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O25 Lupus podocytopathy: a rare form of lupus nephritis – an Italian retrospective multicenter study
  1. Grazia Dea Bonelli1,
  2. Savino Sciascia2,
  3. Marta Calatroni3,
  4. Vincenzo L’imperio4,
  5. Francesco Reggiani3,
  6. Roberta Fenoglio2,
  7. Lorenza Argolini5,
  8. Camillo Carrara6,
  9. Nicola Lepori7,
  10. Fausta Catapano8,
  11. Mariele Gatto9,
  12. Chiara Tani10,
  13. Domenico Santoro11,
  14. Maria Gerosa5,12,
  15. Marta Mosca10,
  16. Dario Roccatello2,
  17. Renato Alberto Sinico12 and
  18. Gabriella Moroni3
  1. 1University of Milano-Bicocca, Milan, Italy
  2. 2University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnet and RITA-ERN Member) CMID-Nephrology and Dialysis Unit, San Giovanni Bosco Hub Hospital, Dept. of Clinical and Biological Sciences, University of Turin, Turin, Italy
  3. 3Dept. of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy; Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy
  4. 4Dept. of Medicine and Surgery, Pathology, University of Milano-Bicocca, IRCCS (Scientific Institute for Research, Hospitalisation and Healthcare) Fondazione San Gerardo dei Tintori, Monza, Italy
  5. 5Division of Rheumatology, ASST Gaetano Pini, Milan, Italy
  6. 6Unit of Nephrology and Dialysis, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy
  7. 7Dept. of Medical Science and Public Health, University of Cagliari, Nephrology, San Michele Hospital, ARNAS G. Brotzu, Cagliari, Italy
  8. 8IRCCS Azienda Ospedaliero-Universitaria di Bologna. U.O. Nefrologia, Dialisi, Ipertensione, Bologna, Italy
  9. 9Unit of Rheumatology, Dept. of Medicine, University of Padua, Padua, Italy
  10. 10Rheumatology Unit, Dept. of Clinical and Experimental Medicine, University of Pisa, Italy
  11. 11Unit of Nephrology and Dialysis, Dept. of Clinical and Experimental Medicine, University of Messina, Messina, Italy
  12. 12Dept. of Clinical Sciences and Community Health, University of Milan, Italy
  13. 13Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy

Abstract

Objective To describe clinical-morphological features and outcomes of lupus podocytopathy (LP) and compare relapsing and non-relapsing (NR) forms.

Methods Inclusion criteria for LP were SLE diagnosis and: 1) ISN/RPS Class I or Class II at kidney biopsy (KB); 2) with or without mesangial immunoglobulin/complement deposition; (3) >70% foot processes effacement at electron microscopy (EM). In the absence of EM, presence of nephrotic syndrome and complete remission to corticosteroids are needed. Complete remission: normal kidney function, proteinuria<0.5g/day; partial remission: persistence of non-nephrotic proteinuria.

Results (tables 1 and 2) 26 patients (24 Females, median age 45 [16–53] years) were enrolled in this retrospective study. Nephrotic syndrome (84.6%), acute kidney injury (AKI) (38.5%) and arterial hypertension (41.7%) were the clinical presentations. Anti-DNA antibodies were positive in 62.5% of patients, C3 and C4 were low in 56% and 20% respectively. KB showed class I in 46.2%, class II in 53.8% and super-imposed focal segmental glomerulosclerosis in 15.4% of cases. 88.5% of patients received corticosteroids (oral or I.V.) associated with immunosuppressive drugs in 65.4% of cases. 24 patients achieved complete and 2 partial remission in a median of 4 (1–8.5) months. After 15 (range 3–65) months of remission, proteinuria (3.9[2.1–4.7]g/day) recurred in 7 patients (26.9%), all were re-treated and achieved remission again within 3 (1–10) months. The flare-free survival at 1,3 and 5 years were respectively 87.9%, 72.6% and 63.5%.

At LP diagnosis, relapsing patients in comparison to NR patients tended to be older (46vs39 year-old), have higher serum creatinine (1.2vs0.8mg/dl,p=0.1), lower eGFR (58vs91ml/min/1.73m2,p=0.07) less frequent anti-DNA positivity (42.8%vs70.6%,P=0.08) and more frequent cutaneous manifestations (57.1vs11.1%,p=0.03).

After a median follow-up of 59 (23–116) months, serum creatinine was 0.8 (0.64–0.92)mg/dl, eGFR was 91(83–103) ml/min/1.73m2 and 24h proteinuria was 0.18(0.15–0.44)g. Two patients ended-up with mild kidney dysfunction (eGFR 37 and 58ml/min/1.73m2).

Conclusions We showed that LP patients, despite minimal lesions at KB, presented clinical an immunologically active disease, supporting the hypothesis of LP being a distinct LN entity. Clinical manifestations rapidly responded to therapy but recurred in around 1/4 of patients. AKI and extrarenal SLE manifestations were more frequent in relapsing patients, suggesting that a more severe onset predisposed to recurrences.

View this table:
Abstract O25 Table 1

Clinical and laboratory characteristics of lupus podocytopathy patients at kidney biopsy

View this table:
Abstract O25 Table 2

Induction therapy at time of biopsy and clinical outcome

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ .

https://doi.org/10.1136/lupus-2024-el.35

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