diffuse parenchymal lung disease
DESCRIPTION
ildTRANSCRIPT
Case Approach in Interstitial
Lung DiseasesSuree Sompradeekul, MD, FCCPAssistant Professor of Medicine
Division of Respiratory Disease & TuberculosisDept of Medicine, Siriraj Hospital
Diffuse Diffuse parenchymalparenchymal lung diseaselung disease
DPLD of known DPLD of known cause or associationcause or association
Idiopathic Idiopathic interstitial interstitial
pneumoniaspneumonias
GranulomatousGranulomatous Other forms of Other forms of interstitial pneumonia interstitial pneumonia
e.g. LAM, PLCH, e.g. LAM, PLCH, eosinophiliceosinophilic
pneumonia, etc.pneumonia, etc.Idiopathic Idiopathic pulmonary pulmonary
fibrosis fibrosis
Interstitial pneumonia Interstitial pneumonia other than idiopathicother than idiopathicpulmonary fibrosispulmonary fibrosis
DesquamativeDesquamative interstitialinterstitialpneumonia pneumonia
Respiratory Respiratory bronchiolitisbronchiolitisInterstitial lung diseaseInterstitial lung disease
Acute interstitial Acute interstitial pneumonia pneumonia
Cryptogenic Cryptogenic organisingorganising pneumoniapneumonia
NonNon--specificspecificInterstitial pneumoniaInterstitial pneumonia
LymphocyticLymphocyticInterstitial pneumoniaInterstitial pneumonia
ILD associated with systemic diseaseRheumatogical
Systemic sclerosisRheumatoid arthritisSystemic lupus erythematosusSjÖgren’s syndromeAnkylosing spondylitisPolymyositis/dermatomyositisMixed connective tissue disease
VasculitisGiant cell arteritisTakayasu’s arteritisMicroscopic polyangiitisWegener’s granulomatosisChurg-Strauss granulomatosisBehcet’s syndromePulmonary-renal syndrome (including Goodpasture’s syndrome)Capillaritis
ILD associated with systemic disease
Vascular Antiphospholipid syndromeCoagulopathiesArteriovenous malformationsPrimary pulmonary hypertensionIdiopathic pulmonary hemosiderosisPulmonary veno-occlusive disease
Disease caused by environmental triggers or drug ingestion
Drug*ChemotherapeuticCardiovascularAntibioticsAnti-inflammatoryIllicitPsychotropicRadiationPesticidesOxygen
Environmental (organic causes)Extrinsic allergic alveolitis
FungalBacterialAvianChemical
Inorganic causesFibrogenic inorganic dusts
AsbestosSilicaHard metal alloyBerylliumCoalAluminium
Non-fibrogenicSiderosisStannosisBaritosisAntimony
Idiopathic interstitial pneumoniasIdiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis)Desquamative interstitial pneumonia (DIP)Acute interstitial pneumonia (AIP)Non-specific interstitial pneumonia (NSIP)Respiratory bronchiolitis/interstitial lung disease (RB-ILD)Cryptogenic organising pneumonia (COP or idiopathic BOOP)Lymphocytic interstitial pneumonia (LIP)
Histologic & Clinical Classification of ILDHistologic patterns Clinical-Radiologic-
Pathologic DxUsual interstitial pneumonitis
Nonspecific interstitial pneumoniaOrganizing pneumonia
Diffuse alveolar damageRespiratory bronchiolitis
Desquamative interstitial pneumoniaLymphoid interstitial pneumonia
Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitisNonspecific interstitial pneumonia (provisional)Cryptogenic organizing pneumonia (BOOP)Acute interstitial pneumoniaRespiratory bronchiolitis interstitial lung diseaseDesquamative interstitial pneumonia
Lymphoid interstitial pneumonia
Granulomatous diseasesSarcoidosisBerylliosisHypersensitivity pneumonitis or Extrinsic allergic alveolitisLangerhan’s cell histiocytosisWegener’s granulomatosisChurg-Strauss syndromeLymphomatoid granulomatosisBronchocentric granulomatosis
Other diffuse lung diseasesInherited disorders
Tuberous sclerosisNeurofibromatosisHermansky-Pudlak syndromeLipid storage disordersFamilial fibrosing alveolitis
Pulmonary eosinophiliaKnown causes, e.g. fungi, parasites, drugsUnknown causes, e.g. acute and chronic idiopathic
eosinophilic pneumoniaLymphangioleiomyomatosisAlveolar proteinosisAlveolar microlithiasisAmyloidosisChronic aspirationChronic left heart failure
Clinical manifestationProgressive dyspneaNon-productive coughPleuritic chest pain (pneumothorax)Hemoptysis +/- Fe def anemiaWheezingAbnormal CXRSymptoms related to associated diseases e.g. CNTRestrictive pattern from spirometry
