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We are presenting a case of TTP with undetectable levels of ADAMTS 13 in a 39-year-old woman. Diagnosis of systemic lupus was evoked in the setting of thrombotic microangiopathy. The patient presented normal renal function but important neurological impairment. Treatment with daily plasmapheresis as well as Rituximab, cyclophosphamide as steroids was required to achieve clinical improvement.

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BackgroundSystemic lupus erythematosus (SLE) is a multi systemic auto immune disease which can affect patients at any age.ObjectivesWe aimed to study influence of age onset in clinical and biological spectrum of SLEMethodsmedical records of 89 patients diagnosed as SLE according to the ACR criteria of 1997, between January 2004 and December 2016, were retrospectively analyzed. Patients were divided into 3 groups according to the age of onset: Juvenile onset patients (group 1) (G1) (≤16 years), Adult onset patients (group 2) (G2) (>16 and <50 years), Late onset patients (group 3) (G3) (≥50 years). Clinical and biological comparative study was conducted between the 3 groups. Data were analyzed by chi-square test and potentially associated factors were tested by binary logistic regression.Resultsamong the patients 11.2% are in G1, 75.3% in G2 and 13.5% in G3. Prevalence of SLE was higher in female than male (F/M=9/1) but predominance of women was lower in G1 (F/M=4/1) compared to G2 (F/M=10/1) and G3 (F/M=11/1). Patients in G3 had more hypertension (41.7%) compared to G2 (6%) (p=0.5) and G1 (0%) (p=0.04). Vespertilio erythema was less frequently found in G3 (33.3%) compared to G2 (64.2%) (p=0.045) and G1 (80%) (p=0.04). Anti Sm antibodies were more frequent in G1 (87.5%) compared to G2 (38.5%) (p=0.009) and G3 (18.2%) (p=0.003). Multivariate analysis showed that hypertension is significantly associated to late onset lupus (OR=29, 95% IC= [2.77 – 320], p=0.05) and anti Sm antibody is more frequent in juvenile onset patients (OR=12, 95% IC= [1.4- 117], p=0.024).Conclusionsaccording to our study, prevalence of lupus is higher in female regardless of age onset. Late onset lupus is associated to a high frequency of co morbidity while anti Sm antibody seems to be a hallmark of juvenile onset.Disclosure of InterestNone declared

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