Interstitial Lung Disease <14ÆÇ>
1. Á¤ÀÇ ¹× °³¿ä
alveolar wall(septa), perialveolar tissue, supporting structure¸¦ ħ¹üÇÏ´Â ¿©·¯°¡Áö »óȲµé
À» ÅëĪÇÔ. nonmalignant, noninfectious, insidious onset, chronic duration
epithelial cell injury -> air space, alveolar wall inflammation
-> intraluminal & mural alveolitis(acute phase)
-> interstitial fibrosis(chronic phase)
inflammationÀº conducting airway¸¦ ħ¹üÇÒ¼öµµ ÀÖ´Ù.
Áö±ÝÀº BOOPÀÌ ILDÀÇ ÇÑ spectrumÀ¸·Î ¿©°ÜÁø´Ù.
2. ºÐ·ù
known cause vs unknown causeÀÇ 2 groupsÀ¸·Î ³ª´©´Âµ¥
°¢°¢ granulomaÀ¯¹«¿¡ µû¶ó subgroupsÀ¸·Î ³ª´«´Ù.
ILD°¡¿îµ¥ Å« ºÎºÐÀ» Â÷ÁöÇÏ´Â °ÍÀÌ occupational & environmental inhalant exposure
(inorganic dust or noxious gases)ÀÌ´Ù.
unknown causeºÎºÐ ¶ÇÇÑ Å«µ¥ IPF, sarcoidosis, ILD associated with collagen vascular
diseaseµîÀÌ ÀÖ´Ù.
*Tab 259-1
Lung response : alveolitis, interstitial inflammation, fibrosis
1)known cause
Asbestos, Fumes, Gas, Ç×»ýÁ¦, ¹æ»ç¼±, Aspiration pneumonia
drug(antibiotics, amiodarone, gold, chemotherapeutic drugs)
2)unknown cause
IPF(UIP), collagen vascular disease(SLEµî), pulmonary hemorrhagic syndrome
(Goodpasture's syndrome, idiopathic pulmonary hemosiderosis),
Pulmonary alveolar proteinosis, Eosinophilic pneumonia, Amyloidosis
Lung response : as above but with Granuloma
1)known cause
Hypersensitivity pneumonitis(organic dust)
Inorganic dust : beryllium, silica
2)unknown cause
Sarcoidosis, Eosinophilic granuloma
Granulomatous vasculitides : Wegener's granulomatosis, Allergic granulomatosis of
Churg-Strauss
Idiopathic pulmonary fibrosis(IPF) = Cryptogenic fibrosing alveolitis
: nonproductive cough, progressive dyspnea
CXR»ó lower lung reticular or reticulonodular shadow
PFT»ó restrictive patternÀ» º¸ÀδÙ.
1. Immunopathogenesis
* alveolar wall˼ type I pneumocyte, type II pneumocyte, mesenchymal cell,
(fibroblast,macrophaseµî), collagen, adhesive proteoglycan µîÀ¸·Î ÀÌ·ç¾îÁ® ÀÖ´Ù.
* upper or conducting airway´Â ħ¹üÇÏÁö ¾ÊÀ¸³ª resp. bronchiolesÀÇ bronchiolitis´Â
ÀÏÀ¸Å³¼ö ÀÖÀ¸¸ç alveolar unit¸¦ ÈçÈ÷ ħ¹üÇÑ´Ù.
