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American College of Rheumatology (ACR) Criteria for the Diagnosis of Hypersensitivity Vasculitis

Purpose: To diagnose hypersensitivity vasculitis based on certain clinical findings and the American College of Rheumatology criteria.

Description

Hypersensitivity vasculitis is a group of clinical syndromes featuring immune complex deposition about small blood vessels. The precipitating antigen may be exogenous or endogenous; the antigen may be related to drugs, food, bacteria, viruses, immunoglobulin, DNA or tumor products.


Histologic features:

(1) involvement of small blood vessels (arterioles, capillaries, venules)

(2) infiltration of polymorphonuclear leukocytes and leukocytoclasia (nuclear fragmentation)

 

Criteria

Comment

age at disease onset > 16 years

 

medication at disease onset

a medication was taken at the onset of symptoms that may have been a precipitating factor

palpable purpura

slightly elevated purpuric rash over one or more areas of the skin; the rash does not blanch with pressure and is not related to thrombocytopenia

maculopapular rash

flat and raised lesions of various sizes over one or more areas of the skin

biopsy involving arteriole and venule

granulocytes in a perivascular or extravascular location

 

Additional findings:

(1) erythema multiforme

(2) livido reticularis

(3) urticaria

(4) symmetrical pattern of involvement, usually in dependent portions of the body

(5) increased erythrocyte sedimentation rate and acute phase reactants

(6) cryoglobulins may be present

(7) decreased complement levels

 

Interpretation:

• The presence of 3 or more criteria indicates that the patient has hypersensitivity vasculitis.

 

Performance:

• The sensitivity of schema is 71%; specificity is 83.9%


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Specialty

Objective

ICD-10