interstitiallungdisease-100122140036-phpapp01.ppt

8/11/2019 interstitiallungdisease-100122140036-phpapp01.ppt

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52 / F comes with complains ofCough with minimum mucoid expectoration 6-7 yrsDOE gradually progressive 3-4 yrsHOPI :-No H/o fever,No h/o pul TBNo h/o palpitations,PND , orthopnea,

O/e:Tachypnoea and Bibasilar Inspiratory CracklesClubbing +nt.

X ray was advised and it showed some B/L interstitialopacities


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How to suspect an INTERSTITIAL LUNG

DISEASE. How to find its Cause How to differentiate using imaging and

simpler procedure rather than doing a TBLB orOpen lung biopsy

Which ILDs have good prognosis

Whats the Supportive Treatment


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COMMON FEATURES OF ILD History :Chronic non productive cough with progressive exertional

dysnoea. Examination :-Tachypnoea +/- Respiratory distressCynosis and clubbingBibasilar Inspiratory cracklesf/s/o pul HT and cor pulmonale IMAGING : - Interstitial pattern PFT:- Restrictive pattern DLco :- Reduced


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IDIOPATHICINTERSTITIALPNEUMONIA

NS- UIP

AIPCOP/BOOPDIPRB-ILD

IPF

Smoking related

Due to KNOWNCAUSE

EnvironmentalPneumoconiosis

HPGases n fumesIatrogenicDrugsIrradiationMicrobes

DCTD

GRANULOMATOSISsarcoidosis

Langerhans cellhistiocytosis

Wegener'sgranulomatosis,

Churg-Strauss

Syndrome

RARE ILD

alv.proteinosisalv.microlithiasisamyloidosiseosinophilic pneumonialymphangioleiomyomatosisidiopathic pulmonaryhemosiderosis

INTERSTITIAL LUNG DISEASE


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INTERSTITIAL LUNG DISEASEOn basis ofPFT and DLco

Is it due toenvironmental /iatrogenic factors

Avoid those factors andmonitor response

Is it due to asystemic diseaseOr microbialorigin

No response

SerologySkin BiopsySputum c/s

HRCT and BAL

TBLB orOpen LungBiopsy

CanDiagnosis andprognosis beestablished

HISTORY


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ILD with obstructive component

Sarcoidosis Hypersensitivity pneumonitis Langerhans cell granulomatosis Lymphangioleiomyomatosis

Tuberous sclerosis Combined COPD and ILD

RELATIVE CONTRA INDICATIONS FOR A LUNG BIOPSY

Honey combing or evidence of end stage diseaseSevere pulmonary dysfunctionMajor operative risk


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Environment Dependent ILD

MINING INDUSTRY:

Coal workers pneumoconiosis

Silicosis Asbestosis

HYPERSENSITVE PNEUMONITIS

GAS or FUME Exposure


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oal minerspneumoconioisis

Rounded opacities between 1 and 5 mm(upper and middle zones)

small irregular and linear opacities

Progressive massive fibrosisalmost always starts in an upper zone

Calcification is not a feature

Cavitation of PMF can occur

Caplan's syndrome is the name given to the combinationof rheumatoid disease and several round nodules (usually1 to 5 cm in diameter) in the lungs of a coal miner.


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SILI OSISClues to diagnosisMicronodular pattern

Simple silicosis :Upper lobesSmall multiple nodulesEgg shell calcification

Complicated :>1 cm nodules

Acute silicosis :

small nodular pattern with ground glassappearance ( crazy paving )

PMF : nodules coalesce to large masses

BAL : dust particles on polarised light


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Clues to diagnosis

X Ray:reticular interstitial patternpleural plaques ( lower lung field , cardiacborder and diaphragm )Irrregular linear opacities first noted in

lower lung fields.

HRCT :Distinct subpleural curvilinear opacities 5-10 mm length parallel to pleural surface

BAL: Asbestos bodies

ASBESTOSIS


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HISTORY of exposure to an offending antigenTemporal association +nt

characteristic signs and symptomsPFT and Imaging ( ILD pattern )presence of granulomatous inflammationAbsence of eiosinophiliaBAL : marked lymphocytosis > 50%

HYPERSENSITIVITY PNEUMONITIS


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Suspect a CTD if

Musculosketetal painWeaknessFatigueJoint pains and swellingPhotosensitivity

Raynauds phenomenonPleuritisDry eyes or mouth

INTERSTITIAL LUNG DISEASE in CTD


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SYSTEMIC SLEROSISLung manifestation may be first SS sign in 55%Lung involvement +nt in 90 % ( detected by PFT )

Vascular Involvement is not vasculitis but intimal hypertrophy (CREST )

RAMC lung manifestation : Fibrosing alveolitisMale predominancePleural diseasePleuro pulmonary nodules (may cavitate to produce pneumothorax )Caplan Syndrome

SLEILD is rare . Pleural involvement is common

POLYMYOSITIS / DERMATOMYOSITISILD in 10 %a combination of patchy consolidation with a peripheral reticularpattern being highly characteristic.


