What is polyarteritis nodosa? morphology and clinical features?

 Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is a systemic vasculitis of small- or medium-sized muscular arteries; it typically involves the renal and visceral vessels and spares the pulmonary circulation. There is no association with ANCAS, but one-third of patients have chronic hepatitis B infection, which leads to the formation of immune complexes containing hepatitis B antigens that deposit in affected vessels. The cause is unknown in the remaining cases.

Pathogenesis of PAN

  • Immune complex–mediated vasculitis, often linked to Hepatitis B.

  • Immune complexes deposit in medium-sized arteries.

  • Activate the complement system → recruit neutrophils.

  • Causes fibrinoid necrosis and inflammation of the vessel wall.

  • Leads to thrombosis, aneurysms, and organ ischemia.

  • Not associated with ANCA.

Morphology of PAN

Classic PAN is a segmental transmural necrotizing inflammation of small- to medium-sized arteries, often with superimposed thrombosis. Kidney, heart, liver, and gastrointestinal tract vessels are affected in descending order of frequency. Lesions usually involve only part of the vessel circumference and have a predilection for branch points. Impaired perfusion may lead to ulcerations, infarcts, ischemic atrophy, or hemorrhages in the distribution of affected vessels. The inflammatory process also weakens the arterial wall, leading to aneurysms and rupture.

What is polyarteritis nodosa?


In the acute phase, there is transmural mixed inflammatory infiltrate composed of neutrophils and mononuclear cells, frequently accompanied by fibrinoid necrosis and luminal thrombosis. Older lesions show fibrous thickening of the vessel wall extending into the adventitia. Characteristically, all stages of activity (from early to late) coexist in different vessels or even within the same vessel, suggesting ongoing and recurrent pathogenic insults.

Clinical Features

PAN is primarily a disease of young adults but can occur in all age groups. The clinical course typically is episodic, with long symptom-free intervals:

  • Systemic symptoms: Fever, weight loss, and fatigue.

  • Renal: Hypertension, hematuria (no glomerulonephritis).

  • Gastrointestinal: Abdominal pain, bleeding, infarction.

  • Musculoskeletal: Myalgia, arthralgia.

  • Neurological: Mononeuritis multiplex (asymmetric neuropathy).

  • Skin: Livedo reticularis, nodules, ulcers, purpura.

  • Cardiac: Myocardial ischemia (due to coronary vasculitis).

Untreated, PAN typically is fatal; however, with immunosuppression, 5-year survival is close to 80%. Relapse occurs in up to 25% of the cases, more often in non-HBV-associated cases than those that follow HBV infection. The latter have a better long-term prognosis.

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