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Porto, Portugal - Researchers report that many patients are diagnosed with multiple sclerosis (MS) when in fact they may have systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). In a review article published in the April 2005 issue of Rheumatology, investigators say that this lapse could have an important effect on quality of life and mortality [ 1 ]. "As seems evident from the literature and from the everyday practice of those working with APS and SLE, a significant number of these patients are probably mislabeled as MS," comment the researchers, led by Dr Susana Ferreira (S Joao Hospital, Porto, Portugal). "Transverse myelitis and multifocal white-matter lesions seem to be the most common confounding factors."
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Ferreira and colleagues note that MS, SLE, and APS are chronic, immune-mediated, relapsing-remitting disorders affecting young adults--often women of childbearing age. Black and Japanese populations are rarely affected by MS, but, the researchers say, they have one of the highest incidences of SLE. The investigators point out that the pathogenesis of all 3 disorders remains largely unknown, and since neurological manifestations and magnetic resonance imaging (MRI) can be indistinguishable, there are no specific diagnostic tools. Yet, the team argues, the treatment and prognosis for these 3 disorders are quite different. They write, "A significant number of patients with APS and SLE are misdiagnosed as MS, but evidence suggests they are distinct nosological entities. It is essential to differentiate them, since APS may be responsive to anticoagulation."
When assessing MS, consider APS and SLE--especially if MS has atypical features
Ferreira and her group describe MS as the most common cause of neurological disability in young adults. They note that the course and prognosis are variable and there is no definitive long-term treatment. They estimate that about 50% of patients will need help with walking 15 years after diagnosis and 70% will have secondary progression despite treatment. The researchers explain that there is controversy about the prevalence and significance of antiphospholipid antibodies (aPL) in MS. "When assessing MS patients, clinicians should consider APS and SLE, especially if the MS has atypical features. A trial of anticoagulation might be worthwhile in some patients with atypical MS and consistently positive aPL."
A significant number of patients with APS and SLE are misdiagnosed as MS, but evidence suggests they are distinct nosological entities. It is essential to differentiate them, since APS may be responsive to anticoagulation.
The investigators describe SLE as a multisystem disease, where central nervous system involvement occurs in about 50% of patients and carries a poor prognosis. They note that a wide spectrum of neurological and psychiatric features is recognized. The researchers add that demyelinating syndromes resembling MS such as lupoid sclerosis are a rare feature of SLE and are a diagnostic challenge. They point out that the pathophysiology of central nervous system lupus remains poorly understood, but there is increasing evidence that it is often associated with aPL and only rarely caused by vasculitis. They write, "This is particularly true for myelopathy." Combined therapy with immunosuppression and anticoagulation is often required. Recent studies have also shown a reduction in mortality in SLE. The researchers hypothesize that this is due to earlier recognition and treatment.
Ferreira and colleagues explain that APS is characterized by thrombosis (venous or arterial) or pregnancy morbidity in the presence of persistent aPL. They note that although only stroke and transient ischemic attack are included in the classification criteria, more complex neurological manifestations are being recognized, and multifocal white-matter lesions on brain MRI are common. The researchers say that APS can occur on its own (primary APS; 53%) or in association with other diseases (secondary APS)--most commonly SLE (36%).
Consistently positive aPL might suggest a different treatment approach
The investigators argue that despite discordance in the literature, because APS is a treatable disease and aPL screening is a noninvasive, widely available, and inexpensive diagnostic test, it seems reasonable to consider it in patients with MS--particularly in those with atypical features. They write, "Repeated negative results make a diagnosis of APS unlikely. Consistently positive aPL might suggest a different treatment approach." Ferreira and her team point out that primary APS is being increasingly recognized and treated, and even healthy individuals with aPL are now regarded as a population at risk. "Treatment definitely improves prognosis," comment the researchers.
They suggest that when dealing with patients with MS or MS-like illnesses, APS and SLE should be considered in the differential diagnosis. They call for a careful medical history looking for manifestations of APS or connective-tissue diseases such as previous thrombosis, recurrent miscarriages, livedo reticularis, thrombocytopenia, Raynaud's phenomenon, photosensitivity, rash, arthralgias, and sicca syndrome.
Ferreira and colleagues argue that antiphospholipid antibodies such as aCL, IgM and IgG should be considered. They note that in some patients, an accurate diagnosis may emerge only after long-term follow-up. If there is persistent aPL positivity or there are features suggesting primary or secondary APS, the researchers recommend that a trial of oral anticoagulation with a target international normalized ratio (INR) of 3 to 4 for 6 months be considered with careful patient counseling of the risks.
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