Systemic autoimmune disorders associated with thrombotic microangiopathy: A cross-sectional analysis from the French National TMA registry: Systemic autoimmune disease-associated TMA.

CONTEXT: The management of systemic auto-immune diseases (SAID) -associated thrombotic microangiopathies (TMA) [SAID-TMA] remains debated. OBJECTIVES: To provide a demographic, clinical and therapeutic picture of SAID-TMA. METHODS: A 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. RESULTS: Forty-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). CONCLUSION: The management of SAID-TMA implies an early initiation of immunosuppressive drugs for flares of the associated SAID, whereas TPE seem ineffective. KEY MESSAGES.

Continuing education: preparing patients to choose a renal replacement therapy.

BACKGROUND: Patients with progressive chronic kidney disease face a series of treatment decisions that will impact the quality of life of themselves and their family. Renal replacement therapy option education (RRTOE), generally provided by nurses, is recommended by international guidelines OBJECTIVES: To provide nurses with advice and guidance on running RRTOE. DESIGN: A consensus conference. PARTICIPANTS: Four nurses, 5 nephrologists and 1 clinical psychologist (9 renal units; 6 European countries) from units that had extensive experience in RRTOE or were performing research in this field. APPROACH: Experts brainstormed and discussed quality standards for the education team, processes, content/topics, media/material/funding and quality measurements for RRTOE. RESULTS: Conclusions and recommendations from these discussions that are particularly pertinent to nurses are presented in this paper. CONCLUSIONS: Through careful planning and smooth interdisciplinary cooperation, it is possible to implement an education and support programme that helps patients choose a form of RRT that is most suited to their needs. This may result in benefits in quality of life and clinical outcomes. APPLICATION TO PRACTICE: There are large differences between renal units in terms of resources available and the demographics of the catchment area. Therefore, nurses should carefully consider how best to adapt the advice offered here to their own situation. Throughout this process, it is crucial to keep in mind the ultimate goal - providing patients with the knowledge and skill to make a modality choice that will enhance their quality of life to the greatest degree.