ICD-10: N04.2

Nephrotic syndrome with diffuse membranous glomerulonephritis

Additional Information

Approximate Synonyms

Nephrotic syndrome with diffuse membranous glomerulonephritis, classified under ICD-10 code N04.2, is a specific condition characterized by a combination of nephrotic syndrome and membranous glomerulonephritis. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the key alternative names and related terms associated with this diagnosis.

Alternative Names

  1. Membranous Nephropathy: This term is often used interchangeably with diffuse membranous glomerulonephritis, as it describes the condition where the membranes of the glomeruli are thickened due to immune complex deposition.

  2. Nephrotic Syndrome Secondary to Membranous Nephropathy: This phrase emphasizes that the nephrotic syndrome is a result of the underlying membranous nephropathy.

  3. Diffuse Membranous Glomerulonephritis: This is a more specific term that highlights the diffuse nature of the glomerular changes associated with the condition.

  4. Idiopathic Membranous Nephropathy: In cases where the cause of the membranous nephropathy is unknown, this term may be used.

  5. Secondary Membranous Nephropathy: This term can be applied when the membranous nephropathy is due to other underlying conditions, such as infections, drugs, or systemic diseases.

  1. Nephrotic Syndrome (N04): This broader category includes various types of nephrotic syndrome, of which N04.2 is a specific subtype.

  2. Glomerulonephritis: A general term for inflammation of the glomeruli, which can encompass various types, including membranous glomerulonephritis.

  3. Proteinuria: A common symptom of nephrotic syndrome, referring to the presence of excess protein in the urine, which is a hallmark of the condition.

  4. Edema: This term describes the swelling that occurs due to fluid retention, commonly seen in patients with nephrotic syndrome.

  5. Hypoalbuminemia: A condition characterized by low levels of albumin in the blood, often associated with nephrotic syndrome.

  6. Hyperlipidemia: Elevated lipid levels in the blood, which can occur as a compensatory response to low protein levels in nephrotic syndrome.

Conclusion

Understanding the alternative names and related terms for ICD-10 code N04.2 is crucial for healthcare professionals involved in the diagnosis and treatment of patients with nephrotic syndrome and membranous glomerulonephritis. These terms not only facilitate clearer communication but also enhance the accuracy of medical records and billing processes. For further information or specific queries regarding nephrotic syndrome, consulting nephrology resources or coding guidelines may provide additional insights.

Description

Nephrotic syndrome with diffuse membranous glomerulonephritis, classified under ICD-10 code N04.2, is a specific type of kidney disorder characterized by a combination of nephrotic syndrome symptoms and the presence of diffuse membranous glomerulonephritis. This condition is significant in nephrology due to its implications for kidney function and overall health.

Clinical Description

Nephrotic Syndrome

Nephrotic syndrome is a clinical condition defined by a triad of symptoms:
1. Proteinuria: Excessive protein in the urine, typically greater than 3.5 grams per day.
2. Hypoalbuminemia: Low levels of albumin in the blood, leading to decreased oncotic pressure.
3. Edema: Swelling due to fluid retention, often noticeable in the legs, abdomen, and around the eyes.

These symptoms arise from damage to the glomeruli, the filtering units of the kidneys, which leads to increased permeability and loss of proteins from the bloodstream into the urine.

Membranous Glomerulonephritis

Diffuse membranous glomerulonephritis is a specific type of glomerular disease characterized by the thickening of the glomerular basement membrane due to the deposition of immune complexes. This condition can be idiopathic or secondary to other diseases, such as infections, drugs, or malignancies. The key features include:
- Immune Complex Deposition: The presence of immune complexes in the subepithelial space of the glomeruli.
- Thickened Membranes: Histological examination reveals a diffuse thickening of the glomerular capillary walls.
- Variable Presentation: Patients may present with nephrotic syndrome, hematuria, or hypertension.

Pathophysiology

The pathophysiological mechanism involves the immune system's response leading to inflammation and damage to the glomeruli. In membranous glomerulonephritis, the immune complexes trigger a cascade of events that result in increased permeability of the glomerular capillaries, allowing proteins to leak into the urine while impairing the kidney's ability to filter waste products effectively.

