간질성 폐질환 diffuse interstitial lung disease, DILD

목차

Ⅰ. 간질성 폐질환의 정의

Ⅱ. 간질성 폐질환의 분류

Ⅲ. 임상 양상

Ⅳ. 치료

Ⅴ. 특발성 폐섬유화증(Idiopathic Pulmonary Fibrosis)

Ⅵ. 미만성 간질성 폐렴(Idiopathic interstitial pneumonia)

Ⅶ. 교원성 질환과 동반된 간질성 폐질환

Ⅷ. 기타 간질성 폐질환

본문내용

nosis
Poor
Good
3) Wegener's granulomatosis
(1) Granulomatous necrotizing vasculitis in upper & lower airway and kidney
(2) ANCA (Anti-neutrophil cytoplasmic antibody) : useful monitor of disease activity
4) Goodpasture's syndrome
(1) diffuse pulmonary hemorrhage
(2) progressive glomerulonephritis
(3) circulating anti-GBM Ab, anti-ABM Ab
(4) Young men(18-35 years)
(5) Hemoptysis, hematuria, increased DLco
(6) Treatment : corticosteroid, cytotoxic drug, plasmapheresis
5) Idiopathic pulmonary hemosiderosis
(1) Intermittent diffuse alveolar hemorrhage without evidence of vasculitis, inflammation, granuloma, necrosis
(2) 80% children, 20% adult, male predominance
6) Lymphangioleiomyomatosis
(1) child-bearing age women
(2) immature smooth muscle cell proliferation
(3) dyspnea
(4) chylous pleural effusion(secondary to obstruction of pleural lymphatics)
(5) recurrent pneumothorax (due to rupture of emphysematous cyst)
(6) lung volume increased
(7) Confirm diagnosis : thoracoscopic or open lung biopsy
(8) most patients die of respiratory failure within 10 years of onset of symptom
(9) pathogenesis : hormonal effect
→ LAM occurs predominantly in premenopausal women
accelerated during pregnancy, postpartum period, exogenous estrogen therapy
(10) Treatment : progesteron
7) Diffuse panbronchiolitis
(1) Japan, Korea
(2) Asthma, paranasal sinusitis, diffuse fine nodular pulmonary infiltrates
(3) Treatment : erythromycin
8) Histiocytosis X (Eosinophilic granuloma)
(1) triad
① Eosinophilic granuloma
② Letterer-siwe disease
③ Hand-Shuller-Christian disease
(2) 20 -40 years
(3) cigarette smoking (>90%)
(4) lung, bones, viscera
(5) spontaneous pneumothorax
cystic honeycombing predominantly involving upper lobe
(6) cystic bone lesion(skull, rib, pelvis)
(7) diabetes insipidus : poor prognostic sign
(8) lung volume are normal or increased
mixed obstructive & restrictive pattern
decreased DLco
(9) diagnosis : thoracoscopic or open lung biopsy
EM with BAL : X bodies or Birbeck granules
(10) treatment : corticosteroid
9) Drug-induced ILD
(1) antibiotics : nitrofurantoin, cephalosporin, sulfonamide, penicillin, isoniazid
(2) anti-inflammatory agent : methotrexate, gold, penicillamine
(3) cardiovascular drug
amiodarone
5 - 10 %에서 발생
risk factor ; maintenance dose (less frequent if does < 400 mg/day)
previous pulmonary disease
drug를 계속 사용해야 하는 경우는 corticosteroid trial
(4) antineoplastic agent
bleomycin-induced lung disease
age (>70)
administering oxygen
radiation therapy
multidrug regimen
cumulative does of > 450 units
10) chronic eosinophilic pneumonia
(1) triad
① peripheral, nonsegmental alveolar infiltrate
② rapid resolution within 2 to 4 days after treating with corticosteroid
③ recurrence of CXR abnormalities in same distribution with clinical relapse
(2) "photographic negative of pulmonary edema"
(3) Diagnosis : TBLB & BAL
open lung biopsy is rarely required
(4) Treatment ; corticosteroid
수 시간내에 호전되며 CXR는 2-4일에 clearing
6 - 12개월간 치료를 요하며 relapse rate가 높다

키워드

간질,   폐,   dild,   심장,   혈관,   혈압