????? ???? ???????????????-?????????????? Diffuse Interstitial-Infiltrative Lung Disease (ILD)

1
????? ???? ???????????????-??????????????Diffuse
Interstitial-Infiltrative Lung Disease (ILD)

• ?"? ????? ?????
• ??? ???? ??' ?????
• ???? ??' ?????? ?????? ??????
• "???? ????? ????"
• ??"? ????, ???-???

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?????

• ????? ????? ???????? ??? ??? 200 ????? ????? !!!
• ??????
• ????? ??????? ????? (??-????? !)
• ?????????????? ????
• ????? ?????? ????
• ????? ??????? ????
• Interstitial misnomer!
• Alveolar wall/space, vessels/bronchioles too !!!

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????? ??????

• 1. ???? ????
• 2. ????? ???-?????????
• 3. ????? ?????
• ????? ???????? ????? ?? ILD
• (??????, ????? ???' ?????? ??? ????????)
• 5. ????? ?????? ???????????????? ???????????
• 6. ???????????

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???? ????

• ?.?.?. ?? 85, ????? ?????, ????? 2, ???? ???
  ?? ????,
• ??????? ???? ????.
• ?.?. ????? ???, ?????, ??? ???.
• ?.?. ????? ??? ???, ?? ?????? ?????? ?????.
  ????? ???? ????? ?? ???? ????? ?????.
• ????? ??????? ?? ???? ?????, ???? ???? ?????,
  ???? ??????? ???????? ???? ?????? ?????? 2 ?????
  ??? ???? ?????.

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???? ???? (????...)

• ?????
• 1. ??? ???-?? ????? ?????? ????/?????-???
  (????? ????? ?????????)
• 2. ??? ?????? ????? ?????, ?? ?????? ?????
  ????.
• ?????? ?????, ??"? 60 ????-?????.
• ????? ??? ???? 2 ??????, ?????? "????
  ?????/??? ? ?????? ???? ???? "???? ?????",
  ?????? ?? ??????????? ? ??? ????? ?????/?????? ?
  CT ??? ? ???? ?????.

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???? ???? (????...)

• ?????? ??? ???? ???, ??? ??"?, ??? ???? ?????
  ??????, ?????? ????? ???. ??? ?????? ?? ??????
  ??????, ??? ???? ?????? ?? ?????. ????? ?????
  ????? ?????????? ???????? ??????? ???? Velcro.
  ??? ??? ?.?.?.
• ?' ??? CT ???

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???? ???? (????...)

• ?????? ???? (PFT)
• TLC 70 VC 55 TGV 89
• FEV1 53 (0.86L) FEV1/FVC 97
• DLco 10.7 ml/mmHg 62
• PO2 (rest) 75 mmHg PO2 (exercise) 49 mmHg

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???? ???? (????...)

• ?????
• CBC normal ESR 52 mm/ 1 h Glob 4 gr
• LDH 628 (618) ANA normal
• Anti-DNA 33 (26) C3,4 N RF N
• ??????? ????????????
• UIP Usual type Interstitial Pneumonitis
• (clinical name IPF Interst. Pulm. Fibrosis)

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????? ????????????

• ?????? ????????????? ???? ???? ?????? ??????? ??
  ??????, ???????, ?????? ????????
• ????? ???? ?????? DLco? (????????? ????? ??
  ??????)
• ???????? ???? ?????? Compliance ?, ???? ????
  ?????? (????? ????? ??????)
• ?????????? ?????? ??????, ????? ????? (R.V?),
  V/Q????, ?????
• ?? ??? ?? ??? ?"? ????? DLco? ?? ?????
  ??????!
• ???????? ?????? ????? ????????? ????? !

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???????? ?????? ?????

1. ???? ????? ?????? ???? ??? ?? ??? ??????/????, ??
   ???? ?? ??? ????/??????.
2. ?/?? ????? ??? ?????
3. ?/?? ????? ??? ?????
4. ?/?? ?????? ???? ??????????

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????? ?? ???? ????? ??????????????

• ????? ????? ??? 200 ????? ????? - ????? ????
  ????? ???????.
• ??? ????? ?????? ???????, ????????
• ?????? ??? ???? ?????? ?????? !!!

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????? ????? ?? ILD(?? ?? ??????????)

• ????? ?????????? ?? ????????
• Known cause
• Asbestosis
• Fumes, gases
• Drugs (amiodarone, gold, antib.)
• Radiation/cytotox drugs

• ????? ????????????
• Known cause
• Organic dusts (HSP)
• Inorganic dusts (Pneumoconiosis)
• -Beryllium, Silica

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????? ????? ?? ILD (????...)

