Approach to INTERSTITIAL LUNG DISEASE -Prof. Dr. MAGHESHKUMAR Unit Dr. Devendra Patil
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
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
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
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
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