Contents

Differential Diagnosis
The differential diagnosis of vasculitis entities are summarized in tables 3-5 and in figure 62. The initial clinical appearances of various other diseases can mimick those of systemic vasculitis: bacterial endocarditis, Lyme disease, hypertensive arteriopathy, effects of vasoconstrictive drugs (e.g. ergotism), thoracic outlet syndrome, cholesterole embolism, and right atrial myxoma.

Diagnostic Criteria of Vasculitis Entities (ACR 1990 Criteria)
Diagnostic clinical criteria for main entities of PSV are summarized in table 8.
For case presentations connect to University of Iowa Virtual Hospital and define search as "hypersensitivity vasculitis":

http://www.vh.org

Table 8: ACR 1990 Diagnostic Criteria of Vasculitides

Giant Cell Arteritis
Patient below 50 years of age at initial diagnosis
Recently developed head aches
Pathologic clinical signs at temporal arteries (pain, pulselessness)
Significantly elevated ESR
Diagnostic arterial biopsy
At least 3 criteria must be present for diagnosis
Sensitivity 93.5%, specificity 91.2%
Takayasu's arteritis
Patient below 40 years or age at initial diagnosis
Claudicatio of extremities
Reduced brachial pulse
Difference in blood pressure between R & L arm >10 mmHg
Bruising above a.subclavia and/or aorta
Arteriographic pathology
At least 3 criteria must be present for diagnosis
Sensitivity 90.5%, specificity 97.8%
Panarteritis nodosa (classical)
Weight loss of ca. 4 kg
Livedo reticularis
Pain or swelling of testicle
Myalgy, muscular weakness, or polyneuropathy
Mononeuropathy of polyneuropathy
Diastolic blood pressure >90 mmHg
Elevated BUN or kreatinine
Proven hepatitis B/C virus infection
Angiographic signs of aneurysms or vascular occlusion
Demonstration of granulocytes in small or medium size vessels
At least 3 of 10 criteria must be present for diagnosis

Kawasaki's disease
Fever of unknown origin for at least 5 days
Changes at extremities such as plantar - or palmar erythema, edema of wrist or periungually
with desquamation (during reconvalescence)
Changes at mucous membranes such as cheilitis, pharyngitis, glossitis
Polymorphous exanthema
Acute non-suppurative lymphadenopathy (at least 1 lymph node >1.5 cm in diameter)
Besides fever, four of the other criteria must be positive for diagnosis

Thromboangiitis obliterans (Buerger's disease)
Age of patient >50 years
History of smoking
Peripheral vascular obstructive disease (beyond knee or elbow)
And three additional criteria such as:
- thrombophlebitis saltans/migrans (acute or in case history)
- involvement of upper extremity
- characteristic angiography

Wegener's disease
Inflammation of nose or mouth (ulcerative, hemorrhagic, purulent)
Pulmonary infiltrations in Xray (focal, cavernous, "fixed")
Nephritic urine sediment (>5 erythrocytes/field, eryhtrocytic casts)
Histology: granulomatous inflammation (affecting vessel wall and perivascular)
At least 2 of these four criteria must be present for diagnosis
Sensitivity 88%, specificity 92%

Churg-Strauss syndrome
Asthma bronchiale
Eosinophilia ( >10% in blood cell count)
Allergy
Mono-/polyneuropathy
Pulmonary infiltrates (migrant, transitory)
Changes in paranasal sinuses
Histology: eosinophilic granulomatous vasculitis and perivasculitis
At least 4 of these six criterial must be present for diagnosis
Specificity 99%, sensitivity 85%

Schoenlein-Henoch's purpura
Patient >20 years of age at initial diagnosis
Palpable purpura
[angina abdominis ]
Biopsy Biopsy : granulocytes in vessel wall
At least 2 criteria must be present for diagnosis
Sensitivity 87.1%, specificity 87.7%

Cutaneous leukocytoclastic angiitis
Patient >16 years of age at initial diagnosis
Medical treatment at occurrence of initial symptoms
Palpable purpura
Maculo-papulous exanthema
Biopsy Biopsy : typical arteriolitis & nulitis
At least 3 criteria must be present for diagnosis
Sensitivity 71%, specificity 83.9%