Epidemiology
- Mean age of onset: 45 y/o
- M:F ratio: 2.5:1
- Associated with: Hepatitis B
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
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
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
Treatment
- Cytotoxic Agents
References
- Hepatitis B-Related Polyarteritis Nodosa Complicated by Pulmonary Hemorrhage. Chest 2001; 119: 1608-1610