Primary lung disease of ILD (Cecel p414-415)
1. IPF
- 40-60¼¼ ÁÖ·Î »ý±â¸ç geographic, gender, racial or seasonal predilectionÀº ¾ø´Ù.
- ÁÖÁõ»ó : insidious onset of breathlessness with exercise, dry, nonproductive cough
ÀϺÎȯÀÚ¿¡¼ fever, fatigue, wt loss, myalgia, arthralgia°¡ ÀÖÀ»¼ö ÀÖ´Ù.
- ÁøÂû¼Ò°ß : late inspiratory fine rale at the lung base(Velcro rale)
late stage¶§ Rt-sided heave(P2¡è, S3 gallop)
- CXR : reticular or reticulonodular infiltrate(lower lung zones)
multiple cystic or honey combed area(0.5-1cm diameter) => poor Px
- HRCT : Ãʱâ patchy air-space opacification or GGO - peripheral(subpleural) & basilar
regions
- ÀÓ»óƯ¡ : honeycomb cyst rupture·Î spontaneous pneumothorax°¡ »ý±æ¼ö ÀÖ´Ù.
hypoxemia : V/Q mismatch
- Ä¡·á : ¨ç corticosteroid : Ä¡·á¹ÝÀÀÀº ´ÜÁö 10-20%¿¡ ºÒ°úÇÏ´Ù.
¨è lung transplantation
medical tx¿¡ ¹ÝÀÀÇÏÁö ¾Ê°Å³ª end-stage diseaseȯÀÚ¿¡¼ °í·Á
- Ä¡·á ¹ÝÀÀ ¹× ¿¹ÈÄ
extensive GGO with minimal honeycombing(HRCT»ó) = steroid¿¡ ´ëÇÑ ¹ÝÀÀÀÌ ÁÁ´Ù.
¹Ý´ë·Î honeycomb cyst´Â minimal or no response
ÀÌó·³ lung biopsy¸¦ Çϸé Ä¡·á¹ÝÀÀÀ» ¿¹ÃøÇÒ¼ö ÀÖ´Ù.
Æò±Õ »ýÁ¸·üÀº 5-7³âÀ̸ç spontaneous regressionÀº °ÅÀÇ ¾ø°í ´ëºÎºÐ Ä¡·á°¡ ÇÊ¿äÇÏ´Ù.
±×·¯³ª Ä¡·á¸¦ ÇÏ´õ¶óµµ Ä¡·á¹ÝÀÀÀº 10-20%¿¡ ºÒ°úÇÏ´Ù.
2. Sarcoidosis - sarcoidosisÆí ÂüÁ¶
3. BOOP
- Áõ»ó : °¡Àå ÈçÇÑ Áõ»óÀº flu-like illness with nonproductive cough
fever, malaise, wt loss, fatigue°¡ dyspnea½ÃÀÛÀü 2-4°³¿ù Áö¼ÓÇÑ´Ù.
º¸Åë Ç×»ýÁ¦ Ä¡·á¿¡ ½ÇÆÐÇÑ´Ù.
raleÀº ÈçÇϳª wheezingÀº µå¹°´Ù.
- Æó±â´É : DLCO¡é, ÁÖ·Î restrictive pattern, °ú°Å Èí¿¬ÀÚ¿¡°Ô¼ obstructive patternÀ»
º¸Àϼö ÀÖ´Ù(20%).
- CXR : bilateral diffuse alveolar opacity with normal lung volume, peripheral & migratory
reticulonodular, honeycombingÀº µå¹°´Ù.
- Áø´Ü : thoracoscopic or open lung Bx
( transbronchial Bx´Â ¼±ÅÃµÈ °æ¿ì¿¡¸¸ Á¦ÇÑÀûÀ¸·Î ½ÃÇà)
honeycombing or diffuse alveolar wall fibrosis´Â BOOPÀÇ Æ¯Â¡Àû ¼Ò°ßÀÌ ¾Æ´Ï´Ù.
- Ä¡·á : corticosteroid tx - 2/3¿¡¼ ȸº¹µÈ´Ù.