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HRCT in RA

bibasilar peripheral reticular pattern,intralobular interstitial thickeningdistortion of the lung parenchymaBilateral is present, predominantly on theleft side

bibasilar peripheral reticular pattern,

pleural effusion

thickening of the interlobular septa,


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VasculiticDisorders Lung Involvement ANCA Interstial Patternseen

Wegenergranulomatosis

Common c-ANCA >> p-ANCA80 90%

Diffuse AlveolarHemorrage withnodules ,cavitation

Microscopicpolyangiitis

Common Common p-ANCA >c-ANCA80%

DAH

Churg-Strausssyndrome

Common p-ANCA > c-ANCA30 50%

DAH withtransient infiltates

Goodpasturesyndrome

Common p-ANCA10%

DAH

Takayasu arteritis Common Negative

INTERSTITIAL LUNG DISEASE inVASCULITIC DISORDERS


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X ray : consolidation, typically resolving within a matter of days, multipleabcessesHRCT : ground-glass partial alveolar filling.Hb : anaemia ( iron defeciency )BAL :- frank blood-staining in sequential lavage (acute presentation) and numerous macrophages containing iron, identified by Perl's stain

Dlco :- may be increased in acute conditions but is chronically low

MC seen is WegenersGranulomatosis

ILD in VASCULITICDISORDERS

Suspect if

Mononeuritis mutiplexRenal involvementSkin lesionshaemoptysis


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DRUG and IRRADIATION and GAS

DRUGSAmiodarone

Bleomycin

BusulphanCarmustine

ChlorambucilCyclophosphamide

Cytosine arabinoside

Lomustine .)

RADIATION


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NS- UIP


IPF

Smoking related

Due to KNOWNCAUSE

EnvironmentalPneumoconiosisHPGases n fumesIatrogenicDrugsIrradiationMicrobesDCTD




Churg-StraussSyndrome

RARE ILD

alv.proteinosisalv.microlithiasisamyloidosiseosinophilic pneumonialymphangioleiomyomatosisidiopathic pulmonaryhemosiderosis



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UIP or IPF MC of all chronic ILD Typical c/f presentation Median survival approximately 3

years, depending on stage atpresentation.

B/L Reticular bibasilar and subpleuralopacities. minimal ground-glass andvariable honeycomb change.

Type I pneumocytes are lost, and thereis proliferation of alveolar type II cells.

"Fibroblast foci" of activelyproliferating fibroblasts andmyofibroblasts.


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Disease AgeM:F

C/F Imaging Prognosis REMARKS

Respiratorybronchiolitis-associatedinterstitiallung disease

younger Heavysmokerswithsimilarcomplains

Like UIP withAirtrappingEmphysematous change

survivalgreaterthan 10years

Spontaneousremission20%.

ILD withObstructivpattern

Acuteinterstitial

pneumonitisHamman-Richsyndrome.

young Apparentlynormal

indistinguishable fromthat ofidiopathicARDS

ARDS

Diffuse b/lairspaceconsolidation with areasof ground-glass

attenuation

POOR Mostsevere

formof ILDPneumonia


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Disease AgeM:F

C/F Imaging Prognosis REMARKS

Nonspecificinterstitial

pneumonitis(NSIP)

40-50 May beindistinguishable

from UIP

LikeBut uniform in

time, suggestingresponse tosingle injury UIPHoneycombing israre.

Prognosisgood but

depends onthe extentof fibrosis atdiagnosisgreater

than 10years.

ButSurgical

Biopsy isneeded toconfirm.

Cryptogenicorganizingpneumonitis(bronchiolitis

obliteransorganizingpneumonia[BOOP])

50 60 Abrupt onset,frequently weeksto a few monthsfollowing a flu-like

illness.constitutionalsymptoms arecommon

Ground glassinfiltratesubpleuralconsolidation

and bronchialwall thickeningand dilation. Xray interstitialpattern withnodules

Good Rule outinfectionand treatwith

steroids


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Acute interstitial pneumonitis


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Nonspecific interstitial pneumonitis(NSIP)


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Cryptogenic organizing pneumonitis (bronchiolitisobliterans organizing pneumonia [BOOP])


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Smoking related ILD

Respiratory bronchiolitis- associatedinterstitial lung disease


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NS- UIP


IPF

Smoking related

Due to KNOWNCAUSE

EnvironmentalPneumoconiosisHPGases n fumesIatrogenicDrugsIrradiationMicrobesDCTD




Churg-StraussSyndrome

RARE ILD

alv.proteinosisalv.microlithiasisamyloidosiseosinophilic pneumonialymphangioleiomyomatosisidiopathic pulmonary

hemosiderosis



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Sarcoidosis

Incidental X-ray (20-30 %) Cough , chest discomfort ( upto 50 60 % ) Skin lesions ( 20 -25 % )


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SARCOIDOSIS ctd.

BAL :- lymphocytosis

CD4 : CD8 > 3.5 is most specificPFT :- Restrictive pattern

But Obstructive component present in manyBiopsy :- non caseating granulomas

lymphocytosis

Sr. ACE levels:-Hyper calciuria or Hypercalcemia


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RARE ILD


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Primary Alveolar Microlithiasis

perilobular and bronchovasculardistribution of microliths and subpleuralconsolidation with calcifications in

the right lung

SAND STORM appearance


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Pulmonary Alveolar Proteinosis

diffuse reticulo-alveolar infiltratesBAT WING distribution

BAL:- milky effulent foamymacrophages with lipoproteinous

intraalveolar material

thickened interlobular septa

crazy paving ground glassfashion, sharply demarked fromnormal lung creating ageographic pattern.


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TREATMENT

Removal of offending agent if noted Aggressive suppression on inflammatory response Supportive management ( O 2 or ) Treatment of Right heart Failure

Treatment of Infections Combined effort from family , doctors , physioherapists.


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CYCLOPHOSPHAMIDE or AZATHIOPRINE

IPF Other ILD as 2 nd line drugs

1-2 mg / kg /day with or without steroids

STEROIDS

BOOP

CTD ILDEiosinophilic pneumoniaInorganic Dust ILDVasculitic ILDOrganic Dust

Dose :-0.5 1 mg / kg prednisone for 4 12weeks and then gradual tapering of thedose with repeated monitoring for flareup activity


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References:

Harrisons 16/e Atlas Of ILD by OP SharmaOxfords Text book of Medicine 4/e

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