The frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

The role of repeat kidney biopsy in lupus nephritis with renal remission is unclear. The aim of this study is to assess this role in a real-life scenario. This retrospective, single-centre study included 56 patients with lupus nephritis diagnosed from 1998 to 2019, with an initial kidney biopsy (KB1) at the onset of lupus nephritis and a second kidney biopsy (KB2) after achieving renal remission. Fifty-one (91.1%) patients were women with a median age of 29.9 [23.4–40.6] years at the time of lupus nephritis diagnosis. KB2 were performed after 41.1 [30.1–52.5] months of KB1. At the time of KB2, complete renal response was achieved in 51 (91.1%) patients. Median activity index decreased from a baseline value of 6.5 [2.8–11] to 0 [0–2] (p < 0.001). Chronicity index worsened from 1 [0–2] to 2 [1–3] (p = 0.01). In patients with proliferative/mixed forms at KB2, chronicity index median value increased to 3 [1.5–4], as well as interstitial fibrosis and tubular atrophy $\ge $ 25%, from 5.4% to 13.5%. Persistent histological active lupus nephritis (activity index ≥ 2) was presented in 11 (19.6%) of KB2. There were no differences when comparing immunological parameters between both groups (activity index ≥ 2 versus < 2) at KB2, nor in the percentage of patients who presented renal flare. Immunosuppressive treatment was withdrawn in 35 (62.5%) patients and maintained/switch in 21 (37.5%). Afterwards, new renal flare occurred in 9 patients per group (25.7% and 43%, respectively), after a median time of 39 [6.5–55] months and 7 [6–30] months, respectively. There was no difference in the number of patients who developed chronic kidney disease (n = 14, 25%) according to the treatment. In conclusion, KB2 provides valuable information to guide the immunosuppressive maintenance therapy.

Teixeira da Silva et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

No abstract