Kidney involvement in myelodysplastic syndromes
Marie-Camille Lafargue, Jean-Paul Duong Van Huyen, Helmut G Rennke, Marie Essig, Mickaël Bobot, Noémie Jourde-Chiche, Hamza Sakhi, Alexandre KARRAS, Idris Boudhabhay, Philippe Brunet, Hugoline Boulay, Vincent Grobost, Carole Philipponnet, Juliette Jeannel, Jonathan Chemouny, Jean-Jacques Boffa, Dorra Braham-Stambouli, Umut Selamet, Leonardo V Riella, Olivier Fain, Lionel Adès, Pierre Fenaux, Camille Cohen, Arsène MekinianAbstract
Introduction
The objective of this study was to describe kidney involvement in patients with myelodysplastic syndrome (MDS), their treatments, and outcomes.
Methods
We conducted a multicenter retrospective study in seven centers, identifying MDS patients with acute kidney injury (AKI), chronic kidney disease (CKD), and urine abnormalities.
Results
Fifteen patients developed kidney disease 3 months after MDS diagnosis. Median urinary protein to creatinine ratio was 1.9 g/g, and median serum creatinine was 3.2 mg/dL. Ten patients had AKI at presentation, and 12 had extra-renal symptoms. The renal diagnoses included antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis, ANCA negative vasculitis, C3 glomerulonephritis, immune complex-mediated glomerulonephritis, polyarteritis nodosa, and IgA vasculitis. All patients but one received a specific treatment for the MDS-associated kidney injury. The effect of MDS treatment on kidney injury could be assessed in 6 patients treated with azacitidine, and renal function evolution was heterogenous. After a median follow-up of 14 months, 4 patients had CKD stage 3, 5CKD stage 4, 3 end stage kidney disease. On the other hand, 3 evolved to an acute myeloid leukemia and 3 died. Compared to 84 MDS controls, patients who had kidney involvement were younger, had a higher number of dysplasia lineages, and were more eligible to receive hypomethylating agents, but no survival difference was seen between the two groups. Compared to 265 AAV without MDS, the 7 MDS-associated pauci-immune vasculitis were older, ANCA serology was more frequently negative, and more cutaneous lesions were seen.
Conclusion
The spectrum of kidney injuries associated with MDS is mostly represented by vasculitis with glomerular involvement, and especially AAV.