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MED - Interstitial Lung Disease, Final Sept08
MED - Interstitial Lung Disease, Final Sept08
MED - Interstitial Lung Disease, Final Sept08
Occupational/Environmental
Lung Diseases
Definition
Pathogenesis
Classification
Clinicalmanifestations
Natural history of disease
Diagnosis
Treatment
Prognosis
Objectives:
A. Septum
B. Pulmonary
A
C. Alveolar
duct
D. Pleura
E. Alveolar
sac
F. Pulmonary
v
Interstitial Lung Disease
Harrison’s 05
What causes it?
Pathogenesis
Multipleinitiating events
Precise pathway from injury to fibrosis not
known
Postulated common pathway
– Acute injury to the lung parenchyma
– Chronic interstitial inflammation?
– Fibroblast activation and proliferation
– Pulmonary fibrosis and tissue destruction
Pathogenesis
Air spaces
Antigenic Alveolar walls
Stimulation
Interstitium
Acute
Vascular
Lymphatic
Injury
Inflammation
Granuloma
Fibrosis
Classification
(Clinical and Histological)
Major Categories of Alveolar and Interstitial Inflammatory Lung Disease
Lung Response: Alveolitis, Interstitial Inflammation, and Fibrosis
KNOWN CAUSE
Asbestos Radiation
Drugs (Antibiotics, amiodarone, gold) and chemotherapy drugs Residual of adult respiratory distress syndrome
UNKNOWN CAUSE
Idiopathic interstitial pneumonias Pulmonary alveolar proteinosis
Idiopathic pulmonary fibrosis ( usual interstitial pneumonia) Lymphocytic infiltrative disorders (lymphocytic interstitial pneumonitis assoc. with
connective tissue diasese
Cryptogenic organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) Inherited Diseases
Nonspecific interstitial pneumonia Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher’s Disease,
Hermansky-Pudlak syndrome
Connective Tissue Diseases Gastrointestinal or liver diseases (Crohn’s disease, primary biliary cirrhosis, chronic active
hepatitis, ulcerative colitis)
Syrematic lupus erythematous, rheumatoid arthritis , ankylosing spondylitis systemic Graft-vs-host disease (bone marrow transplantation; solid organ transplantataion)
sclerosis, Sjogren’s syndrome, polymyositis-dermatomyositis
UNKNOWN CAUSE
Granulomatous vasculitides
Granulomatous
Known
– Primary disease
– Occupational / Environment
– Drugs / Poisons / Infections
Unknown
Fibrosis
Known
– Primary disease
– Occupation / Environmental
– Drugs / Poisons / infections
Unknown
What symptoms will I experience?
Progressive breathlessness
Persistent non productive cough
Abnormal radiograph
Pulmonary symptoms associated with
another disease
Abnormality on simple spirometry
SYMPTOMS
Dyspnea
Cough
– Fatigue
– Weight loss
– Chest pain
– Hemoptysis
– Wheezing
Clinical Manifestations
Acute
Subacute
Chronic
Symptoms
20-40 years
– Sarcoidosis
– ILD with CTD
– Lymphangioleiomyomatosis (LAM)
– PLCH
– Inherited forms of ILD
Older than 50 years
– Idiopathic Pulmonary Fibrosis (IPF)
Gender
Premenopausal women
LAM, tuberous sclerosis
Female preponderance
Lymphocytic interstitial pneumonitis
ILD in Hermansky-Pudlak syndrome
Not specific
Usual
– Tachypnea
– Bibasilar end inspiratory crackles
– Late inspiratory high pitched rhonchi
Wheezing (uncommon)
Late
– Cyanosis and clubbing
– pulmonary hypertension
– Cor pulmonale
Physical exam
Chest radiograph
– Bibasilar reticular pattern
– Nodular or mixed pattern of alveolar filling
– Honeycombing
Late finding
Poor prognosis
Clinical correlation is poor
Other conditions may mimic ILD
– Congestive Heart Failure
– Atypical pneumonia
– Lymphangitic spread of cancer
May be normal in 10% of patients
Chest Imaging Studies
CT Scan (HRCT)
– More sensitive and superior for early detection
– Better assessment of extent and distribution
– Better in evaluating possible co-existing disease
– Can be helpful in determining the most
appropriate site for biopsy
– Patterns usually follow same findings on chest
xray / disease
Pulmonary Function Test
Bronchoscopy
– Bronchio-alveolar lavage
– Transbronchial biopsy
Lung biopsy
Video Assisted Thoracoscopic Surgery
(VATS)
– Confirms diagnosis
– Assess activity of the disease
– Helps in determining prognosis
Important histologic patterns
Usual Interstitial Pneumonia (UIP)
Non specific Interstitial Pneumonia
Respiratory bronchiolitis
Bronchiolitis Obliterans with Organizing
Pneumonia (BOOP)
Desquamative Interstitial Pneumonia
Lymphocytic Interstitial Pneumonia
Pattern of diffuse alveolar damage
Histologic features affecting prognosis
Degree of cellularity
– Abundant inflammatory cells(early phase)
– Less cells, abundant fibrosis (late phase)
Pattern or distribution of cellular reaction
– Collection of cells in alveoli (early alveolitis)
Predominant type of inflammatory or effector
cell
– Many lymphocytes, eosinophils and PMN’s
(better response to corticosteroid therapy)
Algorhythm
Is there a treatment for it?
