CV

Polyarteritis Nodosa

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Polyarteritis Nodosa, Periarteritis Nodosa, Panarteritis Nodosa, Kussmaul Disease, Cutaneous Polyarteritis Nodosa

  • See Also
  1. Vasculitis
  • Epidemiology
  1. Prevalence: May approach 33 Million cases worldwide
  2. Gender: Slight male predominance
  3. Age: 30-40 years old
  • Classification
  1. ANCA negative Vasculitis
  2. Now differentiated from microscopic polyangitis
  • Pathophysiology
  1. Medium vessel Vasculitis involving muscular arteries
  2. Can affect any organ (esp. Kidney, skin, joint, Muscle, nerve, Intestine), but typically does not involve the lung
  3. May be limited to the skin (Cutaneous Polyarteritis Nodosa) with or without mild systemic symptoms
    1. More than half of cutaneous cases progress to extracutaneous involvement
  4. Effects are related to vessel wall thickening, vessel narrowing and ultimately arterial Occlusion with secondary tissue infarction
    1. Medium sized arteries with immune complex deposition
    2. Immune deposits contain C3 and IgM
  • Associated Conditions
  1. Hepatitis B Infection
  2. Hepatitis C Infection
  3. Hairy Cell Leukemia
  • Symptoms
  1. Fever
  2. Weight loss
  3. Hypertension
  4. Abdominal Pain
  5. Melena
  6. Peripheral neuritis
  • Signs
  • Skin
  1. Tender erythematous Nodules similar to Erythema Nodosum
  2. Palpable Purpura
  3. Livedo Reticularis
  4. Skin Ulceration
  5. Vesicobullous lesions
  • Signs
  • Systemic
  1. Renal Insufficiency
  2. Hypertension
  3. Mononeuropathy Multiplex
  4. Neuropathy
  5. Myopathy
  6. Mesenteric arteritis
  7. Coronary ischemia
  • Labs
  1. Vasculitis labs (evaluate differential diagnosis)
  2. ANCA negative
  3. Serum Creatinine
  4. Creatinine Phosphokinase (CPK)
  5. Liver Function Tests
  6. Hepatitis B Surface Antigen
  7. Anti-Hepatitis C Antibody
  8. Urinalysis
  9. C-Reactive Protein (negative result would suggest alternative diagnosis)
  • Imaging
  1. Chest XRay (evaluate differential diagnosis)
  • Diagnosis
  1. Tissue Biopsy (Punch Biopsy, Incisional Biopsy)
    1. Perivascular inflammation with White Blood Cell infiltrate
  2. Imaging (CT Angiogram, MR Angiogram, Echocardiogram)
  • Differential Diagnosis
  1. See Vasculitis
  2. Infection
    1. Endocarditis
    2. Mycotic aneurysm
    3. Hepatitis B Infection
    4. Hepatitis C Infection
    5. HIV Infection
  3. Non-infectious causes
    1. Atypical thromboembolic conditions (e.g. atrial myoma, antiphospholipid Antibody syndrome)
    2. Fibromuscular dysplasia
  • Management
  1. Antiinflammatory Agents
    1. Colchicine
    2. Dapsone
    3. Cyclophosphamide
  2. Biologic Agents
    1. Tumor Necrosis Factor Inhibitors
    2. Interleukin 6 inhibitors
  3. Corticosteroids
    1. Indicated in acute flares on slow taper (e.g. 60 mg to 5-6 mg)
    2. May need to be continued longterm at low dose
  4. Hepatitis B Related Polyarteritis Nodosa
    1. Chronic Hepatitis B Antiviral Therapy
    2. Plasma exchange
  • References
  1. Merkel (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11
  2. Hocevar (2021) Curr Rheumatol Rep 23(3): 14 +PMID:33569653 [PubMed]