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Glomerulonephritis with Non-Randall-type, Non-Cryoglobulinaemic Monoclonal Immunoglobulin G Deposits (PGNMID and ITG).

Ophélie Fourdinier 1, 2 Marc Ulrich 3 Alexandre Karras 4, 5 Jérôme Olagne 6 David Buob 7, 8 Vincent Audard 9 Cécile Vigneau 10, 11 Jean-Baptiste Gibier 12 Dominique Guerrot 13 Ziad Massy 14, 15, 16, 17, 18 Vincent Vuiblet 19, 20 Nolwenn Rabot 21 Jean-Michel Goujon 22, 23, 24 Carole Cordonnier 2 Gabriel Choukroun 2, 25 Dimitri Titeca-Beauport 2 
Abstract : BACKGROUND: Glomerulonephritis (GN) with non-Randall-type, non-cryoglobulinaemic monoclonal immunoglobulin G deposits encompasses rare diseases [proliferative GN with non-organized deposits (PGNMID) and immunotactoid GN] that cannot be distinguished without ultrastructural analysis by electron microscopy (EM). METHODS: Here, we report and analyse the prognosis of 41 EM-proven (PGNMID for 39/41) and 22 non-EM-proven/DNAJB9-negative cases, diagnosed between 2001 and 2019 in 12 French nephrology centres. RESULTS: Median (interquartile range) serum creatinine (SCr) at presentation was 150 (92-256) \textmu mol/L. The predominant histological pattern was membranoproliferative GN (79%), with IgG3 (74%) kappa (78%) deposits the most frequently observed. Disease presentation and patient management were similar between EM-proven and non-EM-proven cases. A serum monoclonal spike was detected for 21 patients and 10 had an underlying haematological malignancy. First-line therapy was mixed between clone-targeted therapy (n = 33), corticosteroids (n = 9) and RAAS inhibitors (n = 19). After 6 months, nine patients achieved complete and 23 partial renal recovery. In univariate analysis, renal recovery was associated with baseline SCr (odds ratio 0.70, P = 0.07). After a median follow-up of 52 (35-74) months, 38% of patients had progressed to end-stage kidney disease independently associated with baseline SCr [hazard ratio (HR) 1.41, P = 0.003] and glomerular crescentic proliferation (HR 4.38, P = 0.004). CONCLUSIONS: Our results confirm that non-cryoglobulinaemic and non-Randall GN with monoclonal IgG deposits are rarely associated with haematological malignancy. The prognosis is uncertain but may be improved by early introduction of a specific therapy.
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Soumis le : vendredi 2 septembre 2022 - 14:10:04
Dernière modification le : lundi 5 septembre 2022 - 09:58:19



Ophélie Fourdinier, Marc Ulrich, Alexandre Karras, Jérôme Olagne, David Buob, et al.. Glomerulonephritis with Non-Randall-type, Non-Cryoglobulinaemic Monoclonal Immunoglobulin G Deposits (PGNMID and ITG).. Clinical Kidney Journal, Oxford University Press, 2022, 15 (9), pp.1727--1736. ⟨10.1093/ckj/sfac085⟩. ⟨hal-03767975⟩



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