¼öÀÏ-¼öÁÖ³» ºü¸¥ ȸº¹À» º¸ÀÌÁö¸¸ steroid taperingÁß¿¡ Àç¹ßÇÒ¼ö ÀÖÀ¸¸ç
À̶§ ÀçÄ¡·á´Â ¼º°øÀûÀÌ´Ù.
cyclophosphamide : progressive dsȯÀÚ Ä¡·á¿¡ »ç¿ë
4. Lymphocytic interstitial pneumonia
lymphocytic infiltration of lung(primary lymphoma, lymphomatoid granulomatosis)¿Í
±¸º°ÇØ¾ß ÇÑ´Ù. ¶§·Î´Â Ư¹ß¼ºÀ̱⵵ ÇÏÁö¸¸ ÈçÈ÷ hypogammaglobulinemic or
hypergammaglobulinemic state, AIDS, systemic rheumatologic disorder, BMT¿Í µ¿¹Ý
µÈ´Ù. Àú°¨¸¶±Û·ÎºÒ¸°Ç÷Áõ ¹× AIDS¿¡¼ infectious CxÀÌ ÀϾÙ.
°á±¹ end-stage lung ds¿Í lymphoma·Î ÁøÇàÇÏÁö¸¸ corticosteroid¿¡ ´ëÇÑ ¹ÝÀÀÀº 50%
ÀÌ´Ù.
5. Histiocytosis X
- ÀÓ»óƯ¡
20-40¼¼
cigarette smoking Hx : 90%
nonproductive cough & exertional dyspnea
pleuritic chest pain & acute dyspnea°¡ ¹ß»ýÇÏ¿´À»¶§´Â pneumothorax¸¦ »ý°¢ÇÏ¿©¾ß
ÇÑ´Ù(25%)
cystic bone lesion(skull, rib, pelvis) -10%
DI (10%) - poor Px
- HRCT : multiple thin-walled cyst(dilated small bronchi & bronchioles, destroyed
parenchyma) nodules(peribronchiolar granuloma) - upper& midlung zone
- PFT : mixed obstructive & restrictive pattern, DLCO¡é
- °æ°ú : ´Ù¾çÇÏ´Ù.(spntaneous remission, stabilization, progression)
- Ä¡·á : corticosteroid°¡ ÀϺÎȯÀÚ¿¡¼ È¿°úÀûÀÌ´Ù.
6. Lymphangioleiomyomatosis
- °³¿ä ¹× ÀÓ»óƯ¡
°¡Àӱ⠿©¼º¿¡¼¸¸ ¹ß»ýÇÏ´Â µå¹® ÁúȯÀ¸·Î lymphatic, peribronchial, perivascular,
interstitial tissue¿¡ smooth muscleÀÌ Áõ½ÄÇÏ´Â °ÍÀÌ Æ¯Â¡ÀÌ´Ù.
´ëºÎºÐ ¹Ì¼¼ÇÑ ¿°ÁõÀÌ Á¸ÀçÇÏÁö¸¸ alveolar wallÀÌ °á±¹Àº ÆÄ±«µÈ´Ù.
dyspnea, chylous pleural effusion(¡ñpleural lymphatic obstruction), recurrent
pneumothorax(¡ñemphysematous cyst rupture)°¡ »ý±ä´Ù.
- º´ÀÎ : hormoneÀÌ °ü¿©ÇÏ´Â °ÍÀ¸·Î »ý°¢µÇ´Âµ¥ ÁÖ·Î Æó°æÀü ¿©¼º¿¡¼ ¹ß»ýÇϸç ÀÓ½Å,
ºÐ¸¸ÈÄ, exogenous estrogen tx¶§ ¾ÇȵDZ⠶§¹®ÀÌ´Ù.
- CXR : coarse reticular infiltrates with cystic dilatation
HRCT : diffuse thin-walled cyst
* ´Ù¸¥ chronic ILD¿¡¼ º¸ÀÌ´Â nodular, interstitial fibrosis, irregular lung pleural interfaces
¿Í °°Àº Ư¡Àº ¾ø´Ù.
pleural effusion & recurrent pneumothorax°¡ À¯ÀÏÇÑ ¹æ»ç¼± ¼Ò°ßÀϼöµµ ÀÖ´Ù.
- Áø´Ü : thoracoscopic or open lung Bx°¡ ÇÊ¿äÇÏ´Ù.
- Ä¡·á : progesterone(10mg/d) or tamoxifen(20mg/d) - DOC
lung transplantation
- ¿¹ÈÄ : Ä¡·á¸¦ ÇÏ´õ¶óµµ ´ëºÎºÐ Áõ»ó¹ßÇö 10³â³» È£ÈíºÎÀüÀ¸·Î »ç¸ÁÇÑ´Ù.