Overview
Systemic polyarteritis nodosa (PAN) is a rare disease that causes inflammation and damage to medium-sized blood vessels (arteries) throughout the body. It is also known simply as polyarteritis nodosa or classic polyarteritis nodosa. When blood vessels become inflamed, they can narrow, weaken, or develop small bulges (aneurysms), which reduces blood flow to the organs they supply. This can affect many parts of the body, including the kidneys, skin, nerves, muscles, joints, and digestive system. Common symptoms include fever, weight loss, fatigue, muscle and joint pain, skin rashes or nodules, numbness or tingling in the hands and feet, abdominal pain, and high blood pressure. In some cases, the disease can damage the kidneys, heart, or intestines, which can be life-threatening if not treated promptly. The exact cause of systemic PAN is not fully understood, but it is believed to involve the immune system mistakenly attacking the body's own blood vessels. In some cases, it has been linked to hepatitis B virus infection. Treatment typically involves medications that suppress the immune system, such as corticosteroids (like prednisone) and cyclophosphamide. With early diagnosis and proper treatment, many patients can achieve remission, though relapses can occur. The treatment approach depends on the severity of the disease and which organs are involved.
Also known as:
Key symptoms:
Unexplained feverUnintentional weight lossSevere fatigueMuscle aches and weaknessJoint painSkin nodules or purplish spotsNumbness or tingling in hands and feetAbdominal pain, especially after eatingHigh blood pressureKidney problemsTesticular pain in menSkin ulcers or livedo reticularis (net-like skin discoloration)Nerve pain or weakness in limbsBlood in the urineChest pain or shortness of breath
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Systemic polyarteritis nodosa.
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Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Systemic polyarteritis nodosa.
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Caregiver Resources
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is my PAN, and which organs are affected?,What treatment plan do you recommend, and what are the side effects I should watch for?,How often will I need blood tests and follow-up visits?,Should I be tested for hepatitis B or other infections before starting treatment?,What are the warning signs that I should go to the emergency room?,Is there a chance my disease could go into remission, and how long might treatment last?,Are there any clinical trials or newer treatments I should consider?
Common questions about Systemic polyarteritis nodosa
What is Systemic polyarteritis nodosa?
Systemic polyarteritis nodosa (PAN) is a rare disease that causes inflammation and damage to medium-sized blood vessels (arteries) throughout the body. It is also known simply as polyarteritis nodosa or classic polyarteritis nodosa. When blood vessels become inflamed, they can narrow, weaken, or develop small bulges (aneurysms), which reduces blood flow to the organs they supply. This can affect many parts of the body, including the kidneys, skin, nerves, muscles, joints, and digestive system. Common symptoms include fever, weight loss, fatigue, muscle and joint pain, skin rashes or nodules,
How is Systemic polyarteritis nodosa inherited?
Systemic polyarteritis nodosa follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Systemic polyarteritis nodosa typically begin?
Typical onset of Systemic polyarteritis nodosa is adult. Age of onset can vary across affected individuals.
Which specialists treat Systemic polyarteritis nodosa?
22 specialists and care centers treating Systemic polyarteritis nodosa are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.