title: Thrombotic thrombocytopenic purpura and systemic lupus erythematosus: a rare and life-threatening association.
abstract: BACKGROUND
  The association between thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) is uncommon. Diagnosis is often difficult because of their clinical and biological similarities. The presence of TTP in SLE worsens the prognosis and causes high mortality in the absence of early therapeutic interventions.
  
  
  METHODS
  We report the case of a 20 year-old man, admitted with nephrotic range proteinuria, hematuria and rapidly progressive renal failure. He also had anemia, thrombocytopenia and pericardial effusion. The diagnosis of SLE was made based on these clinical findings along with positive antinuclear and anti dsDNA antibodies. Renal biopsy revealed class IV/ V lupus nephritis (LN) with active lesions of thrombotic microangiopathy. The evolution of neurological deficit, persistent thrombocytopenia and active microangiopathic changes suggested the diagnosis of associated TTP. The patient was treated initially with corticosteroids and cyclophosphamide. Plasmapheresis could only be started 16 days later. Mycophenolate mofetil and rituximab were successively tried in the absence of improvement in renal function and persistent thrombocytopenia. The patient's neurological condition deteriorated necessitating transfer to the intensive care unit and mechanical ventilation. There he developed pneumonia and died of septic shock two months after presentation.
  
  
  CONCLUSIONS
  The coexistence of TTP and SLE needs to be considered early in SLE patients with complicated course. It may not respond to the conventional immunosuppressive treatment of SLE.
authors.affiliation: Nephrology department, UHC of Casablanca, Morocco. selmasiham@yahoo.fr
authors.forename: Selma Siham
authors.initials: SS
authors.lastname: El Khayat
authors.forename: Ghislaine
authors.initials: G
authors.lastname: Medkouri
authors.forename: Armel Mbourou
authors.initials: AM
authors.lastname: Etomba
authors.forename: Mohamed
authors.initials: M
authors.lastname: Zamd
authors.forename: Mohamed Benghanem
authors.initials: MB
authors.lastname: Gharbi
authors.forename: Benyounes
authors.initials: B
authors.lastname: Ramadani
mesh_terms: D000328:Adult; D002493:Central Nervous System Diseases / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008181:Lupus Nephritis / Q000209:etiology / Q000473:pathology; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D055815:Young Adult
chemical_list: D007155:Immunologic Factors; D007166:Immunosuppressive Agents