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Year : 2018  |  Volume : 2  |  Issue : 3  |  Page : 116-119

Transverse myelitis as presentation of systemic lupus erythematosus

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 Publication4-Oct-2018

Correspondence Address:
Dr. Khaled Alsaeiti
Rheumatology Unit, Aljumhoriya Hospital, Benghazi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/LJMS.LJMS_15_18

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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

How to cite this URL:
Ehsouna H, Alsaeiti K. Transverse myelitis as presentation of systemic lupus erythematosus. Libyan J Med Sci [serial online] 2018 [cited 2022 Oct 1];2:116-9. Available from: https://www.ljmsonline.com/text.asp?2018/2/3/116/242727

  Introduction Top

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.[1] 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.[2] Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself.[3] 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.[4]

The American College of Rheumatology (ACR) has formulated case definitions, reporting standards, and diagnostic testing recommendations for 19 neuropsychiatric SLE syndromes.[5] The frequency of neuropsychiatric symptoms appears to be increasing because of both better testing and increased clinician awareness.[6] 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.[6] 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 Top

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

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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 Top

Neuropsychiatric manifestations are not uncommon in SLE and the observed incidence has varied from 13% to 59%.[7],[8] 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.[9] 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.[10]

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.[11] 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.[12] While symptoms of TM may be the initial feature of SLE,[13] the onset usually coincides with other signs of active lupus, including optic neuritis.[14] 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.[15] 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.[16]

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,[17] but other series found no difference in the incidence of antiphospholipid antibodies in patients with or without TM.[18] Myelopathy can also be due to hematomas, tumors, fractures, disc herniations, infections, vascular occlusions, demyelinating conditions, or epidural abscesses.[19] 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.[16] 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.[14] Cerebrospinal fluid should be obtained to exclude infection; in SLE, there is typically an elevated protein level, a moderate lymphocytic pleocytosis,[20] and there are some reports about detection of the characteristic antibodies of SLE and probable evidence of intrathecal production of these antibodies.[21] Recurrence of myelopathy in particular within the 1st year is common, especially during steroid dose reduction.[14]

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.[14],[22]

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.[17] There are reports of cases where the disease is refractory or recurrent and plasmapheresis or intravenous immunoglobulins have been used with controversial results.[23] It is not well established yet, but some recommend the use of antithrombotic therapy in patients with TM and positive antiphospholipid antibodies.[11] There are reports in which rituximab has been used with apparent success.[24] 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.[25] 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.[26]

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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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.  Back to cited text no. 11
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