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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