Makindo Medical Notes

Related Subjects: |Metabolic acidosis |Lactic acidosis |Acute Kidney Injury (AKI) / Acute Renal Failure |Renal/Kidney Physiology |Chronic Kidney Disease (CKD) |Anaemia in Chronic Kidney Disease |Analgesic Nephropathy |Medullary Sponge kidney |IgA Nephropathy (Berger's disease) |HIV associated nephropathy (HIVAN) |Balkan endemic nephropathy (BEN) |Autosomal Dominant Polycystic kidney disease

IgA nephropathy typically affects young males and often presents with frank haematuria following an episode of pharyngitis.

About

• IgA Nephropathy (Berger's Disease): The most common cause of glomerulonephritis (GN) worldwide, accounting for about 40% of cases.
• Approximately 20% of patients progress to end-stage renal failure (ESRF).
• Initially presents with microscopic haematuria, which later progresses to proteinuria.

Aetiology

• Characterized by the deposition of IgA in the renal mesangium.
• The exact cause is unknown, but it is believed to be an immune-mediated condition.
• IgA nephropathy is thought to be part of the same disease spectrum as Henoch-Schönlein Purpura (IgA vasculitis).

Clinical Features

• Haematuria: Visible haematuria often occurs after upper respiratory infections (e.g., pharyngitis), along with red cell casts in the urine.
• Proteinuria: Can range from mild to severe, leading to nephrotic syndrome in some cases.
• Hypertension: Common, especially in progressive cases.
• Pharyngitis: Frequently precedes the onset of haematuria.

Investigations

• Urinalysis: Shows visible and non-visible haematuria, proteinuria, and red cell casts.
• Renal Biopsy: Key diagnostic test showing:
• Mesangial proliferation with IgA deposition.
• Crescent formation on biopsy suggests a more severe and progressive disease.
• Histopathological pattern often consistent with mesangioproliferative glomerulonephritis.
• Serum Complement: C3 and C4 levels are typically normal, helping to distinguish from other immune complex diseases.
• Serum IgA: May be elevated in some patients, and skin biopsies can show IgA deposits, particularly in Henoch-Schönlein Purpura.

Associations

• IgA nephropathy is associated with several conditions, including:
• Ankylosing spondylitis.
• Chronic liver disease (e.g., cirrhosis).
• Dermatitis herpetiformis (a skin condition linked to gluten sensitivity).

Management

• Blood Pressure Control: Treat hypertension to slow disease progression.
• Proteinuria Management: Angiotensin-converting enzyme inhibitors (ACE inhibitors) or angiotensin II receptor blockers (ARBs) are often used to reduce proteinuria.
• Steroids: Some patients, particularly those with significant proteinuria, may benefit from high-dose corticosteroids.
• Immunosuppression: There is no strong evidence supporting immunosuppression, but it may be considered in select cases.
• Prognosis: Males, older patients, and those with nephrotic syndrome or hypertension tend to have worse outcomes.
• End-Stage Renal Failure (ESRF): Progressive chronic kidney disease may ultimately require renal replacement therapy (e.g., dialysis or transplantation).