Diagnosis

Diagnosis of nephrotic syndrome with diffuse membranous glomerulonephritis typically involves:
- Urinalysis: To detect proteinuria and assess for hematuria.
- Blood Tests: To evaluate serum albumin levels, kidney function (creatinine), and lipid levels.
- Kidney Biopsy: This is often necessary to confirm the diagnosis and assess the extent of glomerular damage, revealing the characteristic changes associated with membranous glomerulonephritis.

Treatment

Management of nephrotic syndrome with diffuse membranous glomerulonephritis focuses on:
- Symptomatic Relief: Diuretics may be used to manage edema, and dietary modifications can help control protein intake.
- Immunosuppressive Therapy: In cases where the condition is severe or progressive, corticosteroids or other immunosuppressive agents may be indicated to reduce inflammation and immune response.
- Management of Complications: This includes monitoring and treating hypertension and hyperlipidemia, which are common in nephrotic syndrome.

Prognosis

The prognosis for patients with nephrotic syndrome due to diffuse membranous glomerulonephritis varies. Some patients may experience spontaneous remission, while others may progress to chronic kidney disease or end-stage renal failure. Regular follow-up and monitoring are essential to manage the condition effectively and mitigate complications.

In summary, ICD-10 code N04.2 encapsulates a complex interplay of symptoms and underlying pathology, necessitating a comprehensive approach to diagnosis and management to optimize patient outcomes.

Clinical Information

Nephrotic syndrome with diffuse membranous glomerulonephritis, classified under ICD-10 code N04.2, is a specific type of nephrotic syndrome characterized by a combination of clinical features, laboratory findings, and patient demographics. This condition primarily affects the kidneys and is associated with significant proteinuria, edema, and other systemic manifestations. Below is a detailed overview of its clinical presentation, signs, symptoms, and patient characteristics.

Clinical Presentation

Definition and Pathophysiology

Nephrotic syndrome is a kidney disorder that results in the excretion of excessive protein in the urine, leading to low serum albumin levels, edema, and hyperlipidemia. When associated with diffuse membranous glomerulonephritis, the condition is characterized by the presence of immune complex deposits along the glomerular capillary walls, which leads to increased permeability and subsequent protein loss[1][2].

Signs and Symptoms

Patients with nephrotic syndrome with diffuse membranous glomerulonephritis typically present with the following signs and symptoms:

  • Edema: This is often the most prominent feature, presenting as swelling in the legs, ankles, and around the eyes (periorbital edema). Severe cases may lead to generalized edema (anasarca) due to fluid retention[2][3].
  • Proteinuria: Patients usually exhibit significant proteinuria, often exceeding 3.5 grams per day. This is typically detected through urine dipstick tests or 24-hour urine collections[1][4].
  • Hypoalbuminemia: Low serum albumin levels (usually < 3 g/dL) are common, resulting from the loss of protein in urine[3][4].
  • Hyperlipidemia: Increased levels of lipids in the blood are often observed, as the liver compensates for low albumin levels by increasing lipid production[2][3].
  • Hypertension: Some patients may develop high blood pressure due to fluid overload and changes in renal function[1][4].
  • Fatigue and Weakness: General malaise and fatigue are common due to the systemic effects of the syndrome and fluid imbalance[2].

Laboratory Findings

  • Urinalysis: Shows significant proteinuria, often with a "frothy" appearance due to the high protein content.
  • Serum Tests: Low serum albumin levels, elevated cholesterol and triglycerides, and possible renal function impairment indicated by elevated creatinine levels[3][4].
  • Imaging: Renal ultrasound may be performed to assess kidney size and rule out other conditions, although it is not diagnostic for membranous nephropathy specifically[1].

Patient Characteristics

Demographics

  • Age: Nephrotic syndrome can occur at any age but is more common in children and young adults. Membranous nephropathy is more frequently diagnosed in adults, particularly those aged 30-50 years[2][3].
  • Gender: There is a slight male predominance in cases of membranous nephropathy, with men being affected more often than women[1][4].
  • Ethnicity: Certain ethnic groups may have a higher prevalence of nephrotic syndrome, with variations in underlying causes and responses to treatment[2].

