Abstract: TH-PO606
Non-Lupus Full House Nephropathy: A Debatable Entity
Session Information
- Glomerular Diseases: Clinical and Epidemiologic Studies
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Uzzo, Martina, Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy
- Kronbichler, Andreas, Department of Internal Medicine IV, Medical University, Innsbruck, Austria
- Alberici, Federico, Nephrology Unit, University of Brescia, Azienda Socio Sanitaria Territoriale Spedali Civili, Brescia, Italy
- Bajema, Ingeborg M., Department of Pathology and Medical Biology, University of Groningen, University Medical Center, Groningen, Groningen, Netherlands
Background
Lupus nephritis (LN) is characterized by a variety of light microscopic findings on kidney biopsy and a full house (FH) pattern at immunofluorescence/immunohistochemistry. A FH pattern and lesions consistent with LN, in a patient without clinical and laboratory features of SLE have led to the descriptive term non-lupus full house nephropathy (NLFHN). We performed a systematic review focussing on NLFHN nomenclature, clinical findings and outcome.
Methods
In a reiterative process, all identified terms for NLFHN and other MeSH terms were searched in PubMed. 346 records were screened according to inclusion and exclusion criteria. The 58 records identified were published between 1982 and 2022 and 60.3% were case reports. The available clinical data of patients from different reports were collected and analysed.
Results
NLFHN was addressed with 22 different names: the same name used by different authors could refer to different entities (Figure 1). We identified 148 patients: 75(50.7%) were males; median age 35(23-58) years. Creatinine and proteinuria at onset were 1.4(0.8-2.5) mg/dL and 5.7(2.7-8.8) g/day. Less than 1/3 of patients achieved complete renal response (CRR). A clear causative agent was identified in 50 patients, mainly infective (50%). Secondary NLFHN were acute, non-relapsing diseases with lower renal function at onset compared to the idiopathic ones (P=0.001). Among the 57 patients with idiopathic NLFHN, CRR was comparable between patients treated with immunosuppression (IS) and supportive therapy; however, proteinuria and creatinine at onset were higher in patients treated with IS (P=0.089 and P=0.066). Only 8 patients developed SLE after a median follow-up of 5.0(1.9-9.0) years.
Conclusion
Despite increasing interest, the place of NLFHN among glomerular diseases is still uncertain, which complicates treatment decisions. Confusion arises by the lack of high-quality evidence and the lack of consensus on nomenclature. The description of three pathogenic categories is a step forward towards a shared framework of this rare entity.