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Pulmonary alveolar proteinosis Murray p1789 - 1801

1. °³¿ä

alveoli & bronchiole¿¡ amorphous, insoluble, proteinaceous materialÀÌ ÃàÀûµÇ´Â º´ÀÌ´Ù.

proteinaceous material¿¡´Â phospholipid & surfactant proteinÀÌ Ç³ºÎÇÏ´Ù.

ÀÌ ¹°ÁúÀÌ ¿°Áõ¹ÝÀÀÀ» ÀÚ±ØÇÏÁö´Â ¾ÊÀ¸¹Ç·Î lung fibrosis´Â °ÅÀÇ ¾ø´Ù.

1Â÷Àû Àå¾Ö´Â »ê¼Ò¿î¹Ý´ÉÀÇ Àå¾ÖÀ̸ç ȯÀÚ´Â È޽ĻóÅ¿¡¼­ Àú»ê¼ÒÁõÀ» º¸ÀδÙ.

±âȸ°¨¿°¿¡ ÀÇÇØ »ç¸ÁÇϱ⵵ Çϳª °á±¹Àº hypoxemia·Î »ç¸ÁÇÑ´Ù.

2. Á¶Á÷º´¸®: PAS(+) cytoplasm

alveolar septa´Â no significant fibrosis

ÀϺο¡¼­ recurrent pneumonia history°¡ ÀÖ´Ù.

Norcardia asteroides¿¡ ´ëÇÑ ¹Ýº¹Àû Æó°¨¿°ÀÌ ÀϾ ¼ö ÀÖ´Ù.

ÀϺο¡¼­ repeat infectionÀÌ fibrosis & inflammatory responseÀÇ ¿øÀÎÀÌ µÈ´Ù.

3. ÀÚ¿¬°æ°ú

1965³â whole-lung lavage°¡ ¼Ò°³µÈÈÄ ¿¹Èİ¡ Çâ»óµÇ¾ú´Ù. ´Ù¸¥ º´°ú´Â º¸Åë °ü°è°¡ ¾øÀ¸³ª

hematologic malignancy, inorganic dust exposure, infection in immunocompromised

person¿¡¼­ º¸°íµÇ¾ú´Ù.

young or middle aged adult¿¡ ÁÖ·Î »ý±â°í ³²³àºñ´Â 2:1ÀÌ´Ù.

Nocardia pneumonia ºóµµ°¡ ³ôÀ¸¸ç, ƯÈ÷ ¸é¿ª¾ïÁ¦È¯ÀÚ¿¡¼­ ºóµµ°¡ ³ô´Ù.

* secondary alveolar proteinosis

HIV(+), malignancy(+), silicoproteinosis

4. ¿øÀÎ ¹× º´ÀÎ

¸¹Àº °¡¼³ÀÌ ÀÖ´Ù.

genetic defect, defective alveolar macrophage, surfactant overproduction or decreased

clearance, immunodeficiency

i) primary

ii) secondary: coexisting abnormality°¡ µ¿¹ÝµÉ ¶§

infection, hematologic malignancy, silicosis

* silicoproteinosis : airspace¿¡ »ó´ç·®ÀÇ silica or silicates°¡ ¹ß°ßµÉ ¶§ silicoproteinosis

¶ó´Â ¿ë¾î¸¦ ¾´´Ù.

5. ÀÓ»ó¹ßÇö

ÁÖÁõ»óÀº ¿îµ¿½Ã È£Èí°ï¶õÀÌ´Ù.

±× ¿Ü, wt loss, malaise, fatigue

fever°¡ ÀÖ´Ù¸é, pulmonary alveolar proteinosis°¡ ¾Æ´Ï°Å³ª superinfectionµÈ °æ¿ìÀÌ´Ù.

6. ¹æ»ç¼±ÇÐÀû ¼Ò°ß

CXR : uniformly infiltration

perihilar¿¡ ´õ ½ÉÇÏ¿© pulmonary edema¶§ º¼ ¼ö ÀÖ´Â "bat wing" appearance¼Ò°ßÀ»

º¸ÀδÙ.

interstitial marking¡è, acinar filling¡è

CT : dense consolidation or ground glass appearance

thickened lobular septa => diffuse "interstitial" pattern

7. Lab: LDH¡è, SGOT: normal

pulmonary surfactant protein A¡è

8. PFT: DLco¡é

9. Áø´Ü: Áõ»ó + CXR => ¸¹Àº ÆóÁúȯ¿¡¼­ ÈçÈ÷ º¼¼ö ÀÖ´Â ¼Ò°ßÀÌ´Ù.

BAL or TBLB(open lung biopsy´Â ÇÇÇϵµ·Ï ÇÑ´Ù)

BALÀÇ LM¼Ò°ß: PAS(+) deep pink

macrophage¸¦ ÈçÈ÷ º¼¼ö ÀÖ°í ´Ù¸¥ cellÀº ¾ø´Ù.

Áø´ÜÀº lung biopsy or BAL fluid·Î Çϵµ·Ï ÇÑ´Ù.

10. Ä¡·á: whole-lung lavage

º¸Åë 6-12°³¿ù °£°ÝÀ¸·Î ½ÃÇàÇÑ´Ù.

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