Interstitial Lung Disease

Approach to INTERSTITIAL LUNG DISEASE

-Prof. Dr. MAGHESHKUMAR UnitDr. Devendra Patil

52 / F comes with complains of Cough 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 interstitial opacities

• 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 or Open lung biopsy

• Which ILDs have good prognosis• Whats the Supportive Treatment

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

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

IDIOPATHIC INTERSTITIAL PNEUMONIA

NS- UIPAIPCOP/BOOPDIPRB-ILD

IPF

Smoking related

Due to KNOWNCAUSE

EnvironmentalPneumoconiosisHPGases n fumesIatrogenicDrugsIrradiationMicrobesDCTD

GRANULOMATOSISsarcoidosis

Langerhans cellhistiocytosis

Wegener'sgranulomatosis,

Churg-StraussSyndrome

RARE ILD

alv.proteinosisalv.microlithiasisamyloidosiseosinophilic pneumonialymphangioleiomyomatosisidiopathic pulmonaryhemosiderosis

INTERSTITIAL LUNG DISEASE

INTERSTITIAL LUNG DISEASEOn basis of PFT and DLco

Is it due to environmental / iatrogenic factors

Avoid those factors and monitor response

Is it due to a systemic diseaseOr microbial origin

No response

SerologySkin BiopsySputum c/s

HRCT and BAL

TBLB or Open Lung Biopsy

Can Diagnosis and prognosis be established

HISTORY

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 disease•Severe pulmonary dysfunction•Major operative risk

Environment Dependent ILD

MINING INDUSTRY:• Coal workers pneumoconiosis• Silicosis• Asbestosis

HYPERSENSITVE PNEUMONITIS

GAS or FUME Exposure

Coal miners pneumoconioisis

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

small irregular and linear opacities

Progressive massive fibrosis almost 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 combination of rheumatoid disease and several round nodules (usually 1 to 5 cm in diameter) in the lungs of a coal miner.

SILICOSISClues to diagnosis

Micronodular pattern

Simple silicosis :Upper lobesSmall multiple nodulesEgg shell calcification

Complicated :>1 cm nodules

Acute silicosis :small nodular pattern with ground glass appearance ( crazy paving )

PMF : nodules coalesce to large masses

BAL : dust particles on polarised light

Clues to diagnosis

X Ray:reticular interstitial pattern pleural plaques ( lower lung field , cardiac border 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

•HISTORY of exposure to an offending antigen•Temporal association +nt• characteristic signs and symptoms•PFT and Imaging ( ILD pattern )•presence of granulomatous inflammation•Absence of eiosinophilia•BAL : marked lymphocytosis > 50%

HYPERSENSITIVITY PNEUMONITIS

Suspect a CTD if

Musculosketetal painWeaknessFatigueJoint pains and swellingPhotosensitivityRaynauds phenomenonPleuritis Dry eyes or mouth

INTERSTITIAL LUNG DISEASE in CTD

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 reticular pattern being highly characteristic.

HRCT in RAbibasilar peripheral reticular pattern, intralobular interstitial thickeningdistortion of the lung parenchymaBilateral is present, predominantly on the left side

bibasilar peripheral reticular pattern,

pleural effusion

thickening of the interlobular septa,

Vasculitic Disorders

Lung Involvement ANCA Interstial Pattern seen

Wegener granulomatosis

Common c-ANCA >> p-ANCA80–90%

Diffuse Alveolar Hemorrage with nodules ,cavitation

Microscopic polyangiitis

Common Common p-ANCA > c-ANCA80%

DAH

Churg-Strauss syndrome

Common p-ANCA > c-ANCA30–50%

DAH with transient infiltates

Goodpasture syndrome

Common p-ANCA10%

DAH

Takayasu arteritis Common Negative “

INTERSTITIAL LUNG DISEASE in VASCULITIC DISORDERS

X ray : consolidation, typically resolving within a matter of days, multiple abcesses

HRCT : ground-glass partial alveolar filling. Hb : anaemia ( iron defeciency )BAL :- frank blood-staining in sequential lavage (acute presentation) andnumerous macrophages containing iron, identified by Perl's stainDlco :- may be increased in acute conditions but is chronically low

MC seen is Wegeners Granulomatosis

ILD in VASCULITIC DISORDERS

Suspect if

Mononeuritis mutiplexRenal involvementSkin lesionshaemoptysis

DRUG and IRRADIATION and GAS

• DRUGS AmiodaroneBleomycin Busulphan CarmustineChlorambucil Cyclophosphamide Cytosine arabinoside

Lomustine ….)

RADIATION



IPF

Smoking related

Due to KNOWNCAUSE






RARE ILD



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

years, depending on stage at presentation.

• B/L Reticular bibasilar and subpleural opacities. minimal ground-glass and variable honeycomb change.

• Type I pneumocytes are lost, and there is proliferation of alveolar type II cells. "Fibroblast foci" of actively proliferating fibroblasts and myofibroblasts.

Disease Age M:F

C/F Imaging Prognosis REMARKS

Respiratory bronchiolitis- associated interstitial lung disease

younger Heavy smokerswith similar complains

Like UIP withAirtrappingEmphysematous change

survival greater than 10 years

Spontaneous remission 20%.

ILD with Obstructiv pattern

Acute interstitial pneumonitisHamman-Rich syndrome.

young Apparently normal

indistinguishable from that of idiopathic ARDS

ARDS

Diffuse b/l airspace consolidation with areas of ground-glass attenuation

POOR Most severe formof ILDPneumonia

Disease Age M:F

C/F Imaging Prognosis REMARKS

Nonspecific interstitial pneumonitis (NSIP)

40-50 May be indistinguishable from UIP

Like But uniform in time, suggesting response to single injury UIPHoneycombing is rare.

Prognosis good but depends on the extent of fibrosis at diagnosis greater than 10 years.

But Surgical Biopsy is needed to confirm.

Cryptogenic organizing pneumonitis (bronchiolitis obliterans organizing pneumonia [BOOP])

50–60 Abrupt onset, frequently weeks to a few months following a flu-like illness. constitutional symptoms are common

Ground glass infiltrate subpleural consolidation and bronchial wall thickening and dilation. Xray – interstitial pattern with nodules

Good Rule out infection and treat with steroids

Acute interstitial pneumonitis

Nonspecific interstitial pneumonitis (NSIP)

Cryptogenic organizing pneumonitis (bronchiolitis obliterans organizing pneumonia [BOOP])

Smoking related ILD

Respiratory bronchiolitis- associated interstitial lung disease



IPF

Smoking related

Due to KNOWNCAUSE






RARE ILD



Sarcoidosis

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

SARCOIDOSIS ctd….

BAL :- lymphocytosis CD4 : CD8 > 3.5 is most specific

PFT :- Restrictive pattern But Obstructive component present in many

Biopsy :- non caseating granulomaslymphocytosis

Sr. ACE levels:-Hyper calciuria or Hypercalcemia

RARE ILD

Primary Alveolar Microlithiasis

perilobular and bronchovasculardistribution of microliths and subpleural consolidation with calcifications inthe right lung

SAND STORM appearance

Pulmonary Alveolar Proteinosis

diffuse reticulo-alveolar infiltrates BAT WING distribution

BAL:- milky effulent foamy macrophages with lipoproteinous intraalveolar material

thickened interlobular septa“crazy paving” ground glass fashion, sharply demarked from normal lung creating a “geographic” pattern.

TREATMENT• Removal of offending agent if noted• Aggressive suppression on inflammatory response• Supportive management ( O2 or )• Treatment of Right heart Failure• Treatment of Infections• Combined effort from family , doctors , physioherapists.

CYCLOPHOSPHAMIDE or AZATHIOPRINE

• IPF• Other ILD as 2nd line drugs

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

STEROIDS

BOOPCTD – ILDEiosinophilic pneumoniaInorganic Dust ILDVasculitic ILDOrganic Dust

Dose :- 0.5 – 1 mg / kg prednisone for 4 – 12 weeks and then gradual tapering of the dose with repeated monitoring for flare up activity

THANK - -

YOUReferences:

Harrisons 16/eAtlas Of ILD by OP SharmaOxford’s Text book of Medicine 4/e

Interstitial Lung Disease

Health & Medicine

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