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American College of Rheumatology (ACR) Criteria for Polyarteritis Nodosa

Purpose: To diagnose polyarteritis nodosa based on the presence of certain clinical findings.

Description

Polyarteritis nodosa (PAN) is a systemic vasculitis which usually does not affect the aorta, primary branches of the aorta or elastic pulmonary arteries. The presentation may be vague and nonspecific, with malaise, fatigue, abdominal pain, fever, weight loss, peripheral nerve deficits and stroke.


Patient selection:  Patients with rheumatoid arthritis or systemic lupus erythematosus with vasculitis were excluded from the study population used to develop the criteria. These diagnoses should be excluded prior to evaluating the patient for PAN.

 

Criteria

Comment

weight loss >= 4 kilograms

weight loss since illness began, not due to dieting or other factors

livedo reticularis

mottled reticular pattern over the skin of portions of the extremities or torso

testicular pain or tenderness

pain or tenderness not due to infection, trauma or other causes

myalgias, weakness or leg tenderness

diffuse myalgias (excluding shoulder and hip girdle), or weakness of muscles, or tenderness of leg muscles

mononeuropathy or polyneuropathy

may include multiple mononeuropathies

diastolic blood pressure > 90 mm Hg

 

elevated BUN or creatinine

BUN > 40 mg/dL or creatinine > 1.5 mg/dL, not due to dehydration or urinary tract obstruction

hepatitis B antigenemia

presence of hepatitis surface antigen in serum, with immune complex formation

arteriographic abnormalities not resulting from arteriosclerosis, fibromuscular dysplasia or other noninflammatory causes

arteriogram showing aneurysms or occlusion of visceral arteries, not due to noninflammatory causes

Biopsy specimens of small or medium muscular artery show granulocytes or granulocytes and mononuclear leukocytes in the artery wall.

Transmural pleomorphic cellular infiltrate, fibrinoid necrosis, thrombosis, and aneurysm formation.

 

Interpretation:

• The presence of 3 or more criteria suggests the diagnosis of polyarteritis nodosa.

• Some authorities classify patients with small artery involvement as having microscopic polyangiitis (MPA).

 

Performance:

• The criteria have a sensitivity 82.2% and specificity 86.6%.


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Specialty

Objective

ICD-10