Nasr SH, Sirac C, Leung N, Bridoux F. Monoclonal immunoglobulin crystalline nephropathies. Kidney Int. 2024 Aug:S0085-2538(24)00319-3. [PubMed link]
Monoclonal Ig crystalline nephropathies are rare lesions resulting from precipitation of monoclonal Igs in the kidney as crystalline inclusions. They can be categorized into lesions with predominant intracellular crystals (light chain [LC] proximal tubulopathy, LC crystal-storing histiocytosis, and LC crystalline podocytopathy) and lesions with predominant extracellular crystals (crystalglobulin-induced nephropathy and crystalline variant of LC cast nephropathy). A review.

Hengel FE, Dehde S, Lassé M, Zahner G, Seifert L, Schnarre A, et al; International Society of Glomerular Disease. Autoantibodies Targeting Nephrin in Podocytopathies. N Engl J Med. 2024 Aug 1;391(5):422-433. [PubMed link]
The authors report that 46 of 105 patients (44%) with minimal change disease and 7 of 74 patients (9%) with primary FSGS had antinephrin autoantibodies; among patients with active disease who had not received immunosuppressive treatment, the percentages were 69% and 90%, respectively.

Reiser J, Ingelfinger JR. Kidney Disease and Antinephrin Antibodies. N Engl J Med. 2024 Aug 1;391(5):463-467. [PubMed link]
An editorial on the previous article.

Vaulet T, Callemeyn J, Lamarthée B, Antoranz A, Debyser T, Koshy P, Anglicheau D, Colpaert J, Gwinner W, Halloran PF, Kuypers D, Tinel C, Van Craenenbroeck A, Van Loon E, Marquet P, Bosisio F, Naesens M. The Clinical Relevance of the Infiltrating Immune Cell Composition in Kidney Transplant Rejection. J Am Soc Nephrol. 2024 Jul 1;35(7):886-900. [PubMed link]
Banff-defined rejection was related to high presence of CD8+ effector T cells, natural killer cells, monocytes/macrophages, and, to a lesser extent, B cells, whereas CD4+ memory T cells were lower in rejection compared with no rejection. Although some specific cell types partly discriminated between rejection phenotypes, the overall estimated immune cell composition of kidney transplants was ill-related to main Banff-defined rejection categories.

Wolf MTF, Bonsib SM, Larsen CP, Hildebrandt F. Nephronophthisis: a pathological and genetic perspective. Pediatr Nephrol. 2024 Jul;39(7):1977-2000. [PubMed link]
Nephronophthisis (NPHP) is an autosomal recessive cystic kidney disease and is one of the most frequent genetic causes for kidney failure (KF) in children and adolescents. The involvement of additional organ systems in syndromic forms of NPHP is explained by shared expression of most NPHP gene products in centrosomes and primary cilia, a sensory organelle present in most mammalian cells. This finding resulted in the classification of NPHP as a ciliopathy. A review.

Jabbour R, Heinzel A, Reindl-Schwaighofer R, Gregorich MG, Regele H, Kozakowski N, Kläger J, Fischer G, Kainz A, Becker JU, Wiebe C, Oberbauer R. Early progression of chronic histologic lesions in kidney transplant biopsies is not associated with HLA histocompatibility. Nephrol Dial Transplant. 2024 May;39(5):808-817. [PubMed link]
More than one-third of patients exhibited a progression of chronic lesion scores by at least one Banff grade in tubular atrophy (ct), interstitial fibrosis (ci), arteriolar hyalinosis (ah) and inflammation in the area of interstitial fibrosis and tubular atrophy (i-IFTA) from the 3- to the 12-month biopsy. There was no association of HLA mismatch scores with progression of histologic lesions.

Verbinnen M, Sprangers B, Abrahams AC, Koshy P, Van Kruijsdijk RCM, Philipse E, Michalak M, Delforge M, Vos JMI, Wetzels J, Dendooven A, Van Craenenbroeck AH. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits in the native or posttransplant kidney. Nephrol Dial Transplant. 2024 May 26;39(5):888-892.
A retrospective analysis of clinicopathological, treatment and outcome parameters of 15 patients.

Koehler S, Hengel FE, Dumoulin B, Damashek L, Holzman LB, Susztak K, Huber TB; International Podocyte Conference Organizing Committee. The 14th International Podocyte Conference 2023: from podocyte biology to glomerular medicine. Kidney Int. 2024 May;105(5):935-952. [PubMed link]
The 14th International Podocyte Conference successfully convened scientists from around the globe, fostering the presentation and discussion of crucial research findings, as summarized in this review.

Ogata A, Deki S, Uchimura T, Inaba A, Otani M, Ito S. Multinucleated podocytes as a clue to diagnosis of juvenile nephropathic cystinosis. Pediatr Nephrol. 2024 Feb;39(2):609-612. [PubMed link]
Early diagnosis of cystinosis, especially the juvenile nephropathic form, remains challenging because typical symptoms only become apparent in adulthood. The authors herein describe a 13-year-old girl who presented with proteinuria only but was diagnosed with juvenile nephropathic cystinosis based on multinucleated podocytes in her kidney biopsy specimen. Spectacular! initial diagnosis only based on biopsy!

Sikosana MLN, Reeve J, Madill-Thomsen KS, Halloran PF; INTERCOMEX Investigators. Using Regression Equations to Enhance Interpretation of Histology Lesions of Kidney Transplant Rejection. Transplantation. 2024 Feb 1;108(2):445-454. [PubMed link]
In regression and random forests, the important features predicting molecular rejection were as follows: for AMR, ptc and g, followed by cg; for TCMR, t > i. V-lesions, C4d and DSA were relatively unimportant.

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