* Ãʱâ - reversible form of alveolar injury
( alveolar & interstitial edema, intraalveolar hyaline membrane formation)
ÁøÇàµÇ¸é - macrophage¿¡ ÀÇÇØ chemokineºÐºñ -> mesenchymal cellÀÚ±Ø
-> fibroblastÁõ½Ä
( Fig 259-1 )
2. ÀÓ»óÀû Ư¡
1) Hx : exertional dyspnea, effort intolerance, dry cough
fatigue, anorexia, wt loss, arthralgiaµ¿¹Ý
2) P/E : Ãʱâ´Â Á¤»ó
¸»±â¿£ cor pulmonale¹ß»ý, pul hypertension(P2 sound¡è), Rt sided heart failure,
RVEF¡é
3) Lab & diagnostic test
¨ç Imaging study
* CXR : diffuse reticular or reticulonodular marking - lower lung
early- hazy "ground glass" appearance of the lower lung field - acute alveolar stage
later- nodular infiltration
end-stage - all lung field linear opacity, cystic & bronchiectatic change(=
honey-combed appearance)
* HRCT : disease activity monitor¿¡ µµ¿ò
* Bx-proven diffuse IPFÁß CXR°¡ Á¤»óÀÎ °æ¿ì°¡ ÀÖ´Ù(14%).
¨è Lab : ESR¡è, circulating immune complex titer, immunoglobulin¡è
¨é PFT
¨ê Bronchoscopy
4-6 transbronchial biopsy½ÃÇà -> definite pathologic diagnosis¸¦ ¾ò´Â°æ¿ì°¡ 1/4.
sarcoidosis¿Í °°Àº diffuse granulomatous interstitial disease´Â
transbronchial biopsy¸¸À¸·Î Á¶Á÷Áø´Ü¿¡ ÃæºÐÇÏ´Ù(80%).
¨ë Lung biopsy
transbronchial Bx·Î Áø´Ü¿¡ ÇÊ¿äÇÑ ÃæºÐÇÑ Á¶Á÷À» ¾òÀ»¼ö ¾ø´Ù¸é
thoracoscopy-guided or open lung biopsy¸¦ °í·ÁÇØ¾ß ÇÑ´Ù.
4) Diagnostic approach & staging
¨ç occupational & environmental Hx°¡ Áß¿ä
¨è aspiration & infection : DDx¿¡ Áß¿ä
¨é CXR or HRCT, PFT, ABGA¸¦ ÃøÁ¤ÇÑ´Ù.
¨ê Áø´ÜÀü¿¡ Á¶Á÷ÇÐÀû ºÐ¼®ÀÌ ÇÊ¿äÇÏ´Ù.
FOB : first invasive procedure
infection, malignancy, ´Ù¸¥ ƯÁ¤ÁúȯÀ» ¹èÁ¦Çϴµ¥ Áß¿ä
¨ë BAL : alveolar inflammation & immunologic activity¸¦ ÆÇ´ÜÇϴµ¥ À¯¿ëÇÏ´Ù.
¨ì 67 Ga lung scan : Áø´ÜÀû Á¤È®¼º ȤÀº activity¸¦ monitoringÇϴµ¥ ´õ ÀÌ»ó µµ¿òÀÌ
¾ÈµÈ´Ù.
¨í bronchoscopy·Î Áø´ÜÀÌ Àǽɽº·¯¿ì¸é thoracoscopic or open lung bipsy¸¦ °í·ÁÇÑ´Ù.
3. Ä¡·á
1) oral Pd : 1mg/kg/d X 8ÁÖ
response(+) -> 0.25 mg/kg/d X 6°³¿ù
response(-) -> immunosuppression(cyclophosphamide)
1.0mg/kg/d(total 50-75mg) + oral Pd(0.25mg/kg/d)
7-10ÀÏ °£°ÝÀ¸·Î 50mg¾¿ Áõ·®
WBC´Â ´ë·« 1/2·Î °¨¼ÒµÇ´Â °ÍÀ» ¸ñÇ¥·Î, 1000°³ ¹Ì¸¸À϶§´Â stop.
