Polyarteritis nodosa

Background

  • Necrotizing vasculitis of small- and medium-sized blood vessels
  • Skin, musculoskeletal, CNS, and GI tract (spares lung)
  • Predilection to arterial bifurcations and branch sites
  • Microaneurysm, thrombosis, emboli, organic ischemia, and infarction
  • Etiology: Idiopathic, HBV, HCV, hairy cell leukemia

Evaluation

Clinical Features

  • Cutaneous lesion + adult onset hypertension
  • Men > women (2:1)
  • Peak age 40-60s
  • Systemic: Fatigue, weight loss, weakness, fever, arthralgia
  • Cutaneous lesions (1/3 patients)
    • Tender erythematous nodules
    • Palpable purpura (fingers, ankles, malleoli, pretibial)
    • Digital cyanosis
    • Splinter hemorrhages
    • Livedo reticularis
  • Renovascular arteritis → hypertension
  • Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
  • Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation)
  • Myocardial ischemia and heart failure
  • Myalgia (elevated CK)

Classification

  • American College of Rheumatology 10 criteria (at least 3, has 82% sensitivity and 87% specificity)
    • Unexplained weight loss greater than 4kg
    • Livedo reticularis
    • Testicular Pain or tenderness
    • Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy
    • Mononeuropathy or polyneuropathy
    • New-onset diastolic blood pressure > 90mmHg
    • Elevated serum BUN (>40mg/dL or 14.3mmol/L) or creatinine (>1.5mg/dL or 132 mmol/L)
    • Evidence of HBV infection (serology)
    • Characteristic arteriographic abnormalities not resulting fro noninflammatory disease processes
    • Biopsy of small- or medium-sized artery containing polymorphonuclear cells

Workup

  • Definitive: Tissue biopsy
  • Labs:
    • Cr, CK, LFT (elevated)
    • CBC (Leukocytosis, normochromic anemia, thrombocytosis)
    • Hepatitis serology
    • Urinalysis (proteinuria)
    • ESR/CRP
  • Imaging
    • Angiography preferred: aneurysm or stenosis of medium-sized vessels
    • Arteriograms
    • CT/MRI
  • To consider: CXR, blood cultures, autoimmune serologic testing (ANCA, ANA, RF) to rule out other diseases

Differential Diagnosis

Management

  • Rheumatology consult
  • May warrant surgical intervention if abdominal involvement
  • Corticosteroid:
  • Immunosuppressive agent for moderate to severe
  • ACEI or ARB for hypertension

References

  • Reference: Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1539-1540.
  • Merkel PA, et al. Clinical manifestations and diagnosis of polyarteritis nodosa in adults. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
  • Merkel PA, et al. Treatment and prognosis of polyarteritis nodosa. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
  • Jacobs-Kosmin, D. (2014, Dec 12). Polyarteritis Nodosa. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/330717-overview.