Case Approach in Interstitial Lung Diseases Suree Sompradeekul, MD, FCCP Assistant Professor of Medicine Division of Respiratory Disease & Tuberculosis Dept of Medicine, Siriraj Hospital
Diffuse parenchymal lung disease Idiopathic interstitial pneumonias
DPLD of known cause or association
Idiopathic pulmonary fibrosis
Granulomatous
Other forms of interstitial pneumonia e.g. LAM, PLCH, eosinophilic pneumonia, etc.
Interstitial pneumonia other than idiopathic pulmonary fibrosis
Desquamative interstitial pneumonia
Respiratory bronchiolitis Interstitial lung disease
Acute interstitial pneumonia
Cryptogenic organising pneumonia
Non-specific Interstitial pneumonia
Lymphocytic Interstitial pneumonia
ILD associated with systemic disease Rheumatogical Systemic sclerosis Rheumatoid arthritis Systemic lupus erythematosus SjÖgren’s syndrome Ankylosing spondylitis Polymyositis/dermatomyositis Mixed connective tissue disease Vasculitis Giant cell arteritis Takayasu’s arteritis Microscopic polyangiitis Wegener’s granulomatosis Churg-Strauss granulomatosis Behcet’s syndrome Pulmonary-renal syndrome (including Goodpasture’s syndrome) Capillaritis
ILD associated with systemic disease Vascular Antiphospholipid syndrome Coagulopathies Arteriovenous malformations Primary pulmonary hypertension Idiopathic pulmonary hemosiderosis Pulmonary veno-occlusive disease
Disease caused by environmental triggers or drug ingestion Inorganic causes Fibrogenic inorganic dusts Asbestos Silica Hard metal alloy Beryllium Coal Aluminium Non-fibrogenic Siderosis Stannosis Baritosis Antimony
Drug* Chemotherapeutic Cardiovascular Antibiotics Anti-inflammatory Illicit Psychotropic Radiation Pesticides Oxygen Environmental (organic causes) Extrinsic allergic alveolitis Fungal Bacterial Avian Chemical
Idiopathic interstitial pneumonias Idiopathic 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 ILD Histologic patterns Usual interstitial pneumonitis Nonspecific interstitial pneumonia Organizing pneumonia Diffuse alveolar damage Respiratory bronchiolitis
Clinical-RadiologicPathologic Dx Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis Nonspecific interstitial pneumonia (provisional) Cryptogenic organizing pneumonia (BOOP) Acute interstitial pneumonia Respiratory bronchiolitis interstitial lung disease Desquamative interstitial pneumonia
Desquamative interstitial pneumonia Lymphoid interstitial pneumonia Lymphoid interstitial pneumonia
Granulomatous diseases
Sarcoidosis Berylliosis Hypersensitivity pneumonitis or Extrinsic allergic alveolitis Langerhan’s cell histiocytosis Wegener’s granulomatosis Churg-Strauss syndrome Lymphomatoid granulomatosis Bronchocentric granulomatosis
Other diffuse lung diseases Inherited disorders Tuberous sclerosis Neurofibromatosis Hermansky-Pudlak syndrome Lipid storage disorders Familial fibrosing alveolitis Pulmonary eosinophilia Known causes, e.g. fungi, parasites, drugs Unknown causes, e.g. acute and chronic idiopathic eosinophilic pneumonia Lymphangioleiomyomatosis Alveolar proteinosis Alveolar microlithiasis Amyloidosis Chronic aspiration Chronic left heart failure
Clinical manifestation Progressive
dyspnea Non-productive cough Pleuritic chest pain (pneumothorax) Hemoptysis +/- Fe def anemia Wheezing Abnormal CXR Symptoms related to associated diseases e.g. CNT Restrictive pattern from spirometry
Physical examination Increased
RR Respiratory distress Crackles Inspiratory squeaks – late inspiratory high-pitched rhonchi; bronchiolitis Cyanosis, clubbing of fingers, cor pulmonale – advanced disease Extrapulmonary findings
Extrapulmonary findings Hypertension Skin: EN, maculopapular rash, heliotrope, telangiectasis, Raynaud’s, Café-au-lait spots, vasculitis, subcut nodules, albinism, calcinosis Eye: uveitis, scleritis, keratoconjunctivitis sicca Salivary gland enlargement Lymphadenopathy Hepatospenomegaly Pericarditis, myocarditis Muscle weakness
Duration of illness
Acute (days to weeks)
Subacute (weeks to months)
Acute idiopathic interstitial pneumonia (AIP, Hammon-Rich syndrome) Eosinophilic pneumonia Hypersensitivity pneumonia Bronchiolitis obliterans with organizing pneumonia (BOOP) or cryptogenic organizing pneumonia (COP) Sarcoidosis Drug-induced Alveolar hemorrhage syndromes Idiopathic BOOP CNT (SLE, polymyositis)
Chronic (months to years)
Idiopathic pulmonary fibrosis (IPF) Sarcoidosis Pulmonary Langerhans cell histiocytosis (PLCH) or eosinophilic granuloma (EG)
Occupation & environmental exposures Inhaled
organic dusts (hypersensitivity pneumonitis)
Thermophilic fungi; farmer’s lung, grain handler’s lung, humidifier or air conditioner lung Bacteria (Bacillus spp); humidifier lung True fungi (aspergillus, cryptostorma coricale, penicillium Animal proteins; bird fancier’s disease
Occupation & environmental exposures
Inhaled inorganic dust
Silicates
Carbon
Silica (silicosis) Asbestosis (asbestosis) Talc (talcosis) Kaolin or china clay Diatomaceous earth (Fuller’s earth) Nepheline (hard rock) Aluminum silicates (sericite, sillimanite, ziolite) Portland cement Mica Beryllium (berylliosis) Coal dust (pneumoconiosis) graphite
Metals
Tins (stannosis) Aluminum; powdered aluminum, bauxite Hard metal dusts: cadmium, titanium, tungsten, hafnium, niobium, cobalt, vanadium carbides Iron (siderosis, arc welder’s lung) Antimony Hematite (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 exposures
Inhaled other agents
Chemical sources
Gases
Oxides of zinc, copper, manganese, cadmium, iron, magnesium, nickel, brass, selenium, tin, antimony Diphenylmethane diisocyanate Trimellitic anhydride toxicity
Vapors
Oxygen Oxides of nitrogen sulfur dioxide Chlorine gases Methyl isocyanate
Fumes
Synthetic-fiber lung (nylon, acrylic, polyesters, orlon) Bakelite worker’s lung Vinyl chloride, polyvinyl chloride powder
Hydrocarbons Thermosetting resins (rubber tire workers) Toluene diisocyanate (TDI-asthmatic reactions prominent) Mercury
Aerosols
Oils Fats Pyrethrum (natural insecticide)
Laboratory data Leukopenia: sarcoidosis, CNT, lymphoma, drug-induced Leukocytosis: HP, lymphoma, vasculitis Eosinophilia: eosinophilic pneumonia, sarcoidosis,
vasculitis, drug-induced (sulfa, methotrexate)
Thrombocytopenia: sarcoidosis, CNT, drug-induced, Gaucher’s dis
Hemolytic anemia: CNT sarcoidosis, lymphoma, druginduced
Normocytic anemia: alv hemorrhage syndrome, CNT, lymphangitic carcinomatosis
Elevated LDH: alv proteinosis, IPF Hypogammaglobulinemia: LIP Hypergammaglobulinemia: CNT,sarcoidosis,
vasculitis, LIP, lymphoma, silicosis
Laboratory data
Serum immune complexes: IPF, LIP, vasculitis, CNT, EG
ACE: sarcoidosis, HP, silicosis, Gaucher’s disease Antibasement membrane Ab (anti-GBM):
Goodpasture’s syndrome
ANCA: Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis
Serum precipitating Ab: HP Lymphocyte transformation test to specific antigens: chronic beryllium disease, aluminum
potroom workers disease, gold-induced pneumonitis
IPF
DIP
RB-ILD
AIP
BOOP
NSIP
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, lymphangitic carcinomatosis, 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, thinwalled cysts
NSIP
Peripheral, subpleural
Predominant ground glass, basal reticular lines, consolidation
Acute onset ILD
Acute interstitial pneumonia
Diffuse alveolar hemorrhage (vasculitis or coagulopathy most common causes)
Acute pneumonia due to rheumatological disease
Drugs (selected)
Eosinophilic lung disease, acute or chronic
Cryptogenic and known causes of organizing pneumonia (BOOP)
Mimics − Pulmonary edema of any cause − Infection, especially opportunistic with Pneumocystis carinii − Acute respiratory distress syndrome
Diffuse lung disease with characteristic features on HRCT Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) Desquamative interstitial pneumonia
Extrinsic allergic alveolitis
Sarcoidosis
Langerhans cell histiocytosis
Lymphangioleiomyomatosis
Alveolar proteinosis
Lymphangitic carcinomatosis
Predominant distribution on HRCT Upper-lobe predominance
Sarcoidosis Coalworker’s pneumoconiosis Silicosis Eosinophilic pneumonia Langerhans cell histiocytosis Extrinsic allergic alveolitis
Lower-lobe predominance
Idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Asbestosis Collagen vascular disease
Peripheral predominance
Idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Asbestosis Collagen vascular disease Eosinophilic pneumonia Organising pneumonia
Central (peribronchovascular) predominance
Sarcoidosis Lymphangitis carcinomatosis
Pattern in HRCT Linear and reticular
Idiopathic pulmonary fibrosis Collagen vascular disease Asbestosis
Nodular or reticulonodular
Sarcoidosis Silicosis Lymphangitis carcinomatosis
Parenchymal opacification (consolidation/ground-glass)
Organising pneumonia Extrinsic allergic alveolitis Desquamative interstitial pneumonia Respiratory bronchiolitis/interstitial lung disease Chronic eosinophilic pneumonia Haemorrhage Alveolar proteinosis
Cystic abnormalities
Langerhans cell histiocytosis Lymphangioleiomyomatosis
BAL cell Neutrophilic (± eosinophilic)
Idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Collagen vascular disease Asbestosis Acute interstitial pneumonia
Lymphocytic
Sarcoidosis Extrinsic allergic alveolitis Silicosis Lymphocytic interstitial pneumonia
Eosinophilic
(Chronic) eosinophilic pneumonia Churg-Strauss syndrome Hypereosinophilic syndrome
Mixed cellularity
Bronchiolitis obliterans organizing pneumonia Collagen/vascular disease Non-specific interstitial pneumonia
Abnormal macrophage morphology
Extrinsic allergic alveolitis Alveolar proteinosis Respiratory bronchiolitis/interstitial lung disease Alveolar hemorrhage
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
Treat
No
If deteriorates
Observe (but monitor with serial pulmonary function tests)
Thank you & GOOD LUCK !!!!!
