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Polyarteritis Nodosa

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Description: Polyarteritis nodosa is a rare autoimmune disease that causes inflammation of the small and medium-sized arteries, leading to organ damage.
Type: Polyarteritis nodosa is a type of vasculitis. It is not typically considered a hereditary disease, and there is no specific mode of genetic transmission associated with it. The exact cause is often unknown, but it may sometimes be associated with infections or other underlying conditions.
Signs And Symptoms: PAN may affect nearly every organ system and thus can present with a broad array of signs and symptoms. These manifestations result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system. Constitutional symptoms are seen in up to 90% of affected individuals and include fever, fatigue, weakness, loss of appetite, and unintentional weight loss.Skin: The skin may show rashes, swelling, necrotic ulcers, and subcutaneous nodules (lumps). Skin manifestations of PAN include palpable purpura and livedo reticularis in some individuals.Neurologic system: Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy). Peripheral nerves are often affected, and this most commonly presents as mononeuritis multiplex, which is the most common neurologic sign of PAN. Mononeuritis multiplex develops in more than 70% of patients with polyarteritis nodosa because of damage to arteries supplying large peripheral nerves. Most cases are marked by asymmetric polyneuropathy, but progressive disease can lead to symmetric nerve involvement. Central nervous system involvement may cause strokes or seizures.Renal system: Kidney involvement is common and often leads to death of parts of the kidney. Involvement of the renal artery, which supplies the kidneys with highly oxygenated blood, often leads to high blood pressure in about one-third of cases. deposition of protein or blood in the urine may also be seen. Almost all patients with PAN have renal insufficiency caused by renal artery narrowing, thrombosis, and infarctions.Cardiovascular system: Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis).Gastrointestinal system: Damage to mesenteric arteries can cause abdominal pain, mesenteric ischemia, and bowel perforation. Abdominal pain may also be seen.Musculoskeletal system: Muscle and joint aches are common.
Prognosis: Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis that affects medium-sized arteries, leading to organ damage. Prognosis for PAN has improved with prompt diagnosis and appropriate treatment, but it remains a serious condition. Without treatment, the disease can be fatal. With modern therapies, many patients achieve remission, although they may require ongoing management to control disease activity and prevent relapse. Factors affecting prognosis include the extent and severity of organ involvement, and the patient's response to treatment. Early diagnosis and intervention are crucial for the best possible outcome.

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Onset: The onset of polyarteritis nodosa can be variable and is often insidious. It typically develops over weeks to months. Symptoms may emerge gradually, making early diagnosis challenging.
Prevalence: The prevalence of polyarteritis nodosa is relatively low, estimated to be about 2 to 9 cases per million people per year. It is considered a rare disease.
Epidemiology: The condition affects adults more frequently than children and males more frequently than females. Most cases occur between the ages of 40 and 60. Polyarteritis nodosa is more common in people with hepatitis B infection.
Intractability: Yes, polyarteritis nodosa (PAN) is often considered an intractable disease due to its chronic nature and the potential for severe complications. It requires long-term management and treatment to control symptoms and prevent progression, often involving immunosuppressive and anti-inflammatory medications. Despite treatment, some patients may experience recurrent or persistent disease.
Disease Severity: Polyarteritis nodosa is a serious and potentially life-threatening disease if left untreated. Disease severity can vary depending on the extent of organ involvement. It commonly affects medium-sized arteries, leading to damage in organs like the kidneys, heart, and gastrointestinal tract. Prompt and aggressive treatment is crucial to manage symptoms and improve prognosis.
Healthcare Professionals: Disease Ontology ID - DOID:9810
Pathophysiology: Polyarteritis nodosa is characterized by systemic necrotizing inflammation of small and medium-sized arteries. It primarily affects the vessel walls, leading to vessel occlusion and tissue ischemia. The exact cause is often unknown, but it can be associated with hepatitis B virus (HBV) infection. Immune complexes deposited in vessel walls trigger the inflammatory response, leading to fibrinoid necrosis. The inflammation can result in aneurysms, thrombosis, and subsequent tissue damage in various organs, including the kidneys, heart, liver, and gastrointestinal tract.
Carrier Status: Polyarteritis nodosa is not a condition associated with a carrier status because it is not typically considered a genetic or heritable disease. It is a rare systemic necrotizing vasculitis that primarily affects medium-sized arteries.
Mechanism: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that primarily affects medium-sized arteries. Its mechanism involves inflammation of the arterial wall, leading to vessel damage, thrombosis, and organ ischemia. The exact cause is often idiopathic, but some cases are associated with infections such as hepatitis B.

### Molecular Mechanisms:
1. **Immune Complexes**: One hypothesized mechanism is the deposition of immune complexes in the vascular walls, which can lead to complement activation and subsequent inflammation.
2. **Endothelial Cells**: Activation of endothelial cells by various stimuli (e.g., infection, immune complexes) can express adhesion molecules that attract leukocytes, leading to inflammation and damage.
3. **Cytokines and Chemokines**: Pro-inflammatory cytokines (e.g., TNF-α, IL-1β) and chemokines play a significant role in recruiting and activating immune cells, perpetuating the inflammatory response.
4. **Genetic Factors**: While not fully understood, certain genetic predispositions may increase the risk of developing PAN.
5. **Molecular Signaling Pathways**: Involvement of signaling pathways such as NF-κB, which regulates the expression of inflammatory mediators, contributes to the pathogenesis of PAN.