Physical examinationIncreased RRRespiratory distressCracklesInspiratory squeaks – late inspiratoryhigh-pitched rhonchi; bronchiolitisCyanosis, clubbing of fingers, corpulmonale – advanced diseaseExtrapulmonary findings
Extrapulmonary findingsHypertensionSkin: EN, maculopapular rash, heliotrope, telangiectasis, Raynaud’s, Café-au-lait spots, vasculitis, subcut nodules, albinism, calcinosisEye: uveitis, scleritis, keratoconjunctivitissiccaSalivary gland enlargementLymphadenopathyHepatospenomegalyPericarditis, myocarditisMuscle weakness
Duration of illnessAcute (days to weeks)
Acute idiopathic interstitial pneumonia (AIP, Hammon-Rich syndrome)Eosinophilic pneumoniaHypersensitivity pneumoniaBronchiolitis obliterans with organizing pneumonia (BOOP) or cryptogenic organizing pneumonia (COP)
Subacute (weeks to months)SarcoidosisDrug-inducedAlveolar hemorrhage syndromesIdiopathic BOOPCNT (SLE, polymyositis)
Chronic (months to years)Idiopathic pulmonary fibrosis (IPF)SarcoidosisPulmonary Langerhans cell histiocytosis (PLCH) or eosinophilicgranuloma (EG)
Occupation & environmental exposures
Inhaled organic dusts (hypersensitivity pneumonitis)
Thermophilic fungi; farmer’s lung, grain handler’s lung, humidifier or air conditioner lungBacteria (Bacillus spp); humidifier lungTrue fungi (aspergillus, cryptostorma coricale, penicilliumAnimal proteins; bird fancier’s disease
Occupation & environmental exposuresInhaled inorganic dust
SilicatesSilica (silicosis)Asbestosis (asbestosis)Talc (talcosis)Kaolin or china clayDiatomaceous earth (Fuller’s earth)Nepheline (hard rock)Aluminum silicates (sericite, sillimanite, ziolite)Portland cementMicaBeryllium (berylliosis)
CarbonCoal dust (pneumoconiosis)graphite
MetalsTins (stannosis)Aluminum; powdered aluminum, bauxiteHard metal dusts: cadmium, titanium, tungsten, hafnium, niobium, cobalt, vanadium carbidesIron (siderosis, arc welder’s lung)AntimonyHematite (siderosilicosis)Mixed dusts of silver and iron oxide (argyrodiserosis)Barium (powder of baryteor BaSO4- (baritosis)CuSO4 neutralized with hydrated lime (vineyard sprayer’s lung)Rare earths, Ocerium scandium, yttrium , lanthanum
Occupation & environmental exposuresInhaled other agents
Chemical sourcesSynthetic-fiber lung (nylon, acrylic, polyesters, orlon)Bakelite worker’s lungVinyl chloride, polyvinyl chloride powder
GasesOxygenOxides of nitrogensulfur dioxideChlorine gasesMethyl isocyanate
FumesOxides of zinc, copper, manganese, cadmium, iron, magnesium, nickel, brass, selenium, tin, antimonyDiphenylmethane diisocyanateTrimellitic anhydride toxicity
VaporsHydrocarbonsThermosetting resins (rubber tire workers)Toluene diisocyanate (TDI-asthmatic reactions prominent)Mercury
AerosolsOilsFatsPyrethrum (natural insecticide)
Laboratory dataLeukopenia: sarcoidosis, CNT, lymphoma, drug-inducedLeukocytosis: HP, lymphoma, vasculitisEosinophilia: eosinophilic pneumonia, sarcoidosis, vasculitis, drug-induced (sulfa, methotrexate)Thrombocytopenia: sarcoidosis, CNT, drug-induced, Gaucher’s disHemolytic anemia: CNT sarcoidosis, lymphoma, drug-inducedNormocytic anemia: alv hemorrhage syndrome, CNT, lymphangitic carcinomatosisElevated LDH: alv proteinosis, IPFHypogammaglobulinemia: LIPHypergammaglobulinemia: CNT,sarcoidosis, vasculitis, LIP, lymphoma, silicosis
Laboratory dataSerum immune complexes: IPF, LIP, vasculitis, CNT, EGACE: sarcoidosis, HP, silicosis, Gaucher’s diseaseAntibasement membrane Ab (anti-GBM):Goodpasture’s syndromeANCA: Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitisSerum precipitating Ab: HPLymphocyte transformation test to specific antigens: chronic beryllium disease, aluminum potroom workers disease, gold-induced pneumonitis
IPFIPF DIPDIP RBRB--ILDILD AIPAIP BOOPBOOP NSIPNSIP
Age (years) 65 40 35 50 55 55
Occurrence in children
No Rare No Rare No Occasional
Onset Chronic Chronic Chronic Acute Acute/subacute
Subacute/Chronic
Clubbing Frequent Frequent No No No Occasional
Fever Rare No No 50% 70% 20%
Mortality (%) 70 27 0 8 13 11
Mean survival 2.8 years 12 years Not reduced
1.5 months
> 10 years
13 years
Response to corticosteroids
Poor Good Good Poor Good Good
Disease Radiographic features CT Differential Dx
IPF Peripheral, basal reticular abnormalities with volume loss
