From the Guidelines
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that primarily affects medium-sized vessels, presenting with systemic symptoms such as fever, weight loss, and various organ-specific manifestations. The most common clinical presentations include:
- Neurologic manifestations such as mononeuritis multiplex and peripheral neuropathy
- Cutaneous manifestations such as nodules and livedo reticularis
- Renal manifestations such as hypertension
- Gastrointestinal manifestations such as abdominal pain 1 These symptoms can develop gradually over weeks to months, and diagnosis is generally confirmed by tissue biopsy of an affected organ or angiography if tissue biopsy cannot be obtained. The diagnosis of PAN requires a high index of suspicion as these symptoms can mimic many other conditions. Typical histologic findings include mixed-cell inflammatory infiltrates in the vessel wall and fibrinoid necrosis, with an absence of granulomas and giant cells 1. Findings on angiography include saccular or fusiform aneurysms and stenotic lesions in the mesenteric, hepatic, and renal arteries and their subsequent branches. Given the potential severity of PAN, with a mortality rate of 40% at 5 years for severe cases 1, prompt recognition and treatment are crucial to improve outcomes.
From the Research
Polyarteritis Nodosa Symptoms
- Weight loss, fever, peripheral neuropathy, renal, musculoskeletal, gastrointestinal tract and/or cutaneous involvement(s), hypertension and/or cardiac failure are the main manifestations of polyarteritis nodosa (PAN) 2
- Peripheral neuropathy is one of the most frequent and earliest symptoms, affecting 50% to 75% of PAN patients 2
- Central nervous system involvement affects only 2% to 10% of PAN patients, often late during the disease course 2
- Some patients may experience disease relapse, with 46 (37%) of patients experiencing a relapse in one study 3
Treatment and Prognosis
- Treatment relies on combining corticosteroids and an immunosuppressant (mainly cyclophosphamide) in patients with poor prognoses 2, 4
- Biologic drugs, such as anti-tumor necrosis factor (TNF)-α agents, may be effective in inducing and maintaining remission in patients with PAN 5
- The 1-year and 5-year survival rates for patients with PAN or microscopic polyangiitis (MPA) were 99% and 92%, respectively, in one study 3
- Disease-free survival was significantly lower for patients with MPA than for those with PAN (P = 0.046) 3