ICD-10: N02.B2

Recurrent and persistent immunoglobulin A nephropathy with focal and segmental glomerular lesion

Clinical Information

Inclusion Terms

  • Recurrent and persistent immunoglobulin A nephropathy with focal and segmental hyalinosis or sclerosis

Additional Information

Description

ICD-10 code N02.B2 refers to Recurrent and persistent immunoglobulin A (IgA) nephropathy with focal and segmental glomerular lesion. This condition is a specific type of kidney disease characterized by the deposition of IgA in the glomeruli, which are the filtering units of the kidneys. Below is a detailed clinical description and relevant information regarding this diagnosis.

Clinical Description

Overview of IgA Nephropathy

IgA nephropathy, also known as Berger's disease, is a common form of glomerulonephritis. It occurs when IgA, an antibody that plays a crucial role in the immune response, accumulates in the glomeruli. This accumulation can lead to inflammation and damage to the kidney tissues, resulting in impaired kidney function over time.

Recurrent and Persistent Nature

The designation of "recurrent and persistent" in the ICD-10 code indicates that the patient experiences repeated episodes of the disease, which may not resolve completely between episodes. This chronic nature can lead to progressive kidney damage and may require ongoing monitoring and management.

Focal and Segmental Glomerular Lesion

The term "focal and segmental glomerular lesion" refers to specific patterns of damage observed in the glomeruli. In this context:
- Focal means that only some of the glomeruli are affected, while others remain normal.
- Segmental indicates that only a portion of an affected glomerulus shows damage.

This pattern can be indicative of more severe disease and may correlate with a poorer prognosis compared to cases where the damage is diffuse.

Clinical Presentation

Symptoms

Patients with IgA nephropathy may present with a variety of symptoms, including:
- Hematuria: Blood in the urine, which may be visible (gross hematuria) or detectable only through laboratory tests (microscopic hematuria).
- Proteinuria: Excess protein in the urine, which can lead to foamy urine.
- Edema: Swelling, particularly in the legs and around the eyes, due to fluid retention.
- Hypertension: High blood pressure, which can develop as kidney function declines.

Diagnosis

Diagnosis typically involves:
- Urinalysis: To check for blood and protein in the urine.
- Blood tests: To assess kidney function and measure levels of IgA.
- Kidney biopsy: This is often necessary to confirm the diagnosis and assess the extent of glomerular damage.

Management and Treatment

Treatment Options

Management of recurrent and persistent IgA nephropathy with focal and segmental lesions may include:
- Medications: Such as corticosteroids or immunosuppressants to reduce inflammation and immune response.
- Blood pressure control: Using ACE inhibitors or angiotensin receptor blockers (ARBs) to protect kidney function.
- Lifestyle modifications: Including dietary changes, weight management, and regular exercise.

Monitoring

Regular follow-up is essential to monitor kidney function and adjust treatment as necessary. Patients may require periodic blood tests and urinalysis to track disease progression.

Prognosis

The prognosis for patients with IgA nephropathy varies widely. Some individuals may experience only mild symptoms and stable kidney function, while others may progress to end-stage renal disease (ESRD) requiring dialysis or kidney transplantation. The presence of focal and segmental lesions often indicates a higher risk of progression.

In summary, ICD-10 code N02.B2 captures a specific and complex condition that necessitates careful clinical management and monitoring. Understanding the nuances of this diagnosis is crucial for healthcare providers in delivering effective care and improving patient outcomes.

Clinical Information

Immunoglobulin A nephropathy (IgAN), classified under ICD-10 code N02.B2, is a kidney disorder characterized by the deposition of immunoglobulin A (IgA) in the glomeruli, leading to inflammation and damage. This condition often presents with a variety of clinical features, signs, and symptoms, which can vary significantly among patients.

Clinical Presentation

Signs and Symptoms

  1. Hematuria: One of the hallmark symptoms of IgAN is the presence of blood in the urine, which may be visible (gross hematuria) or detected only through urinalysis (microscopic hematuria). This symptom often occurs after respiratory infections or exercise[1].

  2. Proteinuria: Patients typically exhibit protein in the urine, which can range from mild to nephrotic levels. This condition may lead to foamy urine due to the high protein content[1][2].

  3. Edema: Swelling, particularly in the legs and around the eyes, can occur due to fluid retention, especially in cases where nephrotic syndrome develops[2].

  4. Hypertension: Elevated blood pressure is common in patients with IgAN, often resulting from fluid overload and kidney dysfunction[1].

  5. Renal Impairment: Over time, IgAN can lead to chronic kidney disease (CKD), with symptoms such as fatigue, decreased appetite, and changes in urination patterns as kidney function declines[2].

Patient Characteristics

  • Demographics: IgAN can affect individuals of any age but is most commonly diagnosed in young adults and adolescents. It has a higher prevalence in males compared to females[1][3].

  • Family History: A family history of IgAN or other kidney diseases may be present, suggesting a genetic predisposition[3].