Asbestosis, CNT, HP, sarcoidosis
DIP Ground glass opacity RB-ILD, HP, sarcoidosis, PCP
RB-ILD Bronchial wall thickening, ground glass opacity
DIP, NSIP, HP
AIP Progressive diffuse ground glass density/consolidation
Hydrostatic edema, pneumonia, acute eosinophilic pneumonia
COP Patchy bilateral consolidation Infection, vasculitis, sarcoidosis, alveolar carcinoma, lymphoma, eosinophilic pneumonia, NSIP
LIP Reticular opacity, nodules Sarcoidosis, lymphangiticcarcinomatosis, PLCH
NSIP Ground glass and reticular opacity
UIP, DIP, COP, HP
Disease Distribution CT Pattern
IPF Peripheral, subpleural, basal Reticular, honeycombing, traction bronchiectasis, minor focal ground-glass
DIP Peripheral, subpleural, basal Predominant ground glass, minor reticular lines
RB-ILD Diffuse Bronchiolar-wall thickening, centrilobular nodules, patchy ground-glass
AIP Diffuse Ground glass with focal lobular sparing, consolidation, later traction bronchiectasis
COP Subpleural, peribronchial Patchy consolidation and/or nodules
LIP Diffuse Centrilobular nodules, ground glass attenuation, septal and bronchial wall thickening, thin-walled cysts
NSIP Peripheral, subpleural Predominant ground glass, basal reticular lines, consolidation
Acute interstitial pneumoniaDiffuse alveolar hemorrhage (vasculitis or coagulopathy most common causes)Acute pneumonia due to rheumatological diseaseDrugs (selected)Eosinophilic lung disease, acute or chronicCryptogenic and known causes of organizing pneumonia (BOOP)Mimics
− Pulmonary edema of any cause− Infection, especially opportunistic with
Pneumocystis carinii− Acute respiratory distress syndrome
Acute onset ILD
Diffuse lung disease with characteristic features on HRCT
Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis)Desquamative interstitial pneumoniaExtrinsic allergic alveolitisSarcoidosisLangerhans cell histiocytosisLymphangioleiomyomatosisAlveolar proteinosisLymphangitic carcinomatosis
Predominant distribution on HRCTUpper-lobe predominance Sarcoidosis
Coalworker’s pneumoconiosisSilicosisEosinophilic pneumoniaLangerhans cell histiocytosisExtrinsic allergic alveolitis
Lower-lobe predominance Idiopathic pulmonary fibrosisDesquamative interstitial pneumonia AsbestosisCollagen vascular disease
Peripheral predominance Idiopathic pulmonary fibrosisDesquamative interstitial pneumoniaAsbestosisCollagen vascular diseaseEosinophilic pneumoniaOrganising pneumonia
Central (peribronchovascular) predominance
SarcoidosisLymphangitis carcinomatosis
Pattern in HRCTLinear and reticular Idiopathic pulmonary fibrosis
Collagen vascular diseaseAsbestosis
Nodular or reticulonodular SarcoidosisSilicosisLymphangitis carcinomatosis
Parenchymal opacification(consolidation/ground-glass)
Organising pneumoniaExtrinsic allergic alveolitisDesquamative interstitial pneumoniaRespiratory bronchiolitis/interstitial lung
diseaseChronic eosinophilic pneumonia HaemorrhageAlveolar proteinosis
Cystic abnormalities Langerhans cell histiocytosisLymphangioleiomyomatosis
Neutrophilic(± eosinophilic)
Idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Collagen vascular diseaseAsbestosisAcute interstitial pneumonia
Lymphocytic SarcoidosisExtrinsic allergic alveolitisSilicosisLymphocytic interstitial pneumonia
Eosinophilic (Chronic) eosinophilic pneumonia Churg-Strauss syndromeHypereosinophilic syndrome
Mixed cellularity Bronchiolitis obliterans organizing pneumoniaCollagen/vascular diseaseNon-specific interstitial pneumonia
Abnormal macrophage morphology
Extrinsic allergic alveolitisAlveolar proteinosisRespiratory bronchiolitis/interstitial lung diseaseAlveolar hemorrhage
BAL cell
Initial assessment:• Breathlessness• Crackles on auscultation• Pulmonary function: reduced
TL CO, restrictive defect• Chest radiograph: compatible
with interstitial fibrosis• Absence of environmental or systemic disease cause
• High-resolution CT• Bronchoalveolar lavage± transbronchial biopsy
Diagnosis and prognosis
Yes No
Thoracoscopic or open lung biopsy
• Reversible disease on biopsy or CT ?• Progressive disease ?• Moderate functional impairment ?
Yes No
Treat If deteriorates
Observe (but monitor with serial pulmonary function tests)
Thank you & GOOD LUCK !!!!!