Epidemiology

• Mean age of onset: 45 y/o
• M:F ratio: 2.5:1
• Associated with: Hepatitis B

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Physiology

• Systemic necrotizing vasculitis of small and medium-sized arteries (with neutrophilic inflammation of blood vessels and perivascular space)
• Tissue ischemia and infarction/aneurysmal dilatation of blood vessels

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Diagnosis

• CBC: leukocytosis (75%)
• HBsAg: positive in 30% of cases
• ABG: elevated A-a gradient
• PFT’s: decreased DLCO, increased Vd/Vt ratio
• p-ANCA: may be positive (diffuse granular pattern)
• Angiogram: aneurysms of small and medium-sized arteries
• Skin Bx (vasculitic lesions): diagnostic

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Clinical Manifestations

Rheumatologic Manifestations (64% of cases)

• Myalgias

Renal Manifestations (60% of cases)

• Vasculitis
• Glomerulonephritis
• Hypertension (60% of cases)

Hepatic Manifestations (44% of cases)

• xxx

Dermatologic Manifestations (43% of cases)

• Cutaneous Nodules
• Skin Ulcers
• Livedo Reticularis

Cardiac Manifestations (36% of cases)

• Coronary Arteritis

Neurologic Manifestations (23% of cases)

• xxxx

Pulmonary Manifestations (usually spared)

• Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage): rare case reports

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Treatment

• Cytotoxic Agents

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References

• Hepatitis B-Related Polyarteritis Nodosa Complicated by Pulmonary Hemorrhage. Chest 2001; 119: 1608-1610