Pediatric IgA Nephropathy Clinical Presentation

Updated: Jun 03, 2022
  • Author: Mohammad Ilyas, MD, FAAP; Chief Editor: Craig B Langman, MD  more...
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Presentation

History

Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by recurrent episodes of macroscopic hematuria accompanied by upper respiratory tract infections or persistent asymptomatic microscopic hematuria with or without proteinuria. IgA nephropathy is frequently classified as primary (idiopathic) or secondary (associated with some other condition).

Primary IgA nephropathy

Although the clinical presentation of IgA nephropathy varies from asymptomatic urinary abnormalities to acute renal failure, the following clinical syndromes are generally recognized:

1. Gross hematuria – Approximately 40-50% of patients present with one or recurrent episodes of gross hematuria, often accompanying an upper respiratory tract infection. These episodes can be provoked by bacterial tonsillitis or by viral upper respiratory tract infections; they may also occur in individuals who have already undergone tonsillectomy. 

2. Microscopic hematuria with or without proteinuria – Another 30-40% of patients have microscopic hematuria and usually mild proteinuria and are incidentally detected on a routine examination or during a diagnostic evaluation for chronic kidney disease. [22]

3. Nephrotic syndrome or rapidly progressive glomerulonephritis – Less than 10% of patients present with either nephrotic syndrome or an acute, rapidly progressive glomerulonephritis characterized by edema, hypertension, and kidney function impairment as well as hematuria. Rarely, IgAN may present with malignant hypertension. 

4. Acute kidney injury – Rarely, patients develop acute kidney injury with or without oliguria. This may be due to crescentic IgAN or to heavy glomerular hematuria leading to tubular occlusion and/or damage by red cells. The latter is usually a reversible phenomenon, although incomplete recovery of kidney function may occur. [23]

Secondary IgA nephropathy

Secondary IgA nephropathy (IgAN) has been attributed to a variety of clinical conditions, including cirrhosis and other forms of severe liver disease, celiac disease, HIV infection, and other disorders. The optimal treatment approach for these forms of secondary IgAN is not well established. In general, therapy should be directed at the underlying primary disease. In certain conditions, such as celiac disease and inflammatory bowel disease, addressing the underlying disease has led to improvement in urinary abnormalities (eg, proteinuria and microscopic hematuria) or clearance of mesangial IgA deposits. [24]

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Physical Examination

In the early stages of primary IgA nephropathy, no physical signs may be observed. However, early diagnosis might be suggested by a urinalysis that reveals microscopic hematuria with or without proteinuria.

Hypertension is infrequent, is mild to moderate, and is usually a late presentation of disease.

Edema due to nephrosis is reported in approximately 10% of patients.

If renal function is compromised at presentation, sign and symptoms of acute kidney injury, including hypertension, hyperkalemia, and acidosis, may be noticed. If chronic kidney disease (CKD) developed, the patient may have symptoms of uremia, anemia, pallor, lethargy, hypertension, fatigue, and swelling due to underlying progressive CKD. 

If IgA nephrology is secondary to underlying disease, such Henoch-Schönlein purpura (HSP) or systemic lupus erythematosus (SLE), the signs and symptoms of that specific primary disease may be apparent.

All patients should have close monitoring of vital signs. Patients need close monitoring of blood pressure during routine follow-up evaluation, because nephritis can lead to hypertension. Sign and symptoms of secondary nephritis should be watched for, such as lupus nephritis and HSP. 

The physical examination depends upon the clinical presentation. Most patients who present with microscopic hematuria do not have any physical findings; however, patients with nephrotic-range proteinuria could present with edema, periorbital edema, lower extremity edema, hypertension, and gross hematuria.

Patients with underlying HSP could have skin rashes and joint symptoms, as well as abdominal pain.

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