{Reference Type}: Journal Article
{Title}: Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy.
{Author}: Kudose S;Cossey LN;Canetta PA;Sekulic M;Vanbeek CA;Huls FB;Gupta I;Bu L;Alexander MP;Cornell LD;Fidler ME;Markowitz GS;Larsen CP;D'Agati VD;Nasr SH;Santoriello D;
{Journal}: Kidney Int Rep
{Volume}: 8
{Issue}: 8
{Year}: 2023 Aug
{Factor}: 6.234
{DOI}: 10.1016/j.ekir.2023.05.007
{Abstract}: UNASSIGNED: Lysozyme-associated nephropathy (LyN), a rare cause of kidney injury in patients with chronic myelomonocytic leukemia (CMML), has not been well described to date. We report the clinicopathologic spectrum of LyN from a multi-institutional series.
UNASSIGNED: We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data.
UNASSIGNED: Thirty-seven patients had a median age of 74 years and included 78% males. Their most common presentation was acute kidney injury (AKI) or AKI on chronic kidney disease (CKD) (66%) with median estimated glomerular filtration rate (eGFR) of 21.7 ml/min per 1.73 m2, and proteinuria of 1.7 g. A minority (15%) had partial Fanconi syndrome. Serum lysozyme levels were elevated in all tested. Hematologic disorder (n = 28, 76%) was the most common etiology, including CMML (n = 15), acute myeloid leukemia (n = 5), and myelodysplastic syndrome (MDS) (n = 5). Nonhematologic causes (n = 5, 14%), included metastatic neuroendocrine carcinoma (n = 3), sarcoidosis, and leprosy. Etiology was unknown in 4 (11%). Pathology showed proximal tubulopathy with abundant hypereosinophilic intracytoplasmic inclusions, with characteristic staining pattern by lysozyme immunostain. Mortality was high (8/30). However, among the 22 alive, including 85% treated, 7 had improved kidney function, including 1 who discontinued dialysis and 6 with increase in eGFR >15 ml/min per 1.73 m2 compared with eGFR at the time of biopsy.
UNASSIGNED: Increased awareness of the full clinicopathologic spectrum of LyN may lead to prompt diagnosis, earlier treatment, and potentially improved outcome of this rare entity.