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10-Dec-2008
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Arch Hellen Med, 25(5), September-October 2008, 636-641 SHORT COMMUNICATION Lupoid sclerosis (multiple sclerosis with systemic lupus erythematosus). I. ALEXIOU,1 A. BOUBOUGIANNI,1 P. ATHANASSIOU,1 I. MYRIOKEFALITAKIS,1 C. ANTONIADES,1 C. PARASKEVAIDIS,2 G. VEZYROGLOU1 |
Two cases are described of a rare syndrome, defined as lupoid sclerosis, which refers to patients fulfilling ACR criteria for systemic lupus erythematosus (SLE), who develop, in the course of their disease, the clinical picture of multiple sclerosis (MS). Case 1: A 56 year-old woman, with a history of a transient polyarthritis in 1990, presented with malaise, arthralgia and myalgia in 1995. She had ANA, thrombocytopenia, leukocytopenia, low C4 levels and typical LB-test. Despite many therapeutic trials with corticosteroids, hydroxychlorokine and cyclophosphamide, the patient developed transient visual loss, tremor, weakness of upper and lower extremities, muscular hypertonia, paresthesia, urinary incontinence and diplopia, within three years (1996-1998). Several symptoms subsequently improved. Brain MRI showed multiple, widespread T2 hypersignals in the central and the deep periventricular white matter, in 1997. Case 2: A 32 year-old woman presented with fever and sensimotor deficit of several peripheral nerves in 1984. She had ANA, anti-dsDNA and low CH50. Brain CT showed no evidences of a pathologic process. In spite of many therapeutic trials with corticosteroids and azathioprine, in 1988 she presented glomerulonephritis with hypertension and had a stroke in 1990. A renal biopsy in 1991 showed evidence of glomerulosclerosis and interstitial nephritis. The patient presented with urinary incontinence in 1993, and right hemiplegia, unsteadiness, diplopia, oligoarthritis and rash in 1998. Brain MRI showed multiple foci of T2 hypersignals in the pons, basal ganglia, periventricular white matter and in the corpus callosum. These cases could be an example of initial SLE followed by clinical and MRI findings of CNS demyelination. However, one cannot exclude the probability of the coexistence of two diseases (SLE and MS) with shared symptoms and findings.
Key words: CNS, Lupoid sclerosis, Multiple sclerosis, SLE.