{
  "abstract": "Objectives Lupus podocytopathy (LP) is an uncommon renal manifestation of systemic lupus erythematosus (SLE), representing less than 1% of cases. It is characterised by podocyte dysfunction without significant glomerular proliferation. Diagnosis requires nephrotic syndrome with biopsy showing minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangial proliferation, but without subendothelial or subepithelial immune deposits and with diffuse foot process effacement (>50%). Although recognised in KDIGO 2024, LP is not yet included in current EULAR/ACR or ISN/RPS classifications.The aim of this study was to describe the clinical, immunological, and histopathological characteristics of a multicentre Spanish cohort with biopsy-proven LP.Methods We conducted a retrospective, descriptive study of patients fulfilling 2019 EULAR/ACR SLE criteria and histologically confirmed LP from three Spanish centres. Clinical, biochemical, immunological, and histological data at diagnosis, as well as treatment response and relapses, were collected.Results Five patients (three women) were included, with a mean age of 49.4 years (range 37–62). All presented with nephrotic syndrome (mean proteinuria 6.2 g/24 h, serum albumin 1.89 g/dL) and microscopic haematuria. Renal function was preserved (mean creatinine 1.04 mg/dL), and two patients were hypertensive. All were ANA positive; three had anti-dsDNA antibodies, and none showed hypocomplementaemia. Biopsies revealed MCD without FSGS. Four patients achieved complete remission and one partial remission after corticosteroid therapy within six weeks. All relapsed early (<16 weeks) and required additional immunosuppressants: calcineurin inhibitors (3/5), mycophenolate (2/5), and belimumab (1/5). The most frequent extrarenal manifestations were arthritis (3/5), cytopenia (2/5), serositis (1/5), and malar rash (1/5).Abstract PO:04:107 Figure 1Conclusions LP was associated with positive ANA and frequent anti-dsDNA positivity but no hypocomplementaemia. MCD was the predominant histological pattern. All patients relapsed, highlighting the need to combine corticosteroids with other immunosuppressive agents (calcineurin inhibitors, B-cell–targeted or anti-BLyS therapies). A presentation of nephrotic syndrome, ANA positivity, and concomitant SLE features should prompt suspicion of LP. Its inclusion as a specific diagnostic criterion in future SLE classifications should be considered.",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital of León, León, Spain"
      ],
      "name": "Clara Moriano"
    },
    {
      "affiliations": [
        "Department of Nephrology, University Hospital of León, León, Spain"
      ],
      "name": "Elena Monfa Guix"
    },
    {
      "affiliations": [
        "Department of Nephrology, Clínica Universidad de Navarra, Pamplona, Spain"
      ],
      "name": "Jose Maria Mora Gutierrez"
    },
    {
      "affiliations": [
        "Department of Nephrology, Costa del Sol Hospital, Marbella, Spain"
      ],
      "name": "Nestor Oliva-Damaso"
    },
    {
      "affiliations": [
        "Department of Nephrology, Clínica Universidad de Navarra, Pamplona, Spain"
      ],
      "name": "Maria Loreto Fernanez Lorente"
    },
    {
      "affiliations": [
        "Department of Nephrology, University Hospital of León, León, Spain"
      ],
      "name": "Caterine Martinez Rosero"
    },
    {
      "affiliations": [
        "Department of Nephrology, University Hospital of León, León, Spain"
      ],
      "name": "Mario Prieto Velasco"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital of León, León, Spain"
      ],
      "name": "Elvira Diez Alvarez"
    }
  ],
  "title": "PO:04:107 Clinical, immunological, and histopathological features of lupus podocytopathy: a multicentre Spanish cohort",
  "uid": "a763381f-13a2-5e9e-9e61-d9e7c36cb37e"
}