  • Associated Conditions: Patients may have a history of respiratory infections, which can trigger the onset of symptoms. Additionally, IgAN is sometimes associated with other conditions such as liver disease, celiac disease, or certain infections[2][3].

Diagnostic Considerations

Diagnosis of IgAN typically involves a combination of clinical evaluation, laboratory tests, and kidney biopsy. Key diagnostic steps include:

  • Urinalysis: To detect hematuria and proteinuria.
  • Blood Tests: To assess kidney function and rule out other causes of kidney disease.
  • Kidney Biopsy: This is often necessary to confirm the diagnosis, revealing IgA deposits in the glomeruli and any associated lesions, such as focal and segmental glomerulosclerosis (FSGS) in more advanced cases[1][2].

Conclusion

IgAN, particularly in its recurrent and persistent form with focal and segmental glomerular lesions, presents a complex clinical picture characterized by hematuria, proteinuria, and potential progression to chronic kidney disease. Understanding the signs, symptoms, and patient characteristics is crucial for timely diagnosis and management. Regular monitoring and appropriate therapeutic interventions can help manage symptoms and slow disease progression, improving patient outcomes.

For further information or specific case studies, consulting nephrology literature or clinical guidelines may provide additional insights into the management of IgAN.

Approximate Synonyms

ICD-10 code N02.B2 refers specifically to "Recurrent and persistent immunoglobulin A nephropathy with focal and segmental glomerular lesion." This condition is characterized by the presence of immunoglobulin A (IgA) deposits in the kidneys, leading to inflammation and damage, particularly affecting the glomeruli.

Alternative Names

  1. IgA Nephropathy: This is the most common alternative name for the condition, emphasizing the role of immunoglobulin A in the disease process.
  2. Berger's Disease: Named after the French physician Jean Berger, who first described the condition, this term is frequently used interchangeably with IgA nephropathy.
  3. Focal Segmental Glomerulosclerosis (FSGS): While FSGS is a broader term that can refer to various types of kidney damage, it is relevant here due to the specific mention of focal and segmental lesions in the context of N02.B2.
  1. Glomerulonephritis: A general term for inflammation of the glomeruli, which can include various types of kidney diseases, including IgA nephropathy.
  2. Chronic Kidney Disease (CKD): This term may be used in the context of IgA nephropathy if the condition progresses to a more severe stage affecting kidney function.
  3. Nephritic Syndrome: A clinical syndrome that may occur in patients with IgA nephropathy, characterized by hematuria, proteinuria, and hypertension.
  4. Immunoglobulin A Vasculitis: Although distinct, this term relates to conditions where IgA plays a role in vascular inflammation, which can sometimes overlap with kidney involvement.

Conclusion

Understanding the alternative names and related terms for ICD-10 code N02.B2 is crucial for accurate diagnosis, treatment, and coding in medical records. These terms reflect the underlying pathology and clinical manifestations associated with recurrent and persistent immunoglobulin A nephropathy, aiding healthcare professionals in communication and documentation.

Diagnostic Criteria

Recurrent and persistent immunoglobulin A (IgA) nephropathy, classified under ICD-10 code N02.B2, is a kidney disorder characterized by the deposition of IgA in the glomeruli, leading to inflammation and potential kidney damage. The diagnosis of this condition typically involves a combination of clinical evaluation, laboratory tests, and histopathological examination. Below are the key criteria used for diagnosis:

Clinical Criteria

  1. Symptoms: Patients may present with symptoms such as hematuria (blood in urine), proteinuria (excess protein in urine), and hypertension. Some may experience edema or signs of chronic kidney disease.

  2. Medical History: A thorough medical history is essential, including any previous episodes of hematuria or urinary tract infections, as well as family history of kidney disease.

Laboratory Tests

  1. Urinalysis: A urinalysis is performed to detect hematuria and proteinuria. The presence of red blood cells and protein in the urine is indicative of glomerular damage.

  2. Serum Creatinine: Measurement of serum creatinine levels helps assess kidney function. Elevated levels may indicate impaired renal function.

  3. Immunological Tests: Testing for serum IgA levels can be useful, although elevated IgA alone is not diagnostic of IgA nephropathy.

Histopathological Examination

  1. Kidney Biopsy: A definitive diagnosis often requires a kidney biopsy. Histological examination typically reveals:
    - Focal and Segmental Glomerulosclerosis (FSGS): This is characterized by scarring in some glomeruli, which is a key feature of N02.B2.
    - Mesangial IgA Deposition: Immunofluorescence microscopy shows IgA deposits in the mesangial areas of the glomeruli.

  2. Electron Microscopy: This may be used to assess the ultrastructural changes in the glomeruli, which can provide additional diagnostic information.

Exclusion of Other Conditions

It is crucial to rule out other causes of glomerular disease, such as:
- Secondary Causes: Conditions like systemic lupus erythematosus, Henoch-Schönlein purpura, or liver disease can mimic IgA nephropathy.
- Other Glomerular Diseases: Differentiating from other forms of glomerulonephritis is essential for accurate diagnosis and management.