Risk Factors

  • Underlying Conditions: Conditions such as systemic lupus erythematosus, infections (e.g., hepatitis B and C), and certain malignancies can predispose individuals to develop membranous nephropathy[1][3].
  • Medications: Some medications, including nonsteroidal anti-inflammatory drugs (NSAIDs) and certain antibiotics, have been implicated in the development of nephrotic syndrome[2][4].

Conclusion

Nephrotic syndrome with diffuse membranous glomerulonephritis presents a complex clinical picture characterized by significant proteinuria, edema, and systemic effects. Understanding the signs, symptoms, and patient demographics is crucial for timely diagnosis and management. Early intervention can help mitigate complications and improve patient outcomes, emphasizing the importance of recognizing this condition in clinical practice. For further management, referral to a nephrologist is often recommended to tailor treatment strategies based on the underlying cause and severity of the syndrome.

Diagnostic Criteria

Nephrotic syndrome with diffuse membranous glomerulonephritis, classified under ICD-10 code N04.2, is a specific condition characterized by a combination of nephrotic syndrome and the presence of diffuse membranous changes in the glomeruli. The diagnosis of this condition involves several clinical criteria and laboratory findings. Below is a detailed overview of the criteria used for diagnosis.

Clinical Criteria for Diagnosis

1. Symptoms of Nephrotic Syndrome

  • Proteinuria: The hallmark of nephrotic syndrome is significant proteinuria, typically defined as the excretion of more than 3.5 grams of protein in a 24-hour urine collection. This can be assessed through urine dipstick tests or more precise laboratory measurements.
  • Edema: Patients often present with generalized edema, which can be periorbital (around the eyes), peripheral (in the legs and feet), or ascitic (fluid accumulation in the abdominal cavity).
  • Hypoalbuminemia: A low serum albumin level (usually less than 3 g/dL) is indicative of nephrotic syndrome due to the loss of protein in urine.
  • Hyperlipidemia: Elevated levels of lipids in the blood, particularly cholesterol and triglycerides, are commonly observed in nephrotic syndrome.

2. Laboratory Findings

  • Urinalysis: A urinalysis will typically show a high level of protein, often with the presence of fatty casts or oval fat bodies.
  • Serum Tests: Blood tests will reveal low serum albumin levels and elevated cholesterol levels. Additionally, renal function tests may be performed to assess the overall kidney function.
  • Immunological Tests: In cases of membranous glomerulonephritis, specific tests may be conducted to identify underlying causes, such as autoimmune diseases or infections.

3. Histological Examination

  • Kidney Biopsy: A definitive diagnosis of diffuse membranous glomerulonephritis often requires a kidney biopsy. Histological examination will reveal diffuse thickening of the glomerular capillary walls due to subepithelial immune complex deposition. This finding is crucial for distinguishing membranous glomerulonephritis from other types of glomerular diseases.

4. Exclusion of Other Causes

  • It is essential to rule out secondary causes of nephrotic syndrome, such as diabetes mellitus, systemic lupus erythematosus, or infections (e.g., hepatitis B or C). This may involve additional blood tests and clinical evaluations.

Conclusion

The diagnosis of nephrotic syndrome with diffuse membranous glomerulonephritis (ICD-10 code N04.2) is based on a combination of clinical symptoms, laboratory findings, and histological evidence from kidney biopsy. Accurate diagnosis is critical for determining the appropriate management and treatment strategies for affected patients. If you suspect this condition, it is advisable to consult a nephrologist for comprehensive evaluation and management.

Treatment Guidelines

Nephrotic syndrome with diffuse membranous glomerulonephritis, classified under ICD-10 code N04.2, is a complex condition characterized by significant proteinuria, hypoalbuminemia, and edema due to damage to the glomeruli in the kidneys. The management of this condition typically involves a combination of pharmacological treatments, lifestyle modifications, and monitoring strategies. Below is a detailed overview of the standard treatment approaches for this condition.

Pharmacological Treatments

1. Corticosteroids

Corticosteroids, such as prednisone, are often the first line of treatment for nephrotic syndrome. They help reduce inflammation and proteinuria by suppressing the immune response. The dosage and duration depend on the severity of the condition and the patient's response to therapy. A common regimen may involve high doses initially, followed by a tapering schedule[3].

