(Interstitial Lung Diseases ILDs)

(Diffuse Parenchymal Lung Diseases DPLDs)

() , . .

. , , ,

,

LAM, HX

/ IPF DIP .- RB-ILD LIP

NSIP COP AIP

IPF : Idiopathic Pulmonary Fibrosis BOOP/COP : Bronchiolitis Obliterans Organizing Pneumonia DIP : Desquamative Interstitial Pneumonia LIP : Lymphocytic IP NSIP : Non Specific Interstitial Pneumonia AIP : Acute IP RB-ILD : Respiratory Bronchiolitis- Interstitial Lung Disease

, , ...() / HRCT ( VC,TLC), (DLCO) (PA-aO2)

-

(, , ) / , (, ) - , ,

-

(LAM) (Wegener, Churg Strauss)

81 /100000 67 /100000 : 32/100000 26/100000 - : , , , , Bernalillo County, New Mexico 1988-1990

- (/, HRCT) - - - (, )

, ,

, , , ,

, , 2,

///

?

. , . , .Goodpasture, . , ARDS . , , ,

-

(, .Churg Stauss, ., )

- , , , ,

, , , ,

( , , , caf au lait spots, , )

(, .Sjogren, . ) (, , )

... velcro , 2 , , ,

/ + High Resolution CT. /. / 80% HRCT 94%.

. (ground glass). ) (nodular pattern). ) (linear pattern). ) (reticular p.). ) (honeycomb) ) (reticulonodular p.). . (mosaic pattern). (crazy paving).

K () (.) (, , ) ( ) ( DIP, BAC, )

:

- BAC

BOOP/COP

( )

(Ground Glass)

, ,

(nodular pattern) , 15mm

tree-in-bud ( )

Tree-in-bud

ree-in-bud

(linear pattern)

(, ) . , . .

(reticular pattern)

A .

, .

(honeycomb pattern)

5-10mm .

UIP

Honeycomb

Honeycomb

LAM LCH

UIP

EMPHYSEMA

LAM

(mosaic pattern) . -. - ( HRCT). .

(crazy paving) . 0,5-1 . (DAD-Diffuse Alveolar Damage), , -LIP.

DLCO P(A-a)O2

, LAM . , , DLCO

E

BAL VATS ( Video Assisted Thoracoscopic Surgery)

ILD ( ), , , ; ILD 200 . . . , .

20 40

- (> 90%) ( 5-30%) (~5%. ) (~25%) (15-54%) (10%) (< 5%) / (1,25 Vit D ) /. , SIADH ( )-, , CT) ( ) (25-39%) (20%), -,

, DLCO / ( )

I.

/ . / / / ( )

II.

I.

II.

(~ 50%) (~ 90%) ( ) Kveim

(~2/3 ), 10-20% . 85% 2 Lupus pernio, , , , , . . 1-5% ( , , )

( ) (, , , )

(30-60 mgr / 24) 4-12 . , . 12-18 , () , ,

- IPF , Honeycombing /

- IPF

NSIP Ground Glass

COP Ground Glass

Ground Glass

LGround Glass

- RB-ILD Ground Glass

DIP Ground Glass

-LIP, -LAM, , .

BAL 100-150ml.

. . , . .

BAL

BAL

( ), .

. . , PCP, CMV . -.

30% , , . . . . . . (>94%), 42% .

: 65, , .

, VATS (Video Assisted Thoracoscopic Surgery). .

: , , ( ) . , , , , . : . : . : +

: , . , , , . : 3-5 . 1 2,5 cm . : , . , , .

-

, , , . . .

. . . . .

. / .

, , . :

/. //. / . .

- HRCT (pattern), , , . HRCT honeycombing , . , , ground-glass .