• Nihal Martis, Matthieu Jamme, Corinne Bagnis-Isnard, Claire Pouteil-Noble, Claire Presne, Cécile Vigneau, Steven Grangé, Stéphane Burtey, Jean-Philippe Coindre, Alain Wynckel, Mohamed A Hamidou, Tarik Kanouni, Elie Azoulay, Miguel Hié, Dominique Chauveau, Agnès Veyradier, Eric Rondeau, Paul Coppo, and French Reference Centre for Thrombotic Microangiopathies.
• Internal Medicine Department, University Hospital of Nice; Côte d'Azur University, Nice, France; French Reference Centre for Thrombotic Microangiopathies, Saint-Antoine Hospital, Paris, France. Electronic address: martis.n@chu-nice.fr.
• Eur. J. Intern. Med. 2021 Nov 1; 93: 78-86.

ContextThe management of systemic auto-immune diseases (SAID) -associated thrombotic microangiopathies (TMA) [SAID-TMA] remains debated.ObjectivesTo provide a demographic, clinical and therapeutic picture of SAID-TMA.MethodsA cross-sectional analysis was conducted on adult patients presenting with SAID and TMA from the French National TMA Registry over a 20-year period. Clinical features were extracted and compared to those from a historical cohort of atypical haemolytic and uremic syndrome (aHUS) patients.ResultsForty-one patients with SAID-TMA were compared to 78 patients with aHUS from a historical cohort. Connective tissue diseases (CTD) were systemic lupus erythematosus (n=18), primary Sjögren's syndrome (n=7), systemic sclerosis (n=11), mixed CTD (n=2) and 2 cases of vasculitides, including 7 overlapping forms and 8 cases of primary antiphospholipid syndromes (APLS). Patients with SAID-TMA generally had pre-existing chronic kidney failure (OR= 3.17, 95%CI: 1.204 to 7.923; p= 0.016) compared to aHUS patients, though creatinine levels were significantly lower (216 [IQR, 108-334] µmol/L vs. 368 [IQR, 170-722] µmol/L; p= 0.002). Patients were less likely to recover if renal replacement therapy was needed at onset (OR= 0.07; 0.02 to 0.34; p <0.0005). Two patients died. Thirty patients responded to immunosuppressive treatment and complete remission was achieved in 25 cases. By contrast, therapeutic plasma exchange (TPE) did not have an early effect on TMA features at Day-7 nor Day-15 (p >0.05).ConclusionThe management of SAID-TMA implies an early initiation of immunosuppressive drugs for flares of the associated SAID, whereas TPE seem ineffective. KEY MESSAGES.Copyright © 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

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