• ????? ?????????? ?? ????????
• UNKNOWN CAUSE
• Idiopathic interst. Pneumonias (7)
• CTD (RA, SLE, Sjogrens, DM/PM,
• Good Past. synd
• IBD

• ????? ???????????
• UNKNOWN CAUSE
• Sarcoidosis
• Langerhans cell gr.
• Granulomatous Vasculitides
• -Wegeners gr
• -Churg Strauss allergic gr.

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???? ???? ?????? ??????? ?? ???? ?????????? ??
ILD ?

• ?. ??????
• 1. ??? gt 50 ??? RA ,IPF
• 2. ??? ???? ????????????????????/
  ?????????????
• CTD (??? ?- RAILD).
• ????? ?????????????.
• 3. ????? ?????- IPF, ??????'? ????????????
• Goodpast. syndrome
• 4. ??? ?????/???????? Atypical Pneumonia ?
• 5. ???????? ??????? IPF, Tub.Scl, Neuro.F,
  Sarcoid

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?. ?????? (????...)

• 5. ??????
• ????? ??????? ??????? ???? !!!
• ?? ????????
• UTI -???????? (?????????)
• ???????? ?????? ????? (???????????)
• ???????? ??????
• ????????? ????????/??????
• ??' ????? - ???

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?. ?????? (??????...)

• ????? ???????? ??????????, ????? ?? ????? !!!
• ????? ??????? ???? ???????
• - ???? ???? (???????) ????? ???? HSP
• - Water Coolers HSP
• - ????/ ???? (????) HSP
• - ????? / ???????/ ?????? (?? ?? ???) ???
  ????????
  (??????????????)
• - ????????/??? (?????/?????? ????)

??? ??????
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?. ?????????

• ??????? ?? ??????. ????? ???? ????? !!
• ????? ????, ??????, ???, ???????? ??????????
  ?????
• ????????? ???????????, ??' ???????
• ??? ??? ?? ????. ?????IHD ?
• ???? ?-CTD, ???????? ??????
  ?????????
• ?????? ???????? ?- CTD ?????????
• ????????/????????, ????, ???, ?????
  ??????, ?????????
• ???? ??????? -(??????? ?????? ???? ???
  ???? ???? ?- RA, SLE, Poly/Dermatomyositis )

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?. ????? ????????

• 1. ???????? (crackels) ???? Velcro ??????
  (?? ?? ?' ??? ????).
• - ???? ???' ????????????? ??-.Lymphangitic
  Ca
• 2. Inspiratory Squeaks ???-??????, ??????,
• ???? ?-Bronchiolitis
• Cor Pulmonale 3 ?????? ????? !
• ,(P2?, RT. gallop, RT prec. lift)
• 4. Cyanosis ?? ????, ????? ?????.

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?. ????? ???????? (????...)

• 6. Clubbing ( ?????? ??????) ????? ?????.
• ???? ?-IPF, ILDCTD, ?????????
• 6. ??????? (???? ?????) ????????, CTD .
• 7. ?????? Uveitis, Gr vasculitis ????????, ????
  CTD .
• ????? ?? ?????? ???-???????
• ???? ????? ?? ? - D.D ! (???? ???? !!)

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?. ????? ???????

• ????? ??? ???? ???????? ????? !!! (?? ?????? ???
  !).
• ?' ??? 90 ?????. 10 ????? (HSP )
• ?????? ????????, ???????,
  ?????/????????
• CT ??? ????????? ????? (HRCT), ????? ?? 1
  ???- HRCT ?????? ????? ????? ??????? ILD !

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?. ?????

• 1. ?????, ?.?? -?? ???????? LDH?, ESR? ,GLOB?,
  ?? ?' ????, ??? (?????????????) ??' ????????
  
• 2. ???????? ???? ????? ?-CTD
• ANCA, ANF/aDNA, R.F, a-GBM, C 3,4, Scl
  70, anti-JO1, RNP, anticentromer, HSP
  precipitins.
• 3 . ?.?.? ???? ?? ???? ??? ?"? ?????
• 4. Gallium 67 ??? ????? ?? ?????? ??? ????
  ?????? ????/????????. ?? ??????.

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(PFT) ?. ?????? ????

• ?????? ????? ??? ! - ???? ??????? TLC, TGV
• ????? ??????? DLco
• ???? ???????? ????? ???? ??????!
• ??"? ?? ?????? ?????
• ????? ????? ?????? ?-DLCO ????
  
• ????????? ? ??????? ILD ?,
  ????? ILD ?
• 3. DLco ??"? ????

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?. ?????? ???? (????...)

• ???? ??????? (ABG) PaO2 ??????/????
• ???? ??? ??? ?????? ?????? ???? (???? ?-
  IPF ).
• 5. ?????????? ???? ????? ?????? ????????? ?????
  ?? ???? ???????.
• ?. ????????????
• ?????? ???? ???????, ???? ????????, HSP , ????
  ????????????. ?????? ????? ??? ????? ?????????
  ????? ??? ???? ??????? ??????? ?????.

