대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Courtesy

Hanyang University Kuri Hospital

Discussion

Clinical Course
- Abdominal pain - appedectomy
- Hematuria, albuminemia
- Anemia
- Rapid resolution of pulmonary opacity
- Biopsy from appendectomy specimen: Necrotizing small vessel vasculitis, consistent with microscopic polyangiitis

Brief Discussion

Causes of Diffuse Pulmonary Hemorrhage
- Anti-basement membrane antibody disease(Goodpasture’s syndrome)
- Immune complex disease (SLE, WG, MPA, PAN, HS disease, RA, MCTD)
- Idiopathic
- Bleeding disorders
- Drug, chemicals

Microscopic Polyangiitis
- Necrotizing vasculitis affects small vessels
- Pathogenesis unclear, no immune complex deposition
- Involvement: kidney, lung, skin, GI
- P-ANCA (majority), anti-PR3, C-ANCA (occ.)
- Histology: intra-alveolar hemorrhage / hemosiderin-laden macrophage / interstitial edema
- Radiology: patchy, bilateral air-space opacities / pleural effusion (16 %) / pulmonary edema (6 %)
- Tx: corticosteroid and cyclophosphamide
- Prognosis.: high mortality in pts with pulmonary hemorrhage

Keywords

Lung, Vasculitis, Microscopic polyangitis,