Discussion
The most prevalent SLE neurological symptoms are seizures, psychosis, or headache. 1-2 percent of SLE patients’ Acute Transverse Myelitis (ATM) is seen, while in one adult study, up to 39 percent of subjects are presenting features (4, 10, 11). S. Zhang et al. (12) demonstrated that patients over the age of 40 years were more likely to have developed severe myelitis initially, explaining why younger individuals have a poor prognosis. Moreover, extensive spinal cord lesions, initial severe neurological impairment, delayed steroid impulse therapy, and hyper-inflammation could predict a poor prognosis of systemic lupus erythematosus with Transverse Myelitis. Meanwhile, another study shows that Transverse myelitis may present as a symptom of SLE or develop more than ten years after diagnosis of SLE. The primary prognostic factor is the severity of the initial neurological flare (with paraplegia). Thus, ATM signs and unexplained sensory complaints should elicit additional examinations in patients with SLE (13).
The majority of cases who develop ATM complications do so within five years after being diagnosed with SLE. The classical presentation of ATM may occur in SLE patients, such as sphincter disturbances, motor weakness, and sensory disturbance. Our patient did not show typical SLE or transverse myelitis symptoms. Our patient was diagnosed with SLE according to the presence of positive immunological markers and an atypical clinical presentation of longitudinal myelitis. Although the mechanism by which ATM causes SLE is unknown, it is most likely caused by immune complex-mediated thrombosis or vasculitis, which results in ischemic spinal lesions, or by anti-phospholipid antibodies cross-reacting with spinal cord phospholipids (11, 14). In SLE patients where ATM is presenting manifestation, numerous patients probably do not fulfill SLE diagnostic criteria; however, during the disease, they may develop further signs and symptoms of SLE in the future (4). Although the ANA and double-stranded DNA antibodies were positive in our reported case, the patient showed no additional clinical symptoms associated with SLE. The patient, after aggressive therapy, showed reasonable improvement.
Steroids in Intravenous pulse methylprednisolone and immunosuppressant such as cyclophosphamide are used in the general therapy strategy (10, 15). This combination of therapies appears to have a more favorable outcome. However, the prognosis is often seen as bad in patients with SLE. Plasmapheresis has been utilized in some patients, although its role is unclear (14). Although most SLE-associated ATM patients have satisfactory neurologic outcomes following high-dose corticosteroid therapy, some patients have a recurrence and permanent neurologic deficits (16).
In conclusion, this study shows that ATMs may, on rare occasions, represent the initial manifestation of SLE. ATM secondary to SLE, despite improved treatment methods, can have a bad prognosis and requires more than only steroids. Early detection and aggressive treatment can help prevent long-term irreparable damage and may positively result. This article underlines the importance of multicenter studies and establishing a registry for individuals with SLE who have ATMs to aid in the study of best management choices.
1. Yen EY, Singh RR. Brief Report: Lupus-An Unrecognized Leading Cause of Death in Young Females: A Population-Based Study Using Nationwide Death Certificates, 2000-2015. Arthritis Rheumatol. 2018;70(8):1251-5.
2. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365(22):2110-21.
3. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. 2008;358(9):929-39.
4. D’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(2):280-5.
5. Petty RE, Laxer RM, Lindsley CB, Wedderburn LR, Mellins E, Fuhlbrigge R. Textbook of pediatric rheumatology. 8. ed. Philadelphia: Elsevier, Inc; 2020. pages cm p.
6. Espinosa G, Mendizabal A, Minguez S, Ramo-Tello C, Capellades J, Olive A, et al. Transverse myelitis affecting more than 4 spinal segments associated with systemic lupus erythematosus: clinical, immunological, and radiological characteristics of 22 patients. Semin Arthritis Rheum. 2010;39(4):246-56.
7. Borchers AT, Gershwin ME. Transverse myelitis. Autoimmun Rev. 2012;11(3):231-48.
8. Transverse Myelitis Consortium Working G. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59(4):499-505.
9. Kerr DA, Ayetey H. Immunopathogenesis of acute transverse myelitis. Curr Opin Neurol. 2002;15(3):339-47.
10. Vieira JP, Ortet O, Barata D, Abranches M, Gomes JM. Lupus myelopathy in a child. Pediatric Neurology. 2002;27(4):303-6.
11. Avcin T, Benseler SM, Tyrrell PN, Cucnik S, Silverman ED. A followup study of antiphospholipid antibodies and associated neuropsychiatric manifestations in 137 children with systemic lupus erythematosus. Arthritis Rheum. 2008;59(2):206-13.
12. Zhang S, Wang Z, Zhao J, Wu DI, Li J, Wang Q, et al. Clinical features of transverse myelitis associated with systemic lupus erythematosus. Lupus. 2020;29(4):389-97.
13. Saison J, Costedoat-Chalumeau N, Maucort-Boulch D, Iwaz J, Marignier R, Cacoub P, et al. Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients. Lupus. 2015;24(1):74-81.
14. Al-Mayouf S, Bahabri S. Spinal cord involvement in pediatric systemic lupus erythematosus: case report and literature review. Clinical and experimental rheumatology. 1999;17(4):505-8.
15. Chen HC, Lai JH, Juan CJ, Kuo SY, Chen CH, Chang DM. Longitudinal myelitis as an initial manifestation of systemic lupus erythematosus. Am J Med Sci. 2004;327(2):105-8.
16. Ahn SM, Hong S, Lim DH, Ghang B, Kim YG, Lee CK, et al. Clinical features and prognoses of acute transverse myelitis in patients with systemic lupus erythematosus. Korean J Intern Med. 2019;34(2):442-51.