Glomerular Diseases: Epidemiology, Classification, Pathogenesis, and Clinical Features, Summaries of Nephrology

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Practical Approach in

Glomerular Disease

Aida Lydia

Division of Nephrology and Hypertension

Cipto Mangunkusumo Hospital

Jakarta

• Epidemiology and Classification
• Pathogenesis
• Clinical feature
• Laboratory studies
• Kidney biopsy
• Differential Diagnosis of GN

Epidemiology Woo, Keng-Thye. Kidney Dis. DOI:10.1159/

Classification of Glomerular Disease Glomerular Disease Defect in glomerular filtration barrier causing one or more of the following: glomerular proteinuria, glomerular hematuria or decrease in GFR. Primary Glomerulopathy Begins in the glomerulus and causing damage only to the glomerulus- Primary FSGS Secondary Glomerulopathy Glomerular disease due to systemic disease 2 nd degree FSGS Glomerular disease due to severe nephron loss. The remaining nephron will be hyperperfused and having focal segmental sclerosis. Am J Nephrol 2013 Minimal change disease Post-streptococcal acute GN Idiopathic membranous nephropathy Anti-GBM disease IgA nephritis Primary FSGS Diabetic nephropathy Lupus Nephritis Amyloidosis ANCA-related vasculitis Type 1, 2, 3 cryoglobulinemia infection-related GN Nonglomerular CKD causing severe nephron loss .

Importance of the site of glomerular injury

• The major determinant of whether the patient presents with GN and an active urine sediment (nephritic syndrome ) or with proteinuria (nephrotic syndrome) and hematuria, the site of glomerular injury— particularly, which glomerular cells are targeted.
• There are three major types of resident glomerular cells: epithelial cells (visceral and parietal) , endothelial cells , and mesangial cells.

Pathogenesis

• Most forms of GN are thought to be immune-mediated.
• The immunopathogenesis of GN is often complex and may

be the result of of genetics and unfavorable environmental

conditions. Genetic factors clearly predispose certain

individuals to develop immune responses that can lead to

GN.

• Glomerular injury is usually mediated by the actions of

multiple elements of both the innate and the adaptive

immune systems, resulting in diverse clinical and pathologic

manifestations.

Am J Nephrol 2013

Mechanism of Immune Injury: Inflammatory and Non-Inflammatory

• Inflammatory injury GN is characterized by glomerular infiltration by hematopoietic cells such as neutrophils and macrophages and/or proliferation of resident glomerular cells. Am J Nephrol 2013

• Noninflammatory GN resulting from immune injury usually target the glomerular podocyte.
• associated with major functional changes in the glomerulus that result in an increase in glomerular permeability to albumin and other proteins
• The major clinical features of noninflammatory glomerular lesions are proteinuria and the nephrotic syndrome , with little or no hematuria and no red blood cell casts.
• Common causes of immune-mediated nephrotic syndrome without inflammatory changes are minimal change disease (MCD ) and membranous nephropathy (MN). Mechanism of Immune Injury Bonegio RGB UTD 2019

Systemic findings

• Constitutional – fevers, chills, weight loss, night sweats, fatigue
• Eye – retinitis or uveitis
• Ear, nose, and throat – epistaxis, sinusitis, oral ulcers
• Cardiovascular – murmurs, pain (pericarditis), or heart failure
• Lungs – hemoptysis, infiltrates, or nodules
• Abdomen – enteritis, colitis, or pancreatitis
• Nervous system – seizures or peripheral neuropathy
• Extremities – digital ischemia or infarction
• Skin – purpura or rash
• Musculoskeletal – arthritis, arthralgias, myalgias
• Infections – particularly evidence of Staphylococcus , Streptococcus , hepatitis virus, or human immunodeficiency virus (HIV), syphilis

History Taking

• Patients may come with symptoms or asymptomatic
• Oedema, hypertension, history of infection may be present
• Systemic illness: SLE, diabetes, hypertension, amyloidosis, vasculitis
• Family history: Alport syndrome, genetic-related FSGS, familial IgA disease Clin J Am Soc Nephrol 2017

Laboratory Studies

• Urinalysis
• 24 - h urine collection
• Urine albumin-creatinine ratio (UACR)
• Serum albumin
• LDH
• Complete blood count
• C3, C4, ANA, anti ds DNA
• Serum protein electrophoresis + free light chain
• HBsAg, Anti-HCV, Anti-HIV
• ANCA
• Routine CKD workup: Electrolytes, blood glucose, intact PTH, blood urea nitrogen, serum creatinine, lipid profile, calcium, phosphate. Am J Nephrol 2013 Kidney Inter Suppl 2012

Laboratory Examination