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10.1093/ckj/sfac085

http://scihub22266oqcxt.onion/10.1093/ckj/sfac085
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36003672!9394706!36003672
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suck abstract from ncbi

pmid36003672      Clin+Kidney+J 2022 ; 15 (9): 1727-1736
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  • Glomerulonephritis with non-Randall-type, non-cryoglobulinaemic monoclonal immunoglobulin G deposits (PGNMID and ITG) #MMPMID36003672
  • Fourdinier O; Ulrich M; Karras A; Olagne J; Buob D; Audard V; Vigneau C; Gibier JB; Guerrot D; Massy Z; Vuiblet V; Rabot N; Goujon JM; Cordonnier C; Choukroun G; Titeca-Beauport D
  • Clin Kidney J 2022[Sep]; 15 (9): 1727-1736 PMID36003672show ga
  • 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) micromol/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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