2) high-dose, pulsed glucocorticoid : special advantage(-)
3) Azathioprine : cyclophosphamide ´ë½Å »ç¿ë
4) colchine : macrophage-produced fibroblast growth factor¾ïÁ¦. 0.6mg/d
5) stop cigarette smoking
6) diuretics : Rt-sided CHF¶§
7) prophylaxis of pneumococcal & influenza vaccine
8) lung transplantation°í·Á
Individual forms of ILD
1. Collagen vascular disorders
1) SLE : 1/2¿¡¼ °á±¹ over lung disese·Î ÁøÇàÇÑ´Ù.
mc : pleuritis, pleural effusion, acute pneumonitis
chronic, progressive ILD : uncommon
2) RA : pleural disease(pleural effusion & subpleural nodules)
parenchymal nodular infiltrates
diffuse interstitial fibrosis
BOOPÀÌ º¸°íµÊ
MTX, goldÄ¡·á½Ã ILD·Î ¹ßÀüÇÒ °¡´É¼º(drug hypersensitivity)ÀÌ Àִµ¥
À̶§ preexisting or developing ILD)¿Í °¨º°ÇØ¾ß ÇÑ´Ù.
Penicillamine Tx : bronchiolitis obliteransÀÇ ¿øÀÎÀÌ µÊ.
3) Ankylosing spondylititis : bilateral upper lobe fibrosis, fibrocavitary dsÇÕº´°¡´É
4) systemic sclerosis
5) sjogren's syndrome
6) polymyositis & dermatomyositis(5-10%): anti-Jo-1 Ab°¡ Àִ ȯÀÚ¿¡¼ ´õ ÈçÈ÷ ¹ß»ý
2. Pul. hemorrhage
1) Goodpasture's syndrome
2) Idiopathic pulmonary hemosiderosis
3. Pulmonary alveolar proteinosis
diffuse alveolar consolidation and/or nodular shadows
typically radiating from the hilar regions
alveoli - PAS(+) granular materials·Î Â÷ÀÖ´Ù.
no inflammation & normal septal structure
intraalveolar material : surfactant phospholipid, LDH, other protein, IgÀÇ º¹ÇÕü
4. Lymphocytic infiltrative disorders
5. Eosinophilic pneumonia
¸¹Àº ¾àµéÀÌ eosinophilc pneumonia¸¦ À¯¹ßÇÑ´Ù. ÁÖ·Î older female
lung Bx·Î Áø´Ü => eosinophilic inflammatory process
6. Lymphangioleiomyomatosis
7. Amyloidosis
8. Inherited disorders
9. GI & liver disease
10. GVHD
11. Granulomatous response
1) Langerhans cell granulomatosis(Eosinophilic granuloma or Histiocytosis X)
unknown etiologyÀÇ ILDÀÇ 1-5%
* dendritic cell : potent stimulatory & accessory cell immune function
interstitium, alveolar septal area & BAL fluid¿¡¼ ¹ß°ß
tissue macrophage¿Í´Â È®¿¬È÷ ´Ù¸§.
BAL cell¿¡¼ 3%ÀÌ»ó ÀÎÁö(other diseaseº¸´Ù ¸¹Àº ¼öÄ¡ÀÓ)
±×·¯³ª Langerhans cellÀº not pathognomonic
CXR : diffuse micronodular shadows & cystic spaces, CPA sparing
PFT : obstructive & restrictive defectÀÌ °øÁ¸
º´ÀÌ ÁøÇàµÉ¼ö·Ï advanced COPD¿Í ºñ½Á
* Ä¡·á
¨ç stop smoking
¨è glucocorticoid : not helpful
¨é penicillamine : prevent fibrosis
¨ê local bone lesion : irradiation
¨ë supportive Tx & bronchodilator : È¿°ú´Â Áߵ
¨ì lung transplantation : ÀϺÎȯÀÚ¿¡¼ °í·Á
2) Granulomatous vasculitis
Wegener's granulomatosis
Churg & Strauss granulomatosis: asthma, eosinophilia°¡ Á¸Àç
3) Lymphomatoid granulomatosis
lung¿¡ ÀÏÂ÷ÀûÀ¸·Î ħ¹üÇϰí, skin, CNS, PNS, kidney¿¡´Â ´ú ÈçÇÏ´Ù.
50%¿¡¼ malignant lymphoma·Î ÁøÇàÇÑ´Ù.
Ä¡·á : glucocorticoid, cyclophosphamide
12. Bronchocentric granulomatosis