Polyarteritis Nodosa (PAN)
Polyarteritis Nodosa (PAN): Overview, Symptoms, and Management
Polyarteritis nodosa (PAN) is a rare systemic vasculitis characterized by inflammation and necrosis of medium-sized arteries, leading to tissue ischemia and organ damage. Untreated PAN can result in life-threatening complications, but early intervention improves outcomes.
Causes and Risk Factors
- Autoimmune Dysregulation: Immune system mistakenly attacks arterial walls, causing inflammation and weakening vessel integrity13.
- Infections: Strongly linked to hepatitis B or C (especially in injection drug users)56.
- Other Triggers: Genetics, blood disorders, or unknown factors6.
- Risk Factors: Middle-aged adults (peak 40–50 years), hepatitis B/C infection, and drug use25.
Symptoms of PAN
PAN affects multiple organs, with symptoms varying by location:
| Organ System | Symptoms |
|---|---|
| General | Fever, fatigue, weight loss, muscle/joint pain127. |
| Skin | Rashes, subcutaneous nodules, livedo reticularis, necrotic ulcers47. |
| Nervous System | Peripheral neuropathy (numbness, weakness), mononeuritis multiplex47. |
| Kidneys | Hypertension, proteinuria, renal failure46. |
| Gastrointestinal | Abdominal pain, bowel ischemia/perforation, blood in stool14. |
| Cardiovascular | Chest pain, heart failure, myocardial infarction46. |
Note: PAN typically spares the lungs46.
Diagnosis
Diagnosis involves clinical evaluation and imaging:
- Biopsy: Shows necrotizing inflammation in arteries (definitive test)26.
- Angiography: Reveals aneurysms (“rosary sign”) and arterial narrowing46.
- Blood Tests: Elevated ESR/CRP, hepatitis B/C screening15.
- Exclusion: Rule out mimics (e.g., granulomatosis with polyangiitis)2.
Treatment
- Immunosuppressants:
- Corticosteroids (prednisone) for acute inflammation26.
- Cyclophosphamide or azathioprine for severe cases26.
- Antiviral Therapy: For hepatitis B/C-associated PAN5.
- Supportive Care: Blood pressure control, pain management6.
Complications
- Organ Failure: Renal insufficiency, bowel perforation, stroke47.
- Aneurysm Rupture: Risk of hemorrhage6.
- Chronic Neuropathy: Persistent numbness or weakness4.
Prognosis
- Untreated: 5-year survival rate 13%4.
- Treated: 5-year survival improves to 80% with early immunosuppression46.
Conclusion
Polyarteritis nodosa (PAN) is a medical emergency requiring prompt diagnosis and aggressive therapy. Early treatment with immunosuppressants and antiviral agents (if hepatitis-linked) significantly reduces morbidity and mortality.
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