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