DIFFUSE LUNG DISEASE
DIFFUSE LUNG DISEASE
Interstitial Lung Disease
It is the result of injury to structures in the alveolar space, interstitial space, or both
PATHOLOGY
Alveolar cell injury
Altered permeability in the alveolocapillary inter phase
Alveolar exudation
Infiltration of interstitial spaces by mononuclear cells, neutrophils and other cells
Increase in the thickness of the pulmonary interstitium
Capillary damage causes increased permeability and edema formation
Interstitial fibrosis
PHYSIOLOGY “Restrictive Defect”
Lung Volumes - decreased
TLC, VC, and RV decreased
Airflow rates - near normal
Lung compliance is decreased and elastic recoil is increased
DLCO is decreased early in the course
Exercise induced hypoxemia
Hypocarbia in early stages
Hypercarbia in late disease
(Respiratory Exhaustion)
ROENTGENOLOGY
HRCT Scan:
Shows characteristics findings
Pulmonary Langerhans Cells Granulloma:
Nodular cystic spaces in the upper lung field
Idiopathic Pulmonary Fibrosis:
Subpleural honey combing
Lymphangioleiomyomