medical decision support medical decision support
Sign In

American College of Rheumatology (ACR) Criteria for the Diagnosis of Churg-Strauss Syndrome

Purpose: Purpose: To evaluate a patient for clinical features of the Churg-Strauss Syndrome (CSS, or allergic granulomatosis and angiitis).

Description

The Churg-Strauss syndrome (allergic granulomatosis and angiitis) involves destructive inflammation of small to medium muscular arteries with fever, hypereosinophilia and a frequent association with asthma. The characteristic "allergic granulomas" are composed of necrotic, eosinophilic exudates, severe fibrinoid collagen changes, and granuloma formation.


 

Criteria

Comment

asthma

history of wheezing or diffuse high-pitched rales on expiration

eosinophilia > 10%

 

mononeuropathy or polyneuropathy

development of mononeuropathy, multiple mononeuropathies, or polyneuropathy (i.e., glove and stocking distribution) attributable to a systemic vasculitis

pulmonary infiltrates, nonfixed

migratory or transitory pulmonary infiltrates on radiographs (not including fixed infiltrates)

paranasal sinus abnormality

history of acute or chronic paranasal sinus pain or tenderness or radiographic opacification of the paranasal sinuses

extravascular eosinophils

biopsy including artery, arteriole or venule, showing accumulations of eosinophils in extravascular areas

 

Other findings:

(1) elevated serum IgE levels

(2) may have antineutrophil cytoplasmic antibodies (ANCA)

(3) renal involvement less common than gastrointestinal or cardiac involvement

(4) skin manifestations such as palpable purpura, maculopapular rash or subcutaneous nodules

 

Interpretation:

• The presence of 4 or more criteria suggests the diagnosis of Churg-Strauss syndrome.

 

Performance:

• The sensitivity is 85% and specificity of 99.7%.


To read more or access our algorithms and calculators, please log in or register.

Specialty

Objective

ICD-10