Henoch-Schönlein Purpura Nephritis: Causes, Symptoms & Treatment

Introduction

Henoch-Schönlein purpura nephritis (HSPN) is a rare but significant kidney condition that can develop as a complication of Henoch-Schönlein purpura (HSP), a type of vasculitis. First described in the 19th century, HSP is characterized by inflammation of small blood vessels, which can lead to a distinctive purplish rash, joint pain, abdominal discomfort, and, in some cases, kidney involvement. When the kidneys are affected, the condition is referred to as Henoch-Schönlein purpura nephritis.

This article offers a detailed overview of HSPN, covering its risk factors, symptoms, diagnostic methods, treatment options, and home care strategies. Whether you or a loved one has been diagnosed, gaining a deeper understanding of this condition can help you make informed decisions about care and management.

What is Henoch-Schönlein Purpura Nephritis?

Henoch-Schönlein purpura nephritis is a kidney disorder caused by inflammation of small blood vessels within the kidneys. This article delves into its risk factors, symptoms, diagnostic tools, treatment approaches, and home care strategies to support effective management of the condition.

Understanding Henoch-Schönlein Purpura Nephritis

Henoch-Schönlein purpura nephritis (HSPN) is a complication of Henoch-Schönlein purpura (HSP), an autoimmune disorder that primarily affects children but can also occur in adults. HSP causes inflammation in small blood vessels, leading to symptoms such as a purplish rash, joint pain, and abdominal discomfort. When this inflammation extends to the kidneys, it results in HSPN, impairing the kidneys’ ability to filter waste and regulate fluid balance.

The severity of HSPN can vary widely. In some individuals, kidney involvement is mild and resolves without intervention. However, in more severe cases, it can lead to long-term kidney damage, including protein leakage into the urine (proteinuria), blood in the urine (hematuria), and, in rare instances, chronic kidney disease or kidney failure.

HSPN is relatively uncommon, affecting 20-50% of individuals diagnosed with HSP. It is most frequently seen in children, particularly those aged 4 to 10, though adults can also develop the condition. While the majority of cases resolve without lasting complications, approximately 1-2% of patients may experience significant kidney damage requiring ongoing medical care.

Early diagnosis and timely medical intervention are crucial to preventing complications and achieving the best possible outcomes.

Risk Factors for Henoch-Schönlein Purpura Nephritis

Lifestyle Risk Factors

Although Henoch-Schönlein purpura nephritis is not directly caused by lifestyle choices, certain factors can increase the likelihood of developing the condition. For instance, exposure to infections, particularly upper respiratory infections such as colds or strep throat, is a common trigger for Henoch-Schönlein purpura. Practicing good hygiene, such as frequent handwashing, can help reduce the risk of infections that may lead to HSP and, subsequently, HSPN.

Stress and a weakened immune system may also play a role in triggering autoimmune conditions like HSP. While these factors are not direct causes, managing stress through relaxation techniques and maintaining a healthy lifestyle can support overall immune health.

Medical Risk Factors

Underlying medical conditions that weaken the immune system can increase the risk of developing Henoch-Schönlein purpura and its complications, including nephritis. For example, individuals with autoimmune diseases such as lupus or rheumatoid arthritis may face a heightened risk.

A recent history of infections, particularly viral or bacterial, is another significant risk factor. These infections can provoke an abnormal immune response, leading to blood vessel inflammation. In rare cases, certain medications, including antibiotics or vaccines, have been reported as potential triggers, although the exact mechanisms remain unclear.

Genetic and Age-Related Risk Factors

Genetics may influence the likelihood of developing Henoch-Schönlein purpura nephritis. Individuals with a family history of autoimmune or kidney diseases may have a higher predisposition to the condition. However, HSPN is not considered a hereditary disease in the traditional sense.

Age is another critical factor. HSP and HSPN are most commonly diagnosed in children, particularly those aged 4 to 10. While less common in adults, the condition tends to be more severe in this group, with a greater likelihood of causing long-term kidney complications.

Gender may also play a minor role, as boys are slightly more likely to develop HSPN than girls. The reasons for this difference are not yet fully understood.

By understanding these risk factors, patients and their families can take proactive steps to minimize triggers and seek early medical attention if symptoms arise.

Clinical Manifestations of Henoch-Schönlein Purpura Nephritis

Rash

A rash is the hallmark symptom of Henoch-Schönlein purpura (HSP), occurring in nearly all cases. Known as “palpable purpura,” this rash appears as small, raised, reddish-purple spots on the skin, typically on the lower extremities, buttocks, and sometimes the arms. The rash results from inflammation and leakage of small blood vessels, a process called vasculitis. In HSP nephritis, the rash may precede or accompany kidney-related symptoms, serving as an early warning sign.