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?????????? ?????????? ??????? ?? ????? ????
??????????????? ?????????? (IIP)ATS/ERS 2001-2,
New Classification

• ????? ??' ???? ??????????????? ?????? ?? ?????
• (???? ???? ???????? ????
  !)
• 1. Usual Interstitial Pneumonia (UIP)
  Idiopathic Pulmonary Fibrosis (IPF). The most
  common IIP
• 2. Desquamative Interstitial Pneumonia (DIP)
  same DIP
• 3. Respiratory Bronchiolitis (RB) Respiratory
  Bronchiolitis Interstitial Lung Disease (RBILD)

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?????????? (?????????????) ?? ????? ????
??????????????? ??????????????

• 4. Diffuse Alveolar Damage (DAD) Acute
  Interstitial Pneumonia (AIP)
• 5. Non-Specific Interstitial Pneumonia (NSIP)
  same (NSIP)
• 6. Organizing pneumonia Cryptogenic organizing
  Pneumonia (COP) or its synonymous- Bronchiolitis
  Obliterans with Organizing Pneumonia (BOOP).
• 7. Lymphocytic Interstitial Pneumonia same (LIP)

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??????? ?????????????? ?- IIP
NSIP AIP DIP/ RBILD UIP ??????
??????? ??????? ??????? ??????? ??? ???????
????? (????? ????????) ??? ??? ??? ????
?????, ?????? ??? ?????, ?????? ????! ?????! ?????
??????, ?????? ?????? ??? ???? ?????! ??????' ????????'
???? ?? ?? ?? "???-??? ? HRCT
???? ?? ?? ?? BOOP
??????, ????? ?? ??, ??????!!! ??????!! ??????, ????? ???????? ??????????
?? ??????, ????? ?? ?? ??????? ????????

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IIP ???????? ???????
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?????? ??????? ?????? ????????

• ?? ?????? ???? ????? ??????? ???? ? IPF. (????
  ???? ?? ???? "??? ???" ?-HRCT ). ?? ??? ????
  ???????????? ?????? ????? ???? ???? ?????
  ?????????.
• ?????? ??? ?????? ????? ?????? ?????/?????? ????
  ?- IPF ) ????? ??? ???? ?.?? ????? ?/?? ??"?
  ???)
• Surgical lung biopsy ???? ???????? ???????.
  ??? ??????? ??????????? (VATS). ????? ???? ?????
  ????? ??? ????? ?? ??? ??? !!

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IPF (clinical) UIP (patholog.)
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Normal Lung
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IPF
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AIP
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????? ????????? ?- IIP

• ????????? ???????? ??????? ??? ???? ? ILD ??????
  IPF
• IPF ????????? ???????, ?????? ?????. ?? 10-20
  ??????. ????? ??????? ???? ?- 3 ????.
• DIP/RBILD ????? ????? CS, ?????? ????.
• AIP ?????????, ????? ????? ???
• COP CS ?????? ????, ????? ?????.

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COP (cryptogenic organizing pneumonia)
Bronchiolitis Obliterans w/Organizing Pneumonia
(BOOP)

• ????????/?????????/ ?????? ???????? ???? ??
  ???????????????
• ???? ???????, ???' ????? ????. ???????? ?????
  ????? ????????. ??"? ???? ???? ?????? ????????'
• ??????? ?? COP/BOOP ????????
• 1. ???, ?????, ???? ?????
• ??????? ??????????/???????
• ?? ???? ???????????? ?? ???? ?????????? ?? ??
  ???? ?????? (????? ? ???????? ????? 1-2 ????).

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COP BOOP
???? ???? ????? ?????
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COP Idiopathic BOOP
????? ????? ??????
???????????
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??????????? SARCOIDOSIS (???? ??????????????
????????????)

• ?????
• ???? ?????? ??-??????? ?????? ?? ?????. ????
  ??????? ?? ?? ???? ????.
• ?????? ????????? ?? ?????????? T/CD4 ?????????
  ???? ????? ????????? ????????????? ??? ??????
  (??? ???????).
• ????? ????? ?????/????? ?? ????? - ??? ?? ?????
  ???? ??????? ?????? ???? ?? ????????.

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?????????

• ?? ?????.
• ????
• ???? ?????? ?????? ??????????? ?????? ???????/??
  ??????? ????? ????? ?? ????? ??? ?? ??????????
  ???? CD4.

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??????

• ?????? ??? ??????, ?? ??????, ?????, ??????
• ???? ????? ????? ??????? 10-40. ???? ??? ????
  ??????.
• ????? ????? ??????? ??? ???? ???? ?????????
  ????- ??????????? 13, ????? 15/105, ?????
  64/105, ???? 10/105. ???? ????????, ???????
  ??????????.
• ???? ?????? ???????, ?????? ??????? ????.
• ???? ??? ??????.
• ???? ?????? ????? ?????? ????? ???????.