Principles of treatment
Major goals
– Permanent removal of offending agent
Glucocorticoids
– Mainstay of therapy
– Success rate low
– No direct evidence that it improves survival
Dose
– 0.5-1 mg/kg
– 0.25-0.5 mg/kg
Length of treatment
– 4-12 weeks -> re evaluated -> tapered
Treatment
Cyclophosphamide
Azathioprin
Methotrexate
Colchicine
Penicillamine
Cyclosporine
Treatment
Other medical
Manage cough and hyper reactive airways
Supplemental oxygen, Phlebotomy
Diuretics and drugs for pulmonary hypertension
Early control of infections and immunizations
Lung transplantation
Non medical
– Smoking cessation
– Regular exercise
– Eat well
– Pulmonary rehabilitation program
Ancillary measures and care
Patient education
Nutritional instructions
Psychological support
Rehabilitation and body conditioning
Smoking cessation
Complications
Most important
Detailed occupational history
– Potential exposure in the workplace
Specific contaminants involved
– Availability of personal respiratory protection
device
– Specific contaminant
Ventilation in the workplace
Size of particles
MEASUREMENT OF EXPOSURES
Inorganic dust
– Asbestosis
– Silica
– Coal
– Beryllium
– Other metals
Organic dust
– Cotton dust
– Grain dust
– Agricultural dust
– Other environmental agents
Asbestos
Clinical manifestations
Pleural disease: pleural plaques, benign pleural
effusions, pleural fibrosis and malignant mesothelioma
Asbestosis
Asbestos
Industries
– Constructions and shipbuilding
Occupations
– Plumbing
– Pipefitting
– insulating
Bystander exposure
Asbestos
Asbestosis
– Diffuse interstitial fibrosing disease (pulmonary fibrosis) of
the lung directly related to intensity and duration of exposure
Lung Cancer
– Squamous cell or adenocarcinoma
– Higher risk among smokers
– Peaks 15-19 yrs after exposure
Mesotheliomas
– Pleural or peritoneal
– Not associated with smoking
– Peaks 30-35 yrs after exposure
Asbestosis
DIAGNOSIS
– History
Progressive dyspnea, cough, chest pain
– PE
Inspiratory crackles, digital clubbing
– CXR and HRCT
Fibrotic changes-lower lobes and subpleural areas
– PFT
Restrictive
Treatment
– Supportive
Silica or Crystalline quartz
Clinical manifestations
– Silicosis (Progressive pulmonary fibrosis with exposure and
occurs in a dose-response fashion after many years of
exposure)
– Auto immune connective tissue disorder
– RA, SLE and scleroderma
Silicotuberculosis (3x)
COPD and Chronic bronchitis
Lung cancer
Silicosis
Fibronodular parenchymal disease (silicotic nodules)
Frequently without symptoms
May have acute or accelerated forms which may
lead to respiratory failure
Chest radiograph
– Small rounded opacities in the upper lobes
– Reticular or irregular densities
– Hilar adenopathy
Calcification of hilar nodes
“Egg shell pattern
Coal dust
Coal mining
– Coal dust; 50% of anthracite miners
– Develop coal macules and focal emphysema
Coal worker’s Pneumoconiosis
– pneumoconiosis with progressive massive fibrosis
& seropositive rheumatoid arthritis
Caplan’s syndrome
Coal Dust
Chest radiograph
– early = reticular: small irregular opacity
– late = nodular: rounded regular opacity
1-5mm
– nodules > 1 cm upper lung in
complicated CWP
– Calcifications are generally not seen
Beryllium
Berylliosis
– Acute pneumonitis or chronic interstitial
pneumonitis
– Exposure: alloys, ceramics, high-tech electronics,
fluorescent lights production
– Biopsy: granulomatous formation
Inorganic Dust
James Froude