Abdominal Pain

Abdominal pain affects 50-75% of individuals with HSP. This pain is often cramp-like and may be accompanied by gastrointestinal symptoms such as nausea, vomiting, or diarrhea. It is caused by inflammation of blood vessels in the gastrointestinal tract, a condition known as mesenteric vasculitis. In some cases, abdominal pain can precede kidney symptoms, making it an important indicator to monitor in the progression of HSP nephritis.

Joint Pain

Joint pain, or arthralgia, occurs in 60-80% of HSP patients. It most commonly affects the knees and ankles but can involve other joints. The pain results from inflammation of small blood vessels in the synovium (joint lining). Unlike other forms of arthritis, HSP-related joint pain is typically non-erosive, meaning it does not cause long-term joint damage. While this symptom often resolves as the disease progresses, it can cause significant discomfort during the acute phase.

Hematuria

Hematuria, or blood in the urine, is a key sign of kidney involvement in HSP nephritis, occurring in 40-50% of patients. It may be microscopic (detectable only through laboratory tests) or macroscopic (visible as pink, red, or cola-colored urine). Hematuria results from inflammation and damage to the glomeruli, the kidney’s tiny filtering units. Persistent hematuria may indicate ongoing kidney damage and requires close monitoring.

Proteinuria

Proteinuria, or excess protein in the urine, is another critical symptom of HSP nephritis, affecting 20-40% of cases. It occurs due to glomerular damage, which allows proteins to leak into the urine. Proteinuria can range from mild to severe and serves as an important marker for assessing kidney involvement. Persistent or worsening proteinuria may signal progressive kidney damage, necessitating prompt medical intervention.

Edema

Edema, or swelling, is a less common but significant symptom of HSP nephritis, occurring in 10-20% of patients. It often results from severe proteinuria. When protein levels in the blood drop due to urinary leakage, fluid can accumulate in tissues, causing swelling. Edema is most noticeable in the legs, feet, and around the eyes and often indicates advanced kidney involvement.

Fatigue

Fatigue is a non-specific symptom that affects many individuals with HSP nephritis. It may result from the body’s inflammatory response, anemia, or the strain of chronic kidney disease. Fatigue can significantly impact quality of life, so patients should discuss this symptom with their healthcare provider for appropriate management.

Nausea and Vomiting

Nausea and vomiting occur in 20-30% of HSP patients, particularly those with significant gastrointestinal or kidney involvement. These symptoms may stem from mesenteric vasculitis, uremia (a buildup of waste products in the blood due to kidney dysfunction), or side effects of treatment. Persistent nausea and vomiting require medical attention to prevent dehydration and other complications.

Diarrhea

Diarrhea is a less common symptom, affecting 10-20% of HSP patients. It is usually linked to gastrointestinal vasculitis and may be accompanied by abdominal pain. In some cases, diarrhea can lead to dehydration and electrolyte imbalances, complicating the management of HSP nephritis. Monitoring and addressing this symptom are essential for maintaining overall health.

Health Conditions with Similar Symptoms to Henoch-Schönlein Purpura Nephritis

IgA Vasculitis

IgA vasculitis, also known as Henoch-Schönlein purpura (HSP), is a condition characterized by inflammation of small blood vessels due to deposits of IgA antibodies. This condition commonly affects the skin, joints, intestines, and kidneys, leading to symptoms such as a purplish rash, abdominal pain, joint discomfort, and kidney-related issues. Kidney involvement may present as hematuria (blood in the urine) and proteinuria (protein in the urine).

How to Differentiate IgA Vasculitis from Henoch-Schönlein Purpura Nephritis

IgA vasculitis and Henoch-Schönlein purpura nephritis are closely linked, with the latter being a kidney-specific complication of IgA vasculitis. Both conditions share overlapping symptoms, including a purpuric rash, abdominal pain, and kidney involvement. However, Henoch-Schönlein purpura nephritis may primarily manifest with kidney-related symptoms, such as blood or protein in the urine, without the hallmark rash or abdominal pain.

To distinguish between the two, healthcare providers may conduct a urinalysis to detect blood and protein in the urine and perform a kidney biopsy to confirm the presence of IgA deposits. If IgA deposits are found without systemic symptoms like rash or joint pain, this may indicate Henoch-Schönlein purpura nephritis rather than the broader IgA vasculitis.

Systemic Lupus Erythematosus (SLE)

Systemic lupus erythematosus (SLE) is an autoimmune disease in which the immune system mistakenly attacks healthy tissues, causing widespread inflammation. It can affect the skin, joints, kidneys, and blood vessels. Lupus nephritis, a kidney complication of SLE, may result in symptoms such as swelling, high blood pressure, and abnormal findings in urine tests.

