Full Papers
Lupus or not? Idiopathic full house glomerulonephritis: a rare nephropathy with unexpectedly severe outcome
S. Sciascia1, M. Cozzi2, A. Fadaeibahreini3, M. Radin4, I. Cecchi5, A. Barreca6, G. Amore7, E. De Simone8, R. Fenoglio9, D. Roccatello10
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy. savino.sciascia@unito.it
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- Division of Pathology, Città Della Salute e Della Scienza Hospital, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
- University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, ASL Città di Torino and Department of Clinical and Biological Sciences, University of Turin, Italy.
CER18414
Full Papers
PMID: 41004327 [PubMed]
Received: 05/12/2024
Accepted : 05/03/2025
In Press: 18/09/2025
Abstract
OBJECTIVES:
A full-house pattern at immunofluorescence in kidney biopsies is usually associated with lupus nephritis. The cases that do not meet criteria for diagnosis of systemic lupus erythematosus (SLE) and have no secondary causes are classified as idiopathic full house (non-Lupus) nephropathy (iFH-N), which is a poorly defined entity. We aimed to evaluate the clinical presentation, renal outcome and development of SLE in the long term.
METHODS:
We carried out a retrospective observational study from 2012 to 2022 on patients with iFH-N, i.e. having a full-house pattern at immunofluorescence, but not meeting the criteria for the diagnosis of SLE and without a secondary cause.
RESULTS:
Of 2210 patients, 91 presented with full-house pattern at immunofluorescence: 84 had the criteria for SLE diagnosis, 2 had secondary causes, 5 were idiopathic. iFH-N cases were all young females with histological pattern of membranous nephropathy and impaired kidney function, at presentation two had a nephrotic syndrome, three a nephrotic range proteinuria. Mean serum creatinine was 2.1 mg/dl (SD± 0.47), mean eGFR 35.2 ml/min/1.73m2 (SD±11), mean proteinuria 7.1 gr/24h (SD±3.2). Four had negative antinuclear antibodies; none had anti-dsDNA, anti-extractable nuclear antigens, antiphospholipid antibodies; three had low C3 levels. All received aggressive immunosuppression (IS), including steroids, cyclophosphamide, mycophenolate mofetil or Intensified B Cell Depletion Protocol. Mean follow-up was 7.4 year (SD ± 2.4). Four patients (80%) developed end stage renal disease, three within 24 months, one patient chronic kidney disease stage 4. One subject developed SLE after two years.
CONCLUSIONS:
All patients with iFH-N had similar clinical presentation, appeared to be refractory to aggressive IS, and had poor renal outcome.
