Polyarteritis Nodosa (PAN)

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)


Treatment

  • Cytotoxic Agents

References

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