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???????

• ????? ????? ??? ?? ??? ???? (??????, ???, ??????,
  ????? ?????).
• ??????? ?????? - 90
• ???????? ??????/????????? 1/3 ??????? (???, ????,
  ?????). ???????? ????? 2/3.
• Lofgren synd. Bilat. Hilar lymphadenopathy
  Erythema Nodosum arthritis
• Heerfordt synd. uveoparotid fever facialis.

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??????????? ??????

• ?????? ??????? ????, ???? ?????, ???/??-?????
  ????.
• ????? ????? ????????????, ??????????????
• ??????? ?????? ?????, ???????, ????????, ??? ??
  ????? (????/???? ).
• ?????? ?????????? ??-??????, ????????,
  ???????????? ???????????.
• ???? ??????? ?????? ? 1-5 ???????, ?? ????.
• 4 ????? ?? ???????? ??????? ?? ????????
  ?????????? !

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??????????? ???????? ???????/??????????
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????????
66
?????? ?????

• ??? 25 ???? ???, ????????, ???????
  ??-?????? Lupus pernio
• ?????? 25 ?? ???????, ?????, ????/????,
  ????????? (?? ?????? ???? )
• ???? ????? ??????? ??, ??????, ??????
• ?????????? 5-10 . ??? 60-90 ????? ????????,
  ???????.
• ????? ????. ????????? ?????????????/????????,
• CNS 5 . ??? ????? ?????????, ???????. ?????????
  ??????? ????.
• ?? 5 ?? ????? ?? ???? ???. ????????,
  ??????????. PMD.

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?????? ????? (????)

• ?????? ??????????? ????????, ??-????, ??????????
  (???? ?????).
• ?????? ??????????? ?????????/ ????????, (Diab
  Insip) , ??????? (??????) - ????.
• ????? ??????. ????????, ??????, ????? ???? ?????
  ???????. ???????? ?? ????????.
• ??-??? 15-40 ???? ???????????.

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?????

• 1. . ??? ????? ??-?????? ????? ???/ HRCT
• 2. ?????????? ??Non-caseating granuloma .
  ??????? ??? ???? ????. ??????? ??? ??????.
• 3. ????? ????? ????? ?????????? ??? ??????
  (??????????, ??????, HIV, ????? ???? ???????
  ?????, ???????, ?????????).
• 4. ???????????? TBB ????????? ???????
  50-85 ???? ?- x-ray stage.
• ??? ?????? ???????????? !! ?????? ?? ??? ??????
  !!!

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?????

• ????? ????? ??????????
• ???, ???????? ?????? ???.
• NSAID
• ?????????, Er. Nodosum, ???? ??????.
• ????????? ????????
• ?????? ????????? ???????? ????? ?????.
• ????????? ????? ??????????????? ????? ?? ??????
  ???????? ?? ??? ????? ???? ????????????? (?????,
  ??, ??????, ?????, CNS )
• ???????? 30-40 ?? ?- 1-3 ? ?????? ????? ??????
  5-10 ?? ????? 12 ?.
• .

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??????? ?????

• ?????????? ?????? ????????. ??????, MTX,
  ???????.
• ????????-?????????? (????????) ???????????
  ?????? ?????.
• ????????? - ??????????? ???????? (????? ??? 1,25
  ?????????????? D ).
• ???????? ???? ????, ?????? ???? ????? ?? ???
  ?????.
• ????? 3-10
• ?????? ??-?????, ????? ???????????
• ????? ???????, ??? ????-??

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?????? ?? ??????? ?????????
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????? BAL ?????? ILD

• IPF ???????????/???????????? ???? ?????
  ??????????
• Sarcoidosis ???????????? CD4/CD8 ???? ????????
  ????
• 8CD ?????, ??????????? gt3, Mast cellsgt1
  ???? ??????
• BOOP ????? ?????? ??????????, ????????????,
  ???????????
• ???????????? ?CD4/CD8
• CTD ???? ???? /????? ??????? ??????, ?????,
  ???????????, ?????
• Alveolar Hemmorhage Synd ????? ????,
  ????????????? (PAM), ?????? ?????????
• Drug-Induced ILD ??"? ?CD4/CD8

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????? BAL ?????? ILD (????)

• Chronic Aspiration Lipid-laden macrophages
  (Sudan black or Oil Red ), Multinucleated giant
  cells
• 
  
• Hypersensitivity Pneumonitis (HSP)
• Lymphocytosis gt 50
• CD4/CD8 lt 1.0
• Mast cells gt 1
• Plasma Cells ! (Continuous expos to Ag)
  
• Eos/Neut - in advanced disease.
  

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?????? ?????? / Clubbing