IGA Nephropathy In Children

Also known as Berger's nephropathy or Berger's disease, it affects more men than women. Children's most prevalent chronic glomerular disease  The most prevalent primary glomerular disease in adults that is linked to hematuria.

Systemic counterpart, known as Henoch Schonlein Purpura (HSP), in which there will be IgA deposits in the mesangium; this nephropathy is characterized by the deposition of IgA in the kidneys' mesangium in the absence of any systemic disease.

Etiopathogenesis Of IGA Nephropathy

The precise etiology is unknown; genetic variables play a role; cases are familially clustered; linkage studies suggest a potential relationship with 6q22-23.

Immune Complex Disease Mechanism

Low-galactosylated IgA1 → Autoantibody formation → Kidney immune complexes → IgAN .  The precise cause is not known.

Cases grouped together in families.  Linkage analyses suggest a potential relationship with 6q22–23.

Mechanism

Type-3 hypersensitivity reaction, another name for immune complex illness.It occurs because: - Poorly galactosylated IgA is formed in patients with IgA nephropathy.  Badly galactosylated IgA functions as an antigen that triggers the formation of autoantibodies.  When autoantibodies attach to IgA, immune complexes are deposited in the kidneys, which causes IgA nephropathy to develop.

Clinical Features

 Periodic, frequently ppt by URI (1-2 days) . Hallmark Recurrent Gross Hematuria.  Every episode → lasts between two and five days.  Additional -  Nephrotic syndrome including mixed nephrotic; proteinuria → <1000 mg/24 hr.  HTN → Mild to moderate. Recurrent gross hematuria is the clinical signature. Episodic illnesses that mimic upper respiratory tract infections usually occur 1-2 days after the onset of URI. Every episode lasts between two and five days (it resolves in five days), and then gross hematuria occasionally recurs.

Other Clinical Manifestations

• Hematuria microscopically.
• The proteinuria.
• Less than 1000 mg/24 hours, not important.
• Nephritic syndrome plus mixed nephrotic syndrome
• Moderate to mild hypertension.
• Most prevalent in nations throughout Southeast Asia
• Western African countries > India > Japan, Korea, China, and Taiwan
• It is more common in senior age groups in the Indian population than in younger age groups.

Investigations And Diagnosis

Serum C3: Normal in 90% & Low in 5–10%; Serum IgA: Elevated only in 15%; not helpful.  Glum Hematuria ± Proteinuria in Urine Analysis. Renal Biopsy mesangioproliferative Pattern: IOC → IgA deposits in mensangium ± C3, IgG ↑ focal & segmental ↑ in mesangium as well as its cellularity.  Rarely · Crescents , diffuse inv.

The diagnostic characteristic is IgA deposition.  Exhibits focal and segmental mesangial proliferation as well as elevated mesangium cellularity.  Immunofluorescence study with anti-IgA serum: elevated IgA accumulation in the layer of mesangia.

Treatment 

Therapy in support is provided.  ACE inhibitors, such as enalapril or ARBs, are thought to be first-line treatments for hypertension (HTN) and proteinuria reactions. ARBs also impede the advancement of the illness.

Omega-3 fatty acids and fish oil also slow the progression of illness.  Dosage: 6–12 g daily.  If GFR is greater than 60 ml/min/m2, take into consideration steroids such as prednisolone if proteinuria is severe or persistent.

Some pilot studies have explored RAAS inhibitors and oral Budesonide.  Speculative or debatable: tonsillectomy and azathioprine or cyclophosphamide.  Although incidences are very high in some patients in Japan, advice varies and is not recommended in a nation such as India.

In more severe situations, renal transplants. Graft recurrences are possible, however the rejection rate ranges from 15% to 30%. Renal transplants are therefore optional.

Prognosis

Because end-stage renal disease (ESRD) is a chronic, slow-moving disease, its incidence is lower in children than in adults. About 15-20 years after the disease's inception, 20-30% of adults acquire a progressive renal disease.

Poor Prognostic Markers 

Chronic hypertension; severe, protracted proteinuria, particularly in the absence of ACE inhibitor response.
Diffuse mesangial growth, crescents, and diffuse tubulointerstitial damage with fibrosis are observed in the histology. Renal insufficiency.

Also Read: Mumps : Virus Characteristics, Pathology and Pathogenesis, Investigations, Complications and Treatment

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