How to Differentiate SLE from Henoch-Schönlein Purpura Nephritis

Both SLE and Henoch-Schönlein purpura nephritis can cause kidney inflammation, leading to symptoms like blood or protein in the urine and swelling in the legs. However, SLE often presents with additional systemic features, such as a butterfly-shaped rash across the cheeks, sensitivity to sunlight, mouth sores, and joint pain without visible redness or swelling.

Blood tests for antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) are typically elevated in SLE but not in Henoch-Schönlein purpura nephritis. A kidney biopsy can also help differentiate the two conditions, as lupus nephritis involves multiple types of antibody deposits, whereas Henoch-Schönlein purpura nephritis is primarily associated with IgA deposits.

Vasculitis

Vasculitis refers to a group of disorders that cause inflammation of blood vessels, which can reduce blood flow and damage organs. Symptoms vary depending on the type of vasculitis and the organs affected but may include fever, fatigue, weight loss, and organ-specific issues such as skin rashes or kidney problems.

How to Differentiate Vasculitis from Henoch-Schönlein Purpura Nephritis

Henoch-Schönlein purpura nephritis is a specific type of vasculitis characterized by IgA deposits in the kidneys. Other forms of vasculitis, such as granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA), may also cause kidney inflammation but are often accompanied by symptoms like nasal congestion, sinus infections, or lung involvement, such as coughing up blood.

Blood tests for antineutrophil cytoplasmic antibodies (ANCA) can help identify other types of vasculitis. A kidney biopsy may reveal distinct patterns of inflammation and antibody deposits, aiding in the diagnosis.

Nephrotic Syndrome

Nephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to symptoms such as swelling (edema), foamy urine, and low levels of protein in the blood. It can arise from various underlying conditions, including diabetes, infections, or autoimmune diseases.

How to Differentiate Nephrotic Syndrome from Henoch-Schönlein Purpura Nephritis

Both nephrotic syndrome and Henoch-Schönlein purpura nephritis can cause proteinuria and swelling. However, nephrotic syndrome typically lacks systemic symptoms such as a purpuric rash or abdominal pain, which are common in Henoch-Schönlein purpura nephritis.

Urinalysis can measure protein levels in the urine, while blood tests can assess albumin levels, which are often low in nephrotic syndrome. A kidney biopsy may provide further clarity, revealing distinct patterns of kidney damage that differentiate nephrotic syndrome from Henoch-Schönlein purpura nephritis.

Infections

Certain infections, such as streptococcal infections, can mimic Henoch-Schönlein purpura nephritis by causing kidney inflammation (post-infectious glomerulonephritis) and systemic symptoms like fever, fatigue, and joint pain.

How to Differentiate Infections from Henoch-Schönlein Purpura Nephritis

Infections often present with fever, sore throat, or other signs of active infection, which are not typical of Henoch-Schönlein purpura nephritis. Blood tests, including a complete blood count (CBC) and markers like C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), can help identify infections. A throat swab or blood cultures may detect specific pathogens. If kidney inflammation resolves after treating the infection, it is more likely to be post-infectious glomerulonephritis rather than Henoch-Schönlein purpura nephritis.

Thrombocytopenic Purpura

Thrombocytopenic purpura is a condition characterized by low platelet counts, which can lead to easy bruising, bleeding, and purplish skin spots (purpura). It may result from immune dysfunction (immune thrombocytopenic purpura) or other causes, such as medications or infections.

How to Differentiate Thrombocytopenic Purpura from Henoch-Schönlein Purpura Nephritis

While both conditions can cause purpura, thrombocytopenic purpura is due to low platelet counts, whereas Henoch-Schönlein purpura nephritis involves blood vessel inflammation and kidney damage. Thrombocytopenic purpura does not typically cause kidney symptoms like blood or protein in the urine.

A blood test to measure platelet levels can help differentiate the two. Platelet counts are significantly reduced in thrombocytopenic purpura but are usually normal in Henoch-Schönlein purpura nephritis.

Allergic Purpura

Allergic purpura, also known as hypersensitivity vasculitis, is an immune reaction triggered by medications, infections, or other factors, leading to inflammation of small blood vessels. Symptoms may include a purpuric rash, joint pain, and occasionally abdominal pain.

How to Differentiate Allergic Purpura from Henoch-Schönlein Purpura Nephritis

Both conditions can cause a purpuric rash and joint pain. However, allergic purpura is more likely to occur after exposure to a specific trigger, such as a new medication or recent infection, and rarely involves the kidneys. A urinalysis can check for kidney involvement. The absence of blood or protein in the urine suggests allergic purpura rather than Henoch-Schönlein purpura nephritis.

Renal Artery Stenosis

Renal artery stenosis is the narrowing of the arteries that supply blood to the kidneys, leading to high blood pressure and reduced kidney function. It is often caused by atherosclerosis or fibromuscular dysplasia.

