C C Mok1, C S Lau, E Y Chan, R W Wong. 1. Department of Medicine, Queen Mary Hospital, Pokfulam, Hong Kong, People's Republic of China.
Abstract
OBJECTIVE: Acute transverse myelopathy (ATM) is a rare manifestation of systemic lupus erythematosus (SLE). The pathogenesis is unclear and the optimal management strategy is uncertain because of the lack of controlled trials. In this study, the clinical presentation, autoantibody profile, treatment, and outcome of cases of ATM in our local SLE population were retrospectively analyzed and compared with SLE controls. RESULTS: Ten cases of ATM were identified among 315 patients with SLE studied, giving a prevalence of 3.2%. In 5 of the patients, ATM was the initial manifestation of SLE. The cervical cord was the most common site of involvement (50%). Cerebrospinal fluid abnormalities were present in 63% of the patients, while magnetic resonance imaging (MRI) of the spinal cord revealed abnormal T2 signals in 56%. Only one patient had lupus nephritis. ATM was not associated with antiribosomal P or anti-extractable nuclear antigen (anti-ENA) antibodies. Positive dsDNA antibody was present in 40% of the ATM cases, which was significantly lower than that of patients with active SLE without spinal cord disease (75%; p = 0.04). No significant differences in the prevalence of anticardiolipin antibodies and lupus anticoagulant between the ATM and the non-ATM group were observed. Only 3 patients with ATM showed hypocomplementemia or disease activity in other organs at the time of diagnosis. All the patients with ATM received corticosteroids, while 9 were given cytotoxic agents in addition. The response to treatment was variable -- 40% of patients had complete motor and sphincter recovery and 30% had mild residual spasticity of the lower limbs. CONCLUSION: In our SLE population, ATM was not associated with antiribosomal P, anti-ENA, or antiphospholipid antibodies. Systemic complement activation was not evident in most patients during the acute phase of myelitis. Early aggressive therapy using a combination of corticosteroid and cytotoxic agents is associated with a satisfactory outcome. Further prospective study is needed to delineate the best treatment and its efficacy in the prevention of relapses.
OBJECTIVE: Acute transverse myelopathy (ATM) is a rare manifestation of systemic lupus erythematosus (SLE). The pathogenesis is unclear and the optimal management strategy is uncertain because of the lack of controlled trials. In this study, the clinical presentation, autoantibody profile, treatment, and outcome of cases of ATM in our local SLE population were retrospectively analyzed and compared with SLE controls. RESULTS: Ten cases of ATM were identified among 315 patients with SLE studied, giving a prevalence of 3.2%. In 5 of the patients, ATM was the initial manifestation of SLE. The cervical cord was the most common site of involvement (50%). Cerebrospinal fluid abnormalities were present in 63% of the patients, while magnetic resonance imaging (MRI) of the spinal cord revealed abnormal T2 signals in 56%. Only one patient had lupus nephritis. ATM was not associated with antiribosomal P or anti-extractable nuclear antigen (anti-ENA) antibodies. Positive dsDNA antibody was present in 40% of the ATM cases, which was significantly lower than that of patients with active SLE without spinal cord disease (75%; p = 0.04). No significant differences in the prevalence of anticardiolipin antibodies and lupus anticoagulant between the ATM and the non-ATM group were observed. Only 3 patients with ATM showed hypocomplementemia or disease activity in other organs at the time of diagnosis. All the patients with ATM received corticosteroids, while 9 were given cytotoxic agents in addition. The response to treatment was variable -- 40% of patients had complete motor and sphincter recovery and 30% had mild residual spasticity of the lower limbs. CONCLUSION: In our SLE population, ATM was not associated with antiribosomal P, anti-ENA, or antiphospholipid antibodies. Systemic complement activation was not evident in most patients during the acute phase of myelitis. Early aggressive therapy using a combination of corticosteroid and cytotoxic agents is associated with a satisfactory outcome. Further prospective study is needed to delineate the best treatment and its efficacy in the prevention of relapses.
Authors: Briele Keiserman; Luiz Fernando Garcias da Silva; Mauro W Keiserman; Carlos Alberto von Mühlen; Henrique Luiz Staub Journal: Rheumatol Int Date: 2009-10-14 Impact factor: 2.631