Understanding these mechanisms provides insight into potential therapeutic targets for managing the disease.
Treatment: Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. When present, underlying hepatitis B virus infection should be immediately treated. In some cases, methotrexate or leflunomide may be helpful. Some patients have entered a remission phase when a four-dose infusion of rituximab is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure.In case of remission, about 60% experience relapse within five years. In cases caused by hepatitis B virus, however, recurrence rate is only around 6%.
Compassionate Use Treatment: Polyarteritis nodosa (PAN) is a rare vasculitis affecting medium-sized arteries. For compassionate use or experimental treatments of PAN, several options have been explored:

1. **Rituximab**: Although primarily used for B-cell non-Hodgkin lymphomas and certain autoimmune diseases, rituximab has been used off-label for PAN, especially in cases refractory to conventional treatments.

2. **Infliximab**: This anti-TNFα monoclonal antibody has also been considered in refractory cases of PAN. Its use is off-label, aiming to reduce inflammation by targeting tumor necrosis factor-alpha (TNFα).

3. **Tocilizumab**: An IL-6 receptor inhibitor typically used in rheumatoid arthritis, tocilizumab has shown promise in some off-label cases, particularly for patients not responding to standard therapies.

These treatments are generally considered when first-line therapies, such as corticosteroids and immunosuppressants (e.g., cyclophosphamide), are ineffective or contraindicated. It is crucial to consult with a healthcare provider experienced in managing PAN to evaluate the suitability and potential risks of these approaches.
Lifestyle Recommendations: Lifestyle recommendations for polyarteritis nodosa include:

1. Medication Adherence: Follow the prescribed treatment regimen strictly to manage inflammation and prevent complications.
2. Regular Monitoring: Schedule frequent follow-ups with a healthcare provider to monitor the disease's progression and adjust treatment as necessary.
3. Healthy Diet: Maintain a balanced diet rich in fruits, vegetables, lean proteins, and whole grains to support overall health.
4. Physical Activity: Engage in regular, moderate exercise as tolerated, but avoid strenuous activities that may exacerbate symptoms.
5. Stress Management: Practice relaxation techniques such as yoga, meditation, or deep-breathing exercises to reduce stress levels.
6. Avoid Smoking and Alcohol: Refrain from smoking and limit alcohol intake as these can exacerbate symptoms and interfere with medications.
7. Infection Prevention: Stay up-to-date with vaccinations and practice good hygiene to lower the risk of infections.

Always consult with your healthcare provider before making any significant changes to your lifestyle or treatment plan.
Medication: For polyarteritis nodosa, common medications used include corticosteroids like prednisone to reduce inflammation, and immunosuppressive drugs such as cyclophosphamide or azathioprine to control the immune system's activity.
Repurposable Drugs: Polyarteritis nodosa (PAN) is a rare, systemic vasculitis of medium-sized arteries which can cause varied organ damage. Repurposable drugs for PAN mainly involve immunosuppressive and anti-inflammatory medications that are commonly used for other autoimmune conditions. These include:

1. **Corticosteroids (e.g., Prednisone)**: Often the first line of treatment to reduce inflammation.
2. **Cyclophosphamide**: An immunosuppressive agent that can be used for severe cases.
3. **Methotrexate**: Often used as a steroid-sparing agent or for less severe cases.
4. **Azathioprine**: Another immunosuppressive drug that may be used for maintenance therapy.
5. **Rituximab**: A monoclonal antibody against CD20, used in some cases particularly when PAN is associated with hepatitis B or resistant to conventional therapy.

These treatments aim to control inflammation and prevent further vascular and organ damage. Proper management typically requires a multidisciplinary approach under the guidance of a rheumatologist or a specialist experienced with vasculitis.
Metabolites: Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis that affects medium and small-sized arteries. The exact etiology remains unknown, and while specific metabolites directly linked to PAN have not been well-characterized, there are some related metabolic disturbances that might be observed. For example:

1. **Elevated Inflammatory Markers:** Common laboratory findings may include elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), which are general markers of inflammation.

2. **Kidney Function Markers:** Since PAN can involve renal arteries, patients might show altered levels of blood urea nitrogen (BUN) and creatinine, indicating kidney dysfunction.

3. **Liver Enzymes:** Elevated liver enzymes (such as ALT and AST) might be seen if there is hepatic artery involvement.

Specific targeted metabolomic studies for PAN are limited; thus, broad spectrum metabolic profiles and inflammatory markers are typically used for monitoring disease activity.
Nutraceuticals: There is currently no substantial evidence to support the use of nutraceuticals in the treatment or management of polyarteritis nodosa. This condition typically requires pharmacological interventions, such as corticosteroids and immunosuppressive agents. Always consult a healthcare professional for appropriate diagnosis and treatment options.
Peptides: Polyarteritis nodosa is a rare systemic vasculitis characterized by inflammation of small and medium-sized arteries, leading to organ and tissue damage. While the exact cause is unknown, it has been associated with infections like hepatitis B. Clinical manifestations can include fever, weight loss, hypertension, renal dysfunction, and neurologic symptoms. The diagnosis is often confirmed through biopsy or angiography. Treatment typically involves immunosuppressive therapies such as corticosteroids and cyclophosphamide. It's important to tailor treatment based on disease severity and organ involvement.