How to Differentiate Renal Artery Stenosis from Henoch-Schönlein Purpura Nephritis

Renal artery stenosis does not cause systemic symptoms like a purpuric rash or abdominal pain. Instead, it is associated with resistant high blood pressure and reduced blood flow to the kidneys. Imaging studies, such as Doppler ultrasound, CT angiography, or MR angiography, can confirm renal artery stenosis, findings that are absent in Henoch-Schönlein purpura nephritis.

Glomerulonephritis

Glomerulonephritis refers to inflammation of the glomeruli, the kidney’s filtering units. It can result from autoimmune diseases, infections, or genetic conditions, causing symptoms such as blood in the urine, proteinuria, and swelling.

How to Differentiate Glomerulonephritis from Henoch-Schönlein Purpura Nephritis

Henoch-Schönlein purpura nephritis is a specific type of glomerulonephritis caused by IgA deposits. Other types, such as post-infectious glomerulonephritis or membranous nephropathy, may have similar kidney symptoms but lack systemic features like a purpuric rash or abdominal pain. A kidney biopsy can identify specific patterns of inflammation and antibody deposits, distinguishing Henoch-Schönlein purpura nephritis from other forms of glomerulonephritis.

Improving Henoch-Schönlein Purpura Nephritis: Home Remedies and When to Seek Medical Help

Managing Henoch-Schönlein purpura nephritis often involves a combination of medical treatments and supportive home strategies to ease symptoms and encourage recovery. Below are some practical and effective approaches:

1. Rest: Prioritizing adequate rest is crucial for recovery, as it helps reduce strain on the kidneys, especially in cases of HSP kidney involvement.
2. Hydration: Drinking plenty of water supports kidney function by flushing out toxins, which is essential for maintaining overall kidney health.
3. Dietary Adjustments: Following a low-sodium, kidney-friendly diet can help minimize swelling and protect kidney function, which is vital for managing Henoch-Schönlein purpura kidney disease.
4. Stress Management: Incorporating relaxation techniques such as meditation or yoga may help lower stress levels, potentially reducing inflammation associated with IgA vasculitis nephritis.
5. Adequate Sleep: Ensuring consistent, quality sleep supports the body’s natural healing processes and aids in recovery from HSP nephritis.
6. Avoiding Allergens: Identifying and avoiding potential triggers, such as allergens, can help prevent flare-ups of Henoch-Schönlein purpura symptoms.
7. Gentle Exercise: Low-impact activities like walking or stretching can improve circulation without overexerting the body, which is especially important for those managing HSP complications.
8. Warm Baths: Soaking in warm water can help relieve joint pain and muscle tension, which are common symptoms of Henoch-Schönlein purpura in both adults and children.
9. Herbal Teas: Teas such as chamomile or ginger may provide mild anti-inflammatory benefits that complement other Henoch-Schönlein purpura treatments.
10. Maintaining a Healthy Weight: A balanced diet combined with regular physical activity can reduce stress on the kidneys, improving outcomes for individuals with HSP kidney disease.

If symptoms persist or worsen despite these measures, it’s important to seek medical attention promptly. Telemedicine offers a convenient way to consult healthcare providers from the comfort of your home, ensuring timely diagnosis and treatment for Henoch-Schönlein purpura nephritis.

Living with Henoch-Schönlein Purpura Nephritis: Tips for a Better Quality of Life

Living with HSP nephritis can be challenging, but with proactive management, you can significantly improve your quality of life. Consider these helpful tips:

1. Follow your treatment plan as prescribed by your healthcare provider to effectively manage Henoch-Schönlein purpura symptoms.
2. Schedule regular check-ups to monitor kidney function and address potential HSP complications early.
3. Maintain open communication with your healthcare provider. Telemedicine can simplify routine follow-ups and medication adjustments, making it especially convenient for managing Henoch-Schönlein purpura in both children and adults.
4. Adopt a healthy lifestyle that includes a balanced diet and regular physical activity to support kidney health and overall well-being.
5. Incorporate stress management techniques, such as mindfulness or deep breathing exercises, to help reduce inflammation and improve mental health.
6. Stay informed about your condition by learning more about Henoch-Schönlein purpura causes, available treatments, and potential long-term effects.

By taking these steps, you can take an active role in managing Henoch-Schönlein purpura nephritis, improving your overall health and quality of life.

Conclusion

Henoch-Schönlein purpura nephritis is a complex condition that requires timely diagnosis and comprehensive care. Early treatment is essential to prevent complications and improve long-term outcomes. If you or a loved one are experiencing symptoms of HSP nephritis, our telemedicine practice is here to provide personalized care and support. Schedule a virtual consultation today to receive expert guidance from the comfort of your home.

James Kingsley

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