diaxytes_diameses_pneumonopathies
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(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 .