AI Article Synopsis

  • - Glomerulonephritis (GN) with non-Randall-type, non-cryoglobulinaemic IgG deposits includes rare forms like PGNMID and immunotactoid GN; differentiation requires electron microscopy (EM).
  • - A study analyzed 41 EM-confirmed GN cases and found that the majority had membranoproliferative GN with specific IgG3 deposits, while patient management strategies varied among cases.
  • - Over a median follow-up of 52 months, 38% of patients progressed to end-stage kidney disease; early specific therapies might improve prognosis despite uncertain outcomes and rare association with hematological malignancies.

Article 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) µmol/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 ( = 33), corticosteroids ( = 9) and RAAS inhibitors ( = 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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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9394706PMC
http://dx.doi.org/10.1093/ckj/sfac085DOI Listing

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