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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¸¦ ¾ÇÈ­½Ã۰í 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