Nonlupus Full House Nephropathy

Key Points Nonlupus full house nephropathy is a rare, complex entity: confusion arises by the low-quality evidence and the lack of consensus on nomenclature. This systematic review supports that systemic lupus erythematosus and nonlupus full house nephropathy are distinct clinical entities, with comparable outcomes. The identification of three pathogenetic categories provides further clues for a shared clinical and diagnostic approach to the disease. Background The presence of a full house pattern at immunofluorescence on kidney biopsy in a patient without clinical and laboratory features of systemic lupus erythematosus (SLE) has led to the descriptive term nonlupus full house nephropathy. This systematic review and meta-analysis focus on nonlupus full house nephropathy nomenclature, clinical findings, and outcomes. Methods In a reiterative process, all identified terms for nonlupus full house nephropathy and other medical subject headings terms were searched in PubMed. Out of 344 results, 57 records published between 1982 and 2022 were included in the analysis. Clinical data of single patients from different reports were collected. Patients were classified into three pathogenetic categories, which were compared according to baseline characteristics, treatments, and outcomes. Results Out of the 57 records, 61% were case reports. Nonlupus full house nephropathy was addressed with 17 different names. We identified 148 patients: 75 (51%) were men; median age 35 (23–58) years. Serum creatinine and proteinuria at onset were 1.4 (0.8–2.5) mg/dl and 5.7 (2.7–8.8) g/d. About half of patients achieved complete response. A causative agent was identified in 51 patients (44%), mainly infectious (41%). Secondary nonlupus full house nephropathy was mostly nonrelapsing with worse kidney function at onset compared with idiopathic disease ( P = 0.001). Among the 57 patients (50%) with idiopathic nonlupus full house nephropathy, complete response was comparable between patients treated with immunosuppression and supportive therapy; however, proteinuria and creatinine at onset were higher in patients treated with immunosuppression ( P = 0.09 and P = 0.07). The remaining 7 patients (6%) developed SLE after a median follow-up of 5.0 (1.9–9.0) years. Conclusions Our data support that SLE and nonlupus full house nephropathy are distinct clinical entities, with comparable outcomes. A small subset of patients develops SLE during follow-up. Nonlupus full house nephropathy is addressed by many different names in the literature. The identification of three pathogenetic categories provides further clues for the management of the disease.