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. 2021;38(3):e2021017.
doi: 10.36141/svdld.v38i3.11488. Epub 2021 Sep 30.

Renal involvement in sarcoidosis: histological patterns and prognosis, an Italian survey

Francesco Rastelli  1   2 , Ivano Baragetti  2 , Laura Buzzi  2 , Francesca Ferrario  2 , Luisa Benozzi  1 , Francesco Di Nardo  3 , Elisabetta Devoti  4 , Giovanni Cancarini  4 , Nicoletta Mezzina  5 , Pietro Napodano  5 , Maurizio Gallieni  6 , Domenico Santoro  6 , Michele Buemi  6 , Paola Pecchini  7 , Fabio Malberti  7 , Valeriana Colombo  8 , Giacomo Colussi  8 , Ettore Sabadini  9 , Giuseppe Remuzzi  9   10 , Lucia Argentiero  11 , Loreto Gesualdo  11 , Guido Gatti  12 , Francesco Trevisani  12 , Giorgio Slaviero  12 , Donatella Spotti  12 , Olga Baraldi  13 , Gaetano La Manna  13 , Eugenia Pignone  14 , Marco Saltarelli  14 , Marco Heidempergher  15 , Michela Tedesco  15 , Augusto Genderini  15 , Michela Ferro  16 , Cristiana Rollino  16 , Dario Roccatello  16 , Gabriella Guzzo  17 , Roberta Clari  17 , Giorgina Barbara Piccoli  17   18 , Cristina Comotti  19 , Giuliano Brunori  19 , Paolo Cameli  20 , Elena Bargagli  20 , Paola Rottoli  20 , Mauro Dugo  21 , Maria Cristina Maresca  21 , Massimo Bertoli  22 , Morena Giozzet  22 , Rachele Brugnano  23 , Emidio Giovanni Nunzi  23 , Marco D'Amico  24 , Claudio Minoretti  24 , Irene Acquistapace  25 , Carla Colturi  25 , Ernesto Minola  26 , Mario Camozzi  26 , Antonella Tosoni  27 , Manuela Nebuloni  27 , Franco Ferrario  28 , Giacomo Dell'Antonio  29 , Stefano Cusinato  1 , Sandro Feriozzi  30 , Claudio Pozzi  2
Affiliations

Renal involvement in sarcoidosis: histological patterns and prognosis, an Italian survey

Francesco Rastelli et al. Sarcoidosis Vasc Diffuse Lung Dis. 2021.

Abstract

Background: Granulomatous interstitial nephritis in sarcoidosis (sGIN) is generally clinically silent, but in <1% causes acute kidney injury (AKI).

Methods: This Italian multicentric retrospective study included 39 sarcoidosis-patients with renal involvement at renal biopsy: 31 sGIN-AKI, 5 with other patterns (No-sGIN-AKI), 3 with nephrotic proteinuria. We investigate the predictive value of clinical features, laboratory, radiological parameters and histological patterns regarding steroid response. Primary endpoint: incident chronic kidney disease (CKD) beyond the 1°follow-up (FU) year; secondary endpoint: response at 1°line steroid therapy; combined endpoint: the association of initial steroid response and outcome at the end of FU.

Results: Complete recovery in all 5 No-sGIN-AKI-patients, only in 45% (13/29) sGIN-AKI-patients (p=0.046) (one lost in follow-up, for another not available renal function after steroids). Nobody had not response. Primary endpoint of 22 sGIN-AKI subjects: 65% (13/20) starting with normal renal function developed CKD (2/22 had basal CKD; median FU 77 months, 15-300). Combined endpoint: 29% (6/21) had complete recovery and final normal renal function (one with renal relapse), 48% (10/21) had partial recovery and final CKD (3 with renal relapse, of whom one with basal CKD) (p=0.024). Acute onset and hypercalcaemia were associated to milder AKI and better recovery than subacute onset and patients without hypercalcaemia, women had better endpoints than men. Giant cells, severe interstitial infiltrate and interstitial fibrosis seemed negative predictors in terms of endpoints.

Conclusions: sGIN-AKI-patients with no complete recovery at 1°line steroid should be treated with other immunosuppressive to avoid CKD, in particular if males with subacute onset and III stage-not hypercalcaemic AKI.

Keywords: AKI; Acute onset; GenPhenReSa phenotypes; Giant cells; Hypercalcaemia; Interstitial fibrosis; Interstitial infiltrate; Renal sarcoidosis; Steroids; Subacute onset; sACE; sGIN.

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Conflict of interest statement

Special thanks to Jacqueline Rodriguez, who revised the manuscript for English language, and to Claudia Giuliani, for graphic support. We express gratitude to Immunopathology Group of Italian Society of Nephrology and to ACSI Onlus “Amici contro la Sarcoidosi Italia”, the Italian national society of Sarcoidosis patients.Francesco Rastelli and Ivano Baragetti were responsible for the work. Other authors contributed to the data collection and reviewed and revised the manuscript as supervisors.Each author declares that he or she has no commercial associations (e.g. consultancies, stock ownership, equity interest, patent/licensing arrangement etc.) that might pose a conflict of interest in connection with the submitted article.

Figures

Figure 1.
Figure 1.
A multinucleate giant cell in sGIN, contributed by Dr Irene Acquistapace.
Figure 2 -
Figure 2 -
Symptoms at exordium
Figure 3 -
Figure 3 -
A) Comorbidities. B) Basal comorbidities
Figure 4 -
Figure 4 -
Urinary sediment
Figure 5 -
Figure 5 -
Renal function
Figure 6 -
Figure 6 -
EndPoints in sGIN-AKI. A:: all sGIN-AKI pts basal NRF & > 1 year FU; B-G: subgroups of sGIN-AKI pts basal NRF & > 1 year FU in the following subgroups: , B: AKI without hypercalcemia, C: Giant Cells, D: Acute onset, E: Subacute onset, F: Severe Interstitial Infiltrate; G: Interstitial fibrosis.
See this image and copyright information in PMC

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