2. Immunosuppressive Agents

In cases where corticosteroids are ineffective or if the patient experiences frequent relapses, additional immunosuppressive medications may be prescribed. These can include:
- Mycophenolate mofetil: This drug is often used in pediatric patients and can be effective in reducing proteinuria and maintaining remission[7].
- Cyclophosphamide: This is another option that may be considered, particularly in adults, to help control the immune response[3].

3. Angiotensin-Converting Enzyme (ACE) Inhibitors

ACE inhibitors, such as lisinopril, are beneficial in managing hypertension and reducing proteinuria. They work by decreasing intraglomerular pressure, which can help protect kidney function over time[3].

4. Diuretics

Diuretics are commonly used to manage edema associated with nephrotic syndrome. Loop diuretics, such as furosemide, can help reduce fluid overload and improve symptoms of swelling[3].

5. Statins

Statins may be prescribed to manage dyslipidemia, which is common in nephrotic syndrome due to increased lipid synthesis in response to low albumin levels. They help reduce cardiovascular risk associated with high cholesterol levels[3].

Lifestyle Modifications

1. Dietary Changes

Patients are often advised to follow a low-sodium diet to help manage edema and hypertension. Additionally, a diet rich in protein may be recommended to counteract protein loss, although this should be tailored to individual needs and kidney function[3].

2. Fluid Management

Monitoring fluid intake is crucial, especially in patients with significant edema. Fluid restrictions may be necessary to prevent overload[3].

Monitoring and Follow-Up

1. Regular Laboratory Tests

Frequent monitoring of kidney function, protein levels, and electrolytes is essential. This includes checking serum creatinine, albumin, and lipid profiles to assess the effectiveness of treatment and adjust medications as needed[3].

2. Assessment of Complications

Patients should be monitored for potential complications, such as infections (due to immunosuppression), thromboembolic events (due to hypercoagulability), and progression to chronic kidney disease[3].

Conclusion

The management of nephrotic syndrome with diffuse membranous glomerulonephritis (ICD-10 code N04.2) requires a comprehensive approach that includes pharmacological treatment, lifestyle modifications, and regular monitoring. The choice of therapy should be individualized based on the patient's specific circumstances, including the severity of the disease, response to initial treatments, and any underlying health conditions. Ongoing research continues to refine these treatment strategies, aiming to improve outcomes for patients with this challenging condition.

Related Information

Approximate Synonyms

  • Membranous Nephropathy
  • Nephrotic Syndrome Secondary to Membranous Nephropathy
  • Diffuse Membranous Glomerulonephritis
  • Idiopathic Membranous Nephropathy
  • Secondary Membranous Nephropathy

Description

  • Proteinuria in urine
  • Hypoalbuminemia low blood albumin
  • Edema swelling due to fluid retention
  • Immune complex deposition in glomeruli
  • Thickened membranes in glomerular capillary walls
  • Variable presentation with nephrotic syndrome hematuria or hypertension

Clinical Information

  • Edema: Swelling in legs, ankles, periorbital
  • Proteinuria: Excessive protein loss in urine
  • Hypoalbuminemia: Low serum albumin levels
  • Hyperlipidemia: Increased lipid levels in blood
  • Hypertension: High blood pressure due to fluid overload
  • Fatigue and weakness: General malaise and fatigue
  • Urinalysis shows significant proteinuria with frothy appearance

Diagnostic Criteria

  • Proteinuria > 3.5g in 24-hour urine collection
  • Significant generalized edema
  • Hypoalbuminemia < 3 g/dL
  • Hyperlipidemia with elevated cholesterol and triglycerides
  • High protein level in urinalysis
  • Low serum albumin levels
  • Elevated cholesterol levels
  • Kidney biopsy showing diffuse thickening of glomerular capillary walls
  • Exclusion of secondary causes like diabetes mellitus and infections

Treatment Guidelines

  • Corticosteroids reduce inflammation and proteinuria
  • Immunosuppressive agents are used in refractory cases
  • ACE inhibitors control hypertension and proteinuria
  • Diuretics manage edema and fluid overload
  • Statins lower lipid levels and cardiovascular risk
  • Low-sodium diet helps manage edema and hypertension
  • Fluid management is crucial to prevent overload

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