychiatric variables. Lupus 2004;13:861-4.
Liang MH, Corzillius M, Bae SC, Lew RA, Fortin PR, Gordon C, et al
. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthrit Rheum 1999;42:599-608.
Moore PM. Neuropsychiatric systemic lupus erythematosus. Stress, stroke, and seizures. Ann N Y Acad Sci 1997;823:1-7.
West SG, Emlen W, Wener MH, Kotzin BL. Neuropsychiatric lupus erythematosus: A 10-year prospective study on the value of diagnostic tests. Am J Med 1995;99:153-63.
West SG. Neuropsychiatric lupus. Rheum Dis Clin North Am 1994;20:129-58.
Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology 2002;59:499-505.
Krishnan C, Kaplin AI, Deshpande DM, Pardo CA, Kerr DA. Transverse myelitis: Pathogenesis, diagnosis and treatment. Front Biosci 2004;9:1483-99.
Sherer Y, Hassin S, Shoenfeld Y, Levy Y, Livneh A, Ohry A, et al.
Transverse myelitis in patients with antiphospholipid antibodies – The importance of early diagnosis and treatment. Clin Rheumatol 2002;21:207-10.
Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-15.
D&39;Cruz DP, Mellor-Pita S, Joven B, Sanna G, Allanson J, Taylor J, et al.
Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: Good functional outcome and relevance of antiphospholipid antibodies. J Rheumatol 2004;31:280-5.
Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, et al.
EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: Report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis 2010;69:2074-82.
Chan KF, Boey ML. Transverse myelopathy in SLE: Clinical features and functional outcomes. Lupus 1996;5:294-9.
Rheu CW, Lee SI, Yoo WH. A catastrophic-onset longitudinal myelitis accompanied by bilateral internuclear ophthalmoplegia in a patient with systemic lupus erythematosus. J Korean Med Sci 2005;20:1085-8.
Kovacs B, Lafferty TL, Brent LH, DeHoratius RJ. Transverse myelopathy in systemic lupus erythematosus: An analysis of 14 cases and review of the literature. Ann Rheum Dis 2000;59:120-4.
Mok CC, Lau CS, Chan EY, Wong RW. Acute transverse myelopathy in systemic lupus erythematosus: Clinical presentation, treatment, and outcome. J Rheumatol 1998;25:467-73.
Hanly JG, Liang MH. Cognitive disorders in systemic lupus erythematosus. Epidemiologic and clinical issues. Ann N
Y Acad Sci 1997;823:60-8.
Schur PH, Khoshbin S, editors. Neurologic Manifestations of Systemic Lupus Erythematosus. Available from: http://www.uptodate.com2012
[Last accessed on 2017 Jan 01].
Mevorach D, Raz E, Steiner I. Evidence for intrathecal synthesis of autoantibodies in systemic lupus erythematosus with neurological involvement. Lupus 1994;3:117-21.
Boumpas DT, Patronas NJ, Dalakas MC, Hakim CA, Klippel JH, Balow JE, et al.
Acute transverse myelitis in systemic lupus erythematosus: Magnetic resonance imaging and review of the literature. J Rheumatol 1990;17:89-92.
Propper DJ, Bucknall RC. Acute transverse myelopathy complicating systemic lupus erythematosus. Ann Rheum Dis 1989;48:512-5.
Armstrong DJ, McCarron MT, Wright GD. SLE-associated transverse myelitis successfully treated with rituximab (anti-CD20 monoclonal antibody). Rheumatol Int 2006;26:771-2.
Tomietto P, D&39;Agostini S, Annese V, De Vita S, Ferraccioli G. Mycophenolate mofetil and intravenous dexamethasone in the treatment of persistent lupus myelitis. J Rheumatol 2007;34:588-91.
Lehnhardt FG, Scheid C, Holtik U, Burghaus L, Neveling M, Impekoven P, et al.
Autologous blood stem cell transplantation in refractory systemic lupus erythematosus with recurrent longitudinal myelitis and cerebral infarction. Lupus 2006;15:240-3.