Conclusion

The diagnosis of recurrent and persistent IgA nephropathy with focal and segmental glomerular lesions (ICD-10 code N02.B2) relies on a combination of clinical symptoms, laboratory findings, and histopathological evidence. A kidney biopsy is often necessary to confirm the diagnosis and assess the extent of glomerular damage. Proper diagnosis is critical for determining the appropriate management and treatment strategies for affected patients.

Treatment Guidelines

Immunoglobulin A nephropathy (IgAN), particularly in its recurrent and persistent forms with focal and segmental glomerular lesions, presents a complex clinical challenge. The management of this condition typically involves a combination of pharmacological treatments, lifestyle modifications, and monitoring strategies. Below is a detailed overview of standard treatment approaches for ICD-10 code N02.B2.

Understanding IgA Nephropathy

IgA nephropathy is characterized by the deposition of IgA antibodies in the glomeruli, leading to inflammation and damage. The presence of focal and segmental glomerular lesions indicates that the damage is not uniform across the kidney, which can complicate treatment and prognosis. Patients may experience recurrent episodes of hematuria (blood in urine) and proteinuria (excess protein in urine), which can progress to chronic kidney disease (CKD) if not managed effectively.

Standard Treatment Approaches

1. Pharmacological Treatments

a. Immunosuppressive Therapy

  • Corticosteroids: Prednisone is often used to reduce inflammation and immune response. The dosage and duration depend on the severity of the disease and the patient's response to treatment.
  • Other Immunosuppressants: Medications such as mycophenolate mofetil or azathioprine may be considered, especially in cases resistant to corticosteroids or when there is significant proteinuria.

b. Angiotensin-Converting Enzyme (ACE) Inhibitors or Angiotensin II Receptor Blockers (ARBs)

  • These medications are crucial for managing hypertension and reducing proteinuria. They help protect kidney function by lowering intraglomerular pressure, which can mitigate further damage to the glomeruli.

c. Antihypertensive Medications

  • Control of blood pressure is essential, particularly in patients with CKD. A combination of diuretics and other antihypertensive agents may be necessary to achieve optimal blood pressure control.

2. Lifestyle Modifications

a. Dietary Changes

  • A low-sodium diet can help manage hypertension and reduce fluid retention. Additionally, a diet low in protein may be recommended to decrease the workload on the kidneys, particularly in advanced stages of the disease.

b. Weight Management

  • Maintaining a healthy weight is important for overall health and can help manage blood pressure and reduce the risk of diabetes, which can further complicate kidney disease.

3. Monitoring and Follow-Up

Regular monitoring of kidney function through blood tests (e.g., serum creatinine, estimated glomerular filtration rate) and urine tests (e.g., urinalysis for protein and blood) is essential. This helps in assessing the effectiveness of treatment and making necessary adjustments.

4. Advanced Therapies

In cases where standard treatments are ineffective, or if the disease progresses to end-stage renal disease (ESRD), more advanced interventions may be necessary:
- Dialysis: For patients with severe kidney failure, dialysis may be required to perform the functions of the kidneys.
- Kidney Transplantation: In suitable candidates, a kidney transplant may be considered, especially if there is a significant decline in kidney function.

Conclusion

The management of recurrent and persistent IgA nephropathy with focal and segmental glomerular lesions requires a comprehensive approach that includes pharmacological treatment, lifestyle modifications, and regular monitoring. Early intervention and tailored treatment plans are crucial to slowing disease progression and preserving kidney function. Collaboration between nephrologists, primary care providers, and patients is essential to optimize outcomes and improve quality of life for those affected by this condition.

Related Information

Description

Clinical Information

  • Hematuria: presence of blood in urine
  • Proteinuria: protein in urine, can be mild to severe
  • Edema: swelling due to fluid retention
  • Hypertension: high blood pressure due to kidney dysfunction
  • Renal Impairment: chronic kidney disease with symptoms like fatigue
  • Demographics: affects young adults and adolescents more
  • Family History: genetic predisposition to IgAN
  • Associated Conditions: respiratory infections, liver disease, celiac disease

Approximate Synonyms

  • IgA Nephropathy
  • Berger's Disease
  • Focal Segmental Glomerulosclerosis (FSGS)
  • Glomerulonephritis
  • Chronic Kidney Disease (CKD)
  • Nephritic Syndrome
  • Immunoglobulin A Vasculitis

Diagnostic Criteria

  • Hematuria in urine
  • Proteinuria present
  • Hypertension common
  • Family history of kidney disease
  • Elevated serum creatinine levels
  • IgA deposits in glomeruli
  • Focal and Segmental Glomerulosclerosis (FSGS)
  • Mesangial IgA deposition
  • Electron Microscopy for ultrastructural changes

Treatment Guidelines

  • Corticosteroids reduce inflammation
  • Immunosuppressants control immune response
  • ACE inhibitors or ARBs lower blood pressure
  • Antihypertensive medications control hypertension
  • Low-sodium diet reduces fluid retention
  • Low-protein diet decreases kidney workload
  • Regular monitoring assesses treatment effectiveness

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