Spectrum of Kidney Involvement in Patients with Myelodysplastic Syndromes

Nora Schwotzer (1) , François Provot (2) , Simon Ville (3) , Laurent Daniel (4, 5) , Awena Le Fur (6) , Sébastien Kissling (1) , Noémie Jourde-Chiche (4, 7) , Alexandre Karras (8) , Anne Moreau (3) , Jean-François Augusto (9) , Viviane Gnemmi (2) , Hélène Perrochia (10, 11) , Stanislas Bataille (4) , Moglie Le Quintrec (10, 12) , Jean-Michel Goujon (13) , Samuel Rotman (1) , Fadi Fakhouri (1)

1 CHUV - Centre Hospitalier Universitaire Vaudois = Lausanne University Hospital [Lausanne]
2 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
3 CHU Nantes - Centre Hospitalier Universitaire de Nantes
4 C2VN - Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research
5 TIMONE - Hôpital de la Timone [CHU - APHM]
6 CHD Vendée - Centre Hospitalier Départemental - Hôpital de La Roche-sur-Yon
7 Centre de néphrologie et transplantation rénale [Hôpital de la Conception - APHM]
8 HEGP - Hôpital Européen Georges Pompidou [APHP]
9 CHU Angers - Centre Hospitalier Universitaire d'Angers
10 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
11 UM - Université de Montpellier
12 Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB)
13 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital

Introduction: Myelodysplastic syndromes (MDS) are characterized by a high prevalence of associated autoimmune manifestations. Kidney involvement has been rarely reported in MDS patients. We report on the spectrum of kidney pathological findings in MDS patients. Methods: We retrospectively identified MDS patients who had undergone a kidney biopsy between 2001 and 2019 in nine Swiss and French nephrology centres. Results: Nineteen patients (median age 74 years [63-83]) were included. At the time of kidney biopsy, eleven (58%) patients had extra-renal auto-immune manifestations and sixteen (84%) presented with acute kidney injury. Median serum creatinine at diagnosis was 2.8 mg/dL [0.6-8.3] and median urinary protein to creatinine ratio was 1.2 g/g [0.2-11]. Acute tubulo-interstitial nephritis (TIN) was present in seven (37%) patients. Immunofluorescence study in one patient with acute TIN disclosed intense IgG deposits along the tubular basement membrane and Bowman's capsule. Other kidney pathological features included ANCA-negative pauci-immune necrotizing and crescentic glomerulonephritis (n = 3), membranous nephropathy (n = 2), IgA nephropathy (n = 1), IgA vasculitis (n = 1), immunoglobulin-associated membrano-proliferative glomerulonephritis type I (n=1), crescentic C3 glomerulopathy (n = 1), fibrillary glomerulonephritis (n = 1) and minimal change disease (n = 1). Eleven (58%) patients received immunosuppressive treatments, among whom one developed a severe infectious complication. After a median follow-up of 7 month [1-96], nine (47%) patients had chronic kidney disease stage 3 (n = 6) or 4 (n = 3) and five (26%) progressed to end-stage kidney disease. Three patients died. Conclusions: MDS are associated to several autoimmune kidney manifestations, predominantly acute TIN. MDS are to be listed among the potential causes of autoimmune TIN.

Mots clés: Autoimmunity; Acute tubulointerstitial nephritis; Myelodysplastic syndromes.

Domaines: Sciences du Vivant [q-bio]; Médecine humaine et pathologie; Urologie et Néphrologie

Nora Schwotzer, François Provot, Simon Ville, Laurent Daniel, Awena Le Fur, et al.. Spectrum of Kidney Involvement in Patients with Myelodysplastic Syndromes. Kidney International Reports, 2021, 6 (3), pp.746-754. ⟨10.1016/j.ekir.2020.12.030⟩. ⟨hal-03208678⟩