Haemorrhagic Lupus: A Diagnosis Emergency, Report about 6 Cases

Abstract

Introduction: Haemorrhagic manifestations rarely represent the mode of onset of lupus and are a therapeutical and diagnosis emergency. We report 6 cases of haemorrhagic manifestations disclosing lupus. Patients and methods: It’s about a retrospective, descriptive and multicentric study ranged from 1st January 2014 to 5th January 2019. Were included in the study all cases of lupic disease revealed by hemorrhagic disorders, recorded in 3 Internal Medicine Departments in Dakar: DalalJamm, Dantec, Pikine. The diagnosis of lupus was made on the basis of the 1997 modified classification criteria of ACR for SLE. Results: We gathered 6 cases of women affected by lupic disease. The mean age was 32 years old with extremes ranged from 17 to 56 years. According to haemorrhagic disorders, the diagnosis of lupus was automatically made in 5 of the cases and it took 6 months to confirm the last one. Haemorrhagic features were ruled by purpura (3/6 cases) and nose-bleeding associated with gumbleeding (2/6 cases), epistaxis and melena (1/6 case), hypermenorrhea (1/6 case), cerebral haematoma (1/6case). Cutaneous (5/6), articular (4/6), renal (2/6) and serous (2/6) disorders specially represented the other manifestations. Biological findings showed cytopenia with 3 cases of anaemia, 1 case of leucopenia and 6 cases of thrombopenia. The average of the platelet count was at 20,000 cells/mm3 with extremes ranged from 1000 to 35,000 cells/mm 3 . Thrombocytopenia was related to autoimmune thrombocytopenic purpura in 3 cases, to Evans’ syndrome in 2 cases, to macrophage activation syndrome in 1 case. Immunological investigations found the presence of antinuclear antibodies (1/6), anti-dsDNA (2/6) and anti-Sm (4/6). All patients received a bolus of Solumedrol followed by an administration of Prednisone at a dosage of 1.5 mg/kg (3 cases) and 1 mg/kg to the other patients. A transfusion of platelet concentrates was performed for all of them. The evolution was favorable in 4 cases. Two patients died according to a septic and haemorrhagic shock. Conclusion: We report 6 cases of haemorrhagic manifestations disclosing lupus. Cutaneomucous location was more frequent and was related to moderate to severe thrombocytopenia with the presence of anti-Sm antibodies.