Sjögren syndrome, characterized by lymphocytic proliferation and drying up of exocrine secretions ("sicca syndrome") due to glandular destruction, is well known for it's ocular and salivary localizations. However, other glandular epithelia (bronchial, vaginal . . .) or parenchyma (such as the kidney) may be involved. Damage to the kidney is rarely prominent; it is usually discovered late in the course of the disease. Moreover the renal manifestations are multiple and variably associated: diabetes insipidus, tubular acidosis, Fanconi syndrome, tubular and interstitial nephritis, and glomerulonephritis.
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Annu Rev Pathol
January 2025
Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas, USA; email:
Focal segmental glomerulosclerosis (FSGS) is the morphologic manifestation of a spectrum of kidney diseases that primarily impact podocytes, cells that create the filtration barrier of the glomerulus. As its name implies, only parts of the kidney and glomeruli are affected, and only a portion of the affected glomerulus may be sclerosed. Although the diagnosis is based primarily on microscopic features, patient stratification relies on clinical data such as proteinuria and etiological criteria.
View Article and Find Full Text PDFJ Kidney Cancer VHL
December 2024
Department of Urology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
Central nervous system hemangioblastoma (CNS-HB) is the most common manifestation of von Hippel-Lindau disease (VHL). The main axis of the CNS-HB pathway is the VHL-HIF signaling pathway. Recently, we proposed an alternative VHL-JAK-STAT pathway in CNS-HB.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Nephrology, Ningbo No.2 Hospital, Ningbo, Zhejiang, China.
Background: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibrous inflammatory disease. Recently, an association between IgG4-RD and tuberculosis (TB) has been reported.
Case Summary: We report a 56-year-old man complaining of a cough and poor appetite for 2 months and oliguria for 1 day.
Iran J Basic Med Sci
January 2025
Department of Nephrology, Affiliated Hospital of Jiangnan University, Wuxi, China.
Objectives: To investigate the natural product sulforaphane (SFN) in protection of membranous nephropathy (MN) by inhibiting oxidative stress-associated podocyte pyroptosis.
Materials And Methods: A passive Heymann nephritis (PHN) model was established and treated with SFN. Clinical manifestations were examined by testing 24-hr urine protein, albumin, total cholesterol, triglyceride, high-density and low-density lipoprotein levels.
Kidney Int
February 2025
Institute of Physiology, University of Zurich, Zurich, Switzerland; Division of Nephrology, Cliniques universitaires Saint-Luc, UCLouvain Medical School, Brussels, Belgium. Electronic address:
The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations.
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