title: [Catastrophic antiphospholipid syndrome and rituximab: a new report].
abstract: BACKGROUND
  Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels. Early diagnosis and aggressive therapies are essential in this condition because of its extremely high mortality rate. Therapeutic management include heparine, high dose steroids, cyclophosphamide, plasma exchange, intravenous immunoglobuline, however a number of patients are refractory to treatment.
  
  
  OBJECTIVE
  We review and discuss alternative and emerging treatment options by rituximab for patients who fail or cannot tolerate conventional therapy.
  
  
  METHODS
  A 36-year-old female with a two mounths history of dyspnea, palpitation and chest pain was admitted. Physical examination upon admission revealed a fever, ischemic digital necrosis, scleroderma of the hands and beaking of the nose. Laboratory tests showed normal level of liver enzymes, elevation of creatinine level, lymphopenia, haemolytic anaemia with negative Coombs tests, low platelet count, prolonged partial thromboplastin time. The D-Dimer value was 158 ng/ml. Urinalysis revealed a proteinuria. Antinuclear antibody tests and lupus anticoagulant were strongly positive. Echocardiography revealed severe pulmonary hypertension and pericarditis. There was no pulmonary embolism on thoracic angio tomodensitometry. The diagnosis of catastrophic antiphospholipid antibody syndrome associated with systemic lupus and scleroderma was established. She was treated with anticoagulants, corticotherapy, one pulse of intravenous cyclophosphamide, 2 doses of intravenous immunoglobuline and 5 sessions of plasmapheresis. Because of lack of response 2 doses of 375 mg weekly rituximab i.v. were added but she developed pulmonary embolism, alveolar haemorrhage and she died.
  
  
  CONCLUSIONS
  Effectiveness of Rituximab for the CSAPL should be demonstrated by further studies.
authors.affiliation: Service de médecine interne, hôpital Habib Thameur, Tunis, Tunisie.
authors.forename: Sonia
authors.initials: S
authors.lastname: Ketari Jamoussi
authors.forename: Imène
authors.initials: I
authors.lastname: Zaghdoudi
authors.forename: Besma
authors.initials: B
authors.lastname: Ben Dhaou
authors.forename: Samir
authors.initials: S
authors.lastname: Kochbati
authors.forename: Khaoula
authors.initials: K
authors.lastname: Mir
authors.forename: Zeineb
authors.initials: Z
authors.lastname: Ben Ali
authors.forename: Fatma
authors.initials: F
authors.lastname: Boussema
authors.forename: Ouahida
authors.initials: O
authors.lastname: Cherif
authors.forename: Lilia Mâamar M
authors.initials: LM
authors.lastname: Rokbani
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab