Overview
Primary polyarteritis nodosa (also called classic polyarteritis nodosa or PAN) is a rare disease that causes inflammation of medium-sized blood vessels (arteries) throughout the body. Unlike some other forms of polyarteritis nodosa that occur alongside infections like hepatitis B, primary PAN happens without a known underlying cause. The inflammation damages the walls of arteries, which can lead to narrowing, weakening, or blockage of blood vessels. This reduces blood flow to important organs and tissues. The disease can affect many parts of the body, including the kidneys, skin, nerves, muscles, joints, and digestive system. Common symptoms include fever, weight loss, muscle and joint pain, skin rashes or nodules, numbness or tingling in the hands and feet, abdominal pain, and high blood pressure. In severe cases, damaged blood vessels can form small bulges (aneurysms) that may rupture and cause internal bleeding. Treatment focuses on controlling the inflammation and preventing organ damage. Doctors typically use corticosteroids like prednisone as a first-line treatment. For more severe cases, stronger immune-suppressing medications such as cyclophosphamide or azathioprine may be added. With early diagnosis and proper treatment, many patients can achieve remission, though relapses can occur. Regular monitoring by a specialist team is essential to manage the disease and watch for complications.
Also known as:
Key symptoms:
Unexplained feverUnintentional weight lossFatigue and general feeling of being unwellMuscle aches and painJoint painSkin rashes, lumps, or purplish spotsNumbness or tingling in hands and feetAbdominal pain, especially after eatingHigh blood pressureKidney problemsTesticular pain in menSkin ulcers that are slow to healWeakness in arms or legsNight sweatsLivedo reticularis (net-like purple skin pattern)
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 Primary 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 Primary polyarteritis nodosa.
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Disease timeline:
New trial: Facilitators and Barriers in Neuroscience-based Pain Education Programmes in Primary Care Physiother
Phase NA trial recruiting. Survey
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 polyarteritis nodosa, and which organs are affected?,What treatment plan do you recommend, and how long will I need to be on medication?,What side effects should I watch for with my medications?,How often will I need blood tests and follow-up visits?,What are the signs of a relapse, and what should I do if I notice them?,Are there any activities, foods, or medications I should avoid?,Should I see any other specialists for my condition?
Common questions about Primary polyarteritis nodosa
What is Primary polyarteritis nodosa?
Primary polyarteritis nodosa (also called classic polyarteritis nodosa or PAN) is a rare disease that causes inflammation of medium-sized blood vessels (arteries) throughout the body. Unlike some other forms of polyarteritis nodosa that occur alongside infections like hepatitis B, primary PAN happens without a known underlying cause. The inflammation damages the walls of arteries, which can lead to narrowing, weakening, or blockage of blood vessels. This reduces blood flow to important organs and tissues. The disease can affect many parts of the body, including the kidneys, skin, nerves, musc
How is Primary polyarteritis nodosa inherited?
Primary polyarteritis nodosa follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Primary polyarteritis nodosa typically begin?
Typical onset of Primary polyarteritis nodosa is adult. Age of onset can vary across affected individuals.
Which specialists treat Primary polyarteritis nodosa?
1 specialists and care centers treating Primary polyarteritis nodosa are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.