BackgroundData from the PEXIVAS trial challenged the role of plasma exchange (PLEX) in ANCA-associated vasculitides (AAV). We aimed to describe kidney biopsy from patients with AAV treated with PLEX, evaluate whether histopathologic findings could predict kidney function, and identify which patients would most benefit from PLEX.MethodsWe performed a multicenter, retrospective study on 188 patients with AAV and AKI treated with PLEX and 237 not treated with PLEX. The primary outcome was mortality or KRT at 12 months (M12).ResultsNo significant benefit of PLEX for the primary outcome was found. To identify patients benefitting from PLEX, we developed a model predicting the average treatment effect of PLEX for an individual depending on covariables. Using the prediction model, 223 patients had a better predicted outcome with PLEX than without PLEX, and 177 of them had >5% increased predicted probability with PLEX compared with without PLEX of being alive and free from KRT at M12, which defined the PLEX-recommended group. Risk difference for death or KRT at M12 was significantly lower with PLEX in the PLEX-recommended group (−15.9%; 95% CI, −29.4 to −2.5) compared with the PLEX not recommended group (−4.8%; 95% CI, 14.9 to 5.3). Microscopic polyangiitis, MPO-ANCA, higher serum creatinine, crescentic and sclerotic classes, and higher Brix score were more frequent in the PLEX-recommended group. An easy to use score identified patients who would benefit from PLEX. The average treatment effect of PLEX for those with recommended treatment corresponded to an absolute risk reduction for death or KRT at M12 of 24.6%.ConclusionsPLEX was not associated with a better primary outcome in the whole study population, but we identified a subset of patients who could benefit from PLEX. However, these findings must be validated before utilized in clinical decision making.

Background Hereditary transthyretin amyloidosis (ATTRv) is a disabling and life-threatening disease that primarily affects the nervous system and heart. Its kidney involvement has not been systematically studied, particularly in non-V30M mutations, and is not well known to nephrologists. Material and Methods We conducted a retrospective study describing kidney phenotype of all prevalent patients with ATTR mutations, with neurological or cardiac involvement or presymptomatic carriers, followed-up in two university hospitals from the South of France, between June 2011 and June 2021. Results 103 patients were included, among whom 79 were symptomatic and 24 presymptomatic carriers. Patients carried 21 different ATTR mutations, and 54% carried the V30M mutation. After a mean follow up of 7.9 ± 25.7 years, 30.4% of the symptomatic patients had developed chronic kidney disease (CKD), and 20.3% had a urinary protein/creatinine ratio ≥ 0.5 g/g. None of the presymptomatic carriers had CKD or proteinuria. In a multivariate analysis, late onset of symptom (after 60 years), the V122I mutation, and proteinuria were significantly associated with CKD. Median CKD-free survival in symptomatic patients was estimated 81.0 [77.1; 84.9] years. It did not differ between V30M and non-V30M patients, but was lower in patients with the V122I mutation. The average age of CKD onset was 69.3 ± 13.0 years. In one 38-year-old V30M female, who presented a kidney-predominant phenotype, treatment with patisiran resulted in remission of the nephrotic syndrome. Conclusion CKD affects almost one third of patients with symptomatic ATTRv. The role of ATTRv per se in the development of CKD in this population remains to be determined, but some patients may benefit from specific therapies.

Background Rheopheresis is a double‐filtration plasmapheresis that removes high‐molecular‐weight molecules from the plasma and thereby lowers blood viscosity. This treatment has been proposed in hemodialysis (HD) patients for chronic limb‐threatening ischemia (CLTI), but very few studies have evaluated the usefulness of this technique. Principal Objective To assess 1‐year amputation‐free survival (AFS) of HD patients suffering from CLTI treated by rheopheresis. Material and Method We conducted a retrospective study of 28 consecutive HD patients treated by rheopheresis in three French dialysis centers between 1 February 2017 and 30 April 2019 in two indications resulting from CLTI, namely chronic ulceration or recent minor amputation with delayed healing. Results One‐year AFS rate reached 53.6 (−19.8; +16.3)%. One‐year overall survival rate reached 67.9 (−20.5; +13.1)%. Main causes of death were infections and related to palliative care implying reduction or withdrawal of regular dialysis treatment. Hypotension episodes were the main rheopheresis adverse events with a prevalence rate of 13.5%. Rheopheresis sessions significantly reduced fibrinogen, C‐reactive protein, α2‐macroglobulin, total cholesterol, low‐density lipoprotein cholesterol, high‐density lipoprotein cholesterol, triglycerides, IgM, and estimated plasma viscosity (P < .0001). Conclusion Rheopheresis may improve clinical outcomes of CLTI in HD patients. The assessment of rheopheresis effectiveness needs to be confirmed by a multicenter randomized controlled trial, such as the ongoing project in France (RHEO‐PAD, NCT: 03975946).

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BACKGROUND AND AIMS Hereditary transthyretin amyloidosis (ATTR) is a disabling and life-threatening disease that primarily affects the nervous system and heart. Its kidney involvement has been poorly studied and is not well known to nephrologists. METHOD We conducted a retrospective study describing kidney phenotype of all prevalent patients with ATTR mutations, with symptomatic amyloidosis or asymptomatic carriers, followed-up in two university hospitals from the South of France, between June 2011 and June 2021. RESULTS 103 patients were included, among whom 79 were symptomatic and 24 asymptomatic carriers, and 54% carried a V30M mutation. After a mean follow-up of 7.9 ± 25.7 years, 30.4% of the symptomatic patients had developed chronic kidney disease (CKD) and 20.7% had a urinary protein/creatinine ratio ≥0.5 g/g. None of the asymptomatic carriers had CKD or proteinuria. Median CKD-free survival in the global cohort was 81.0 years, and the average age of CKD onset was 69.3 ± 13.0 years. In a multivariate analysis, late onset of ATTR symptoms (after 60 years), the V122I mutation and proteinuria were significantly associated with CKD. In a 38-year-old V30M female, who presented a kidney-predominant phenotype, treatment with Patisiran resulted in remission of the nephrotic syndrome. CONCLUSION CKD affects almost one-third of patients with symptomatic ATTR amyloidosis. The role of ATTR amyloidosis per se in the development of CKD in this population remains to be determined, but some patients may benefit from specific therapies.

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