Journal Article

FRI0302 Systemic lupus erythematosus-associated thrombotic microangiopathies in 60 patients: clinical features, prognosis and treatment in a french multicentric cohort

Galicier, L., Deroux, A., Coppo, P., Amoura, Z., Pineton de Chambrun, M., Costedoat-Chalumeau, N., Roumier, M., Melki, I., Couture, P., Hié, M., Mathian, A., Kanouni, T.

2018

Overview

BackgroundThe occurrence of a TMA during SLE is a rare event that complicates 1% to 4% of lupus. The full spectrum of TMA can be encountered in the course of SLE: acquired thrombotic thrombocytopenic purpura (PTT), TMA associated with glomerulonephritis (TMA GN), atypical hemolytic uremic syndrome (aHUS), microangiopathic antiphospholipid syndrome (MAPS; defined or probable catastrophic antiphospholipid syndrome) or HELLP syndrome during pregnancy.ObjectivesTo describe the clinical phenotype of TMA in lupus, to specify the prognosis and to identify factors allowing early classification and management of the different subtypes.MethodsWe performed a French multicentric retrospective study from January 1987 to December 2017 in units of internal medicine, nephrology and ICU. Inclusion criteria associated SLE defined by the ACR and/or SLICC criteria and a TMA defined either by the presence of microangiopathic anaemia and peripheral thrombocytopenia, either by histological signs of TMA.Results68 events of TMA occurred in 60 SLE patients; median age was 24 years; 56 women (F/M ratio: 14). Diagnosis of SLE was assessed at a paediatric age in 24 (35.8%) patients. Clinical manifestations of SLE were: acute cutaneous lupus (n=48, 71%), arthritis (n=36, 53%), pleuritis (n=13, 19%), pericarditis (n=25, 37%), renal involvement (n=39, 57% including 20 class IV glomerulonephritis) and auto-immune cytopenia (n=22, 32%). Triggering factors of TMA were: lupus flare (n=38), infections (n=12), pregnancy and peripartum (n=3), or therapeutic rupture (n=4). Clinical and biological features at diagnosis of each subtypes of TMA are presented in table 1. A platelet count lower than 28 000/mm3 was predictive of TTP diagnosis (Se=90,5%, Sp=88,5%), likewise a creatinine plasma level greater than 100 µmol/L was predictive of TMA GN (Se=88,5%, Sp=85%). The treatment of TMA included (alone or in combination): corticosteroids in all cases, plasma exchange (n=52), cyclophosphamide (n=28), Rituximab (n=15), Eculizumab (n=2), antiplatelet agent (n=36), and/or effective anticoagulation (n=26). The median duration of follow-up was 150 months. Among TMA GN patients, the final median GFR was 58 mL/min (range: 0–120) with 5 individuals on chronic dialysis and 1 kidney transplant. Among TTP patients, one died from TMA, whereas the final median GFR of survivors (n=59) was 97 mL/min (range: 64–150), without any patient requiring dialysis.Table 1 Comparisons of clinical and laboratory data in different subtypes of TMA in SLEConclusionsSLE-associated TMA is a heterogeneous syndrome. TTP with a decreased ADAMTS13 activity and a low platelets level (<28000/mm3) have a good renal prognosis, whereas TMA GN with a high creatinine level (>100 µmol/L) have a poor renal prognosis. Early subtypes classification is mandatory for the clinician to provide prompt and appropriate management of this life-threatening complication.Disclosure of InterestNone declared

• Publisher: Elsevier Limited
• Subject: Antiphospholipid syndrome / Antiplatelet therapy / Arthritis / Classification / Corticosteroids / Creatinine / Cyclophosphamide / Diagnosis / Dialysis / Glomerulonephritis / Hemolytic uremic syndrome / Kidney transplantation / Lupus / Monoclonal antibodies / Nephrology / Patients / Pericarditis / Phenotypes / Platelets / Pleurisy / Pregnancy / Prognosis / Purpura / Rituximab / Systemic lupus erythematosus / Thrombocytopenia / Thrombocytopenic purpura / Thrombotic thrombocytopenic purpura