Interstitial lung disease Harrison 15ÆÇ
1. °³¿ä
unknown cause ILDÁß mc : sarcoidosis, IPF, pulmonary fibrosis associated with CTD
known cause ILDÁß mc : occupational & environmental exposure
ƯÈ÷ inorganic dusts, organic dusts, various fumes or gases
2. º´ÀÎ
* 2 major histopathological patterns
i) granulomatous patterns
2 major DDx: sarcoidosis & hypersensitivity pneumonitis
ii) inflammation & fibrosis predominate
Tab 259-1 14ÆÇ°ú ¾à°£ ´Þ¶óÁø ³»¿ë
<Alveolitis, interstitial inflammation, and fibrosis with unknown cause>
Idiopathic interstitial pneumonia : IPF(UIP), DIP, AIP(diffuse alveolar damage), COP, NSIP
°¡ »õ·Î Ãß°¡µÊ.
3. Initial evaluation
1) history
¨ç duration of illness
i) acute presentation(¼öÀÏ-¼öÁÖ) unusual
allergy(drug, fungi, helminth), acute idiopathic interstitial pneumonia,
eosinophilic pneumonia, hypersensitivity pneumonitis
CXR»ó diffuse alveolar opacity·Î ³ªÅ¸³ª¹Ç·Î atypical pneumonia·Î È¥µ¿Çϱ⠽±´Ù.
ii) subacute presentation(¼öÁÖ-¼ö°³¿ù) ¸ðµç ILD¿¡¼ ³ªÅ¸³¯ ¼ö ÀÖ´Ù.
ƯÈ÷, sarcoidosis, drug-induced ILD, alveolar hemorrhage syndromes,
cryptogenic organizing pneumonia(COP), acute immunologic pneumonia
(SLE or PM)
iii) chronic presentation(¼ö°³¿ù-¼ö³â) ´ëºÎºÐÀÇ ILD´Â ¸¸¼ºÀûÀÌ´Ù.
¿¹, IPF, sarcoidosis, pulmonary langerhans cell histiocytosis(PLCH, eosinophilic
granuloma), pneumoconiosis, CTD
¨è age: IPF´Â 50¼¼¡è, ±× ¿Ü ±âŸ 20-40¼¼
¨é gender: LAM, tuberous sclerosis - premenopausal women
¨ê family history
AD: tuberous sclerosis, neurofibromatosis
AR: Niemann-Pick disease, Gaucher's disease, Hermansky-Pudlak syndrome
±âŸ: sarcoidosis, familial pulmonary fibrosis
¨ë smoking history
PLCH, DIP, Goodpasture's syndrome, respiratory bronchiolitis
: Ç×»ó current or former smoker
IPFÀÇ 75% smoking history(+)
¨ì occupation & environmental history : hypesensitivity pneumonitis, ±× ¿Ü ƯÁ¤Á÷¾÷
2) sx & sign 3) P/E »ý·«
4) Lab
ANA, RF, immune complex
LDH¡è: nonspecific
sarcoidosis: angiotensin converting enzyme¡è
hypersensitivity pneumonitis: serum precipitin
ANCA or anti-BM Ab: vasculitis°¡ ÀÇ½ÉµÉ ¶§ À¯¿ë
5) Chest imaging studies
¨ç CXR : nodular or mixed pattern of alveolar filling & reticular marking¡è
* upper lung zone predilection
: sarcoidosis, PLCH, chronic hypersensitivity pneumonitis, silicosis,
berylliosis, RA(necrobiotic nodular form), ankylosing spondylitis
CXR¾Æ ÀÓ»ó, Á¶Á÷ÇÐÀû stage¿Í´Â poor correlation
honeycombingÀº pathologic finding & progressive fibrosis¿Í correlation
(=poor prognosisÀǹÌ)
´ëºÎºÐ CXR´Â ºñƯÀÌÀûÀ¸·Î ƯÁ¤Áø´Ü¿¡ µµ¿òÀÌ µÇÁö´Â ¾Ê´Â´Ù.
¨è HRCTÀÇ À¯¿ë¼º
i) ILD°¡ ÀÇ½ÉµÉ ¶§ Á¶±âÈ®ÀÎ ¹× È®ÁøÀ» À§ÇØ ÇÊ¿äÇÏ´Ù.
ii) disease extent & distributionÀ» Æò°¡Çϴµ¥ À¯¿ëÇÏ´Ù.
iii) coexisting disease¸¦ È®ÀÎÇÒ¼ö ÀÖ´Ù.
¿¹> mediastinal adenopathy, carcinoma or emphysema
iv) ƯÁ¤ ÀÓ»ó»óȲ¿¡¼´Â lung biopsy¸¦ ÁÙÀϼö ÀÖ´Ù.
¿¹> IPF, sarcoidosis, hypersensitivity pneumonitis, asbestosis, lymphangitic carcinoma,
PLCH
v) Bx°¡ ÇÊ¿äÇÏ´Ù¸é bxÀ§Ä¡¸¦ Á¤Çϴµ¥µµ µµ¿òÀÌ µÈ´Ù.
¨é radionuclide scanning
Ga-67 scan: limited value
99mTc-DTPA scan: pulmonary epithelial permeability index
IPF¿¡¼ normal 99mTc-DTPA clearance =stable diseaseÀǹÌ
rapid clearance = deterioation risk¡è
6) PFT
¨ç spirometry & lung volume
´ëºÎºÐÀÇ ILD´Â restrictive defect, RLC, FRC, RV¡é
FEV1¡é, FVC¡é, FEV1/FVC : normal or ¡è
lung volume reductionÀº lung stiffness¸¦ ¾ÇȽÃ۰í disease progressionÀ» Áõ°¡½ÃŲ´Ù.
*¸î¸î ÁúȯµéÀº CXR»ó interstitial opacity¸¦ ³ªÅ¸³»°í PFT»ó obstructive airflow limitationÀ»
º¸ÀδÙ.
common: tuberous sclerosis, LAM
uncommon: sarcoidosis, hypersensitivity pneumonitis
7) FBO & BAL
8) tissue & cellular exam
lung biopsy°¡ È®Áø°ú disease activity Æò°¡¿¡ ÀÖ¾î °¡Àå È¿°úÀûÀÎ ¹æ¹ýÀÌ´Ù.
Ä¡·á °¡´ÉÇÑ °úÁ¤À» ÀÎÁöÇÒ¼ö ÀÖ´Ù. ƯÈ÷ chronic HP, COP, respiratory-associated ILD,
or sarcoidosis¿¡¼.
Ä¡·áÀü¿¡ biopsy¸¦ ½ÃÇàÇØ¾ß ÇÑ´Ù.
È®ÁøÀ» Çϸé Ä¡·áÁß ¹ÝÀÀÇÏÁö ¾Ê°Å³ª serious side effect°¡ ¹ß»ýÇßÀ» ¶§ ¿À´Â ºÒ¾È°ú
È¥¶õÀ» ¾ø¾Ù¼ö ÀÖ´Ù.
* biopsy´Â FOB with multiple TBLB(4-8 specimens)°¡ initial choiceÀÌ´Ù.
: sarcoidosis, lymphangitic carcinomatosis, eosinophilic pneumonia, Goodpasture's
syndrome, or infection
TBLB·Î Áø´ÜÀÌ ¾ÈµÇ¸é surgical lung biopsy°¡ ÇÊ¿äÇÏ´Ù.
* relative contraindications
serious cardiopulmonary disease, "honeycombing" diffuse end-stage diseaseÀÇ X-ray
finding, severe pulmonary dysfunction, major op risks(³ëÀÎ µî)
9) Ä¡·á
hypoxemia(PaO2 < 55 mmHg) : supplemental oxygen
cor pulmonale : diuretic tx & phlebotomy
* drug tx
i) glucocorticoid - mainstay of treatment. ±×·¯³ª ¼º°ø·üÀÌ ³·´Ù.
symptomatic ILD with idiopathic interstitial pneumonia
eosinophilic pneumonia, COP, CTD, sarcoidosis, acute inorganic dust exposure,
acute radiation pneumonitis, DAH, drug-induced ILD
ii) cyclophosphamide & azathioprine c/s glucocorticoid
Idiopathic interstitial pneumonia
1. Idiopathic pulmonary fibrosis(IPF)
Á¶Á÷ÇÐÀûÀ¸·Î UIP(usual interstitial pneumonia)¸¦ È®ÀÎÇÔÀ¸·Î½á È®ÁøÇÒ¼ö ÀÖ´Ù.
TBLB´Â UIPÁø´Ü¿¡ µµ¿òÀÌ ¾ÈµÇ°í surgical biopsy°¡ ÇÊ¿äÇÏ´Ù.
5YSR : 30-50%
<Ä¡·á> glucocorticoid, cytotoxic agent(azathioprine, cyclophosphamide)
antifibrotic agent(colchicine, perfenidone, or IFN-¥ã 1b)
2. Desquamative interstitial pneumonia(DIP) rare
cigarette smoker¿¡¼¸¸ ¹ß°ß. 30-40´ë
* histologic hallmark: extensive accumulation of macrophages in intraalveolar spaces with
minimal interstitial fibrosis
CXR : diffuse hazy opacities
DLCO¡é, restrictive pattern
¿¹ÈÄ´Â ÁÁ´Ù(10 YSR ¡70%)
Èí¿¬Áß´Ü ¹× systemic glucocorticoid Ä¡·á¿¡ ¹ÝÀÀÀÌ ÁÁ´Ù.
respiratory bronchiolitis-associated ILD´Â DIPÀÇ ÇÑ Á¾·ù·Î »ý°¢µÈ´Ù.
3. Acute interstitial pneumonia(AIP)(Hamman-rich syndrome )
rare, fulminant form of lung injury characterized by diffuse alveolar damage on lung
biopsy
40¼¼ ÀÌÈÄ
ARDS presentation°ú À¯»çÇÏ´Ù.
¹ßÇöµÇ±â Àü(7-14ÀÏ)¿¡ prodromal illness°¡ ÈçÇÏ´Ù: fever, cough, dyspnea
CXR: diffuse, bilateral, air-space opacification
HRCT: bilateral patchy, symmetric GGO
* Áø´Ü: idiopathic ARDS & pathologic confirmation of organizing diffuse alveolar damage
¡Å lung biopsy°¡ ÇÊ¿ä
´ëºÎºÐ moderate to severe hypoxemia & respiratory failure·Î ¹ßÀü
& mechanical ventilationÇÊ¿ä
»ç¸Á·ü > 60%
´ëºÎºÐ ¹ßÇöÈÄ 6°³¿ù³» »ç¸Á. Àç¹ßÀÌ º¸°íµÇ¾ú´Ù.
ÁÖÄ¡·á´Â supportive
glucocorticoidÈ¿°ú´Â È®½ÇÄ¡ ¾Ê´Ù.
4. Nonspecific interstitial pneumonia(NSIP) : idiopathic interstitial pneumoniaÀÇ subgroup
ÀÓ»óÀû ¹× º´¸®ÀûÀ¸·Î UIP, DIP, AIP & idiopathic BOOP°ú ±¸º°ÇÒ¼ö ÀÖ´Ù.
lung biopsy: ´Ù¾çÇÑ Á¤µµÀÇ chronic interstitial inflammation & fibrosis
subacute restrictive process
UIPº¸´Ù younger age
clubbing(-)
HRCT : GGO & consolidation
IPF¿Í ´Þ¸® ´ëºÎºÐ good prognosis
glucocorticoid Ä¡·áÈÄ ´ëºÎºÐ È£Àü
5. Cryptogenic organizing pneumonia(COP) = idiopathic BOOP
40-50´ë
flu-like illness with cough, fever, malaise, fatigue, weight loss
CXR: bilateral, patchy, or diffuse alveolar opacities
recurrent & migratory pulmonary opacities
HRCT: air-space consolidation
GGO, small nodular opacities & bronchial wall thickening & dilatation
lung biopsy: granulation tissue within small airways, alveolar ducts, and airspaces
Ä¡·á: glucocorticoid: 2/3¿¡¼ ÀÓ»óÀûÀΠȣÀüÀ» º¸ÀδÙ.
ÀϺΠȯÀÚ´Â glucocorticoid tx¿¡µµ ºÒ±¸Çϰí ÁøÇàÇÏ¿© fatal outcome.
<Á¤¸®> idiopathic interstitial pneumoniaÁß good px? DIP, NSIP, COP