대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Courtesy

S

Lab

Hematuria (+++), C-ANCA (+), 1:320

Differential Diagnosis

Tumor

Lymphoma

Bronchioloalveolar cell carcinoma, multifocal

Metastasis

Infection

Tbc

Fungal infection(Aspergillosis, mucormycosis, candidiasis)

Noninfectious disease

Vasculitis( Wegener’s granulomatosis, Churg-strauss syndrome)

Inflammatory disease

PIE (pulmonary infiltration with eosinophilia)

Reviewy

The American College of Rheumatology 1990 criteria

1.Abnormal urinary sediment

2. Abnormal findings on chest radiograph

3. Oral ulcers or nasal discharge

4. Granulomatous inflammation on biopsy

sensitivity of 88.2%

specificity of 92.0%

(Leavitt et al. Arthritis Rheum 1990)



Chapel Hill Consensus (CHC) Definition

1. Respiratory tract involvement with necrotizing vasculitis affecting small to medium sized vessels

2. Necrotizing glomerulonephritis: common

3. Cytoplasmic-pattern ANCA(C-ANCA): very sensitive marker (>90%)

(Jennette et al., Arthritis Rheum 1994)



Clinical Manifestations

1. Commonly insidious, Sometimes, acute and fulminating course

2. Nonspecific symptoms: fever, malaise, weight loss, and fatigue

3. Frequency of organ involvement:

Lung (94%)

Paranasal sinuses (91%)

Kidney (85%)

Joints (67%)

Eye (58%)

Skin (45%)



Imaging findings
1. Nodules or masses seen in 27 of 30 (90%) patients:

Multiple in 23 (85%)

Bilateral in 18 (67%)

Subpleural in 24 (89%)

Peribronchovascular in 11 (41%)

2. Bronchial wall thickening

Segmental or subsegmental bronchi in 22 (73%)

Large airways were abnormal in 9 (30%)

Patchy areas of consolidation in 7 (23%)

3. Ground glass opacity in 7 (23%)

(Lee KS et al., Eur Radiol 2003)



Differential diagnosis
1. Churg-Strauss syndrome from WG

Asthma (only rarely seen in WG)

Much higher cardiac involvement (up to 47%)

Less severe renal and sinus disease

Churg-Strauss syndrome: p-ANCA (40-60%), eosinophilia (80%)

Wegener's granulomatosis: c-ANCA (90%)

Keywords

Lung, Vasculitis, ANCA associated vasculitis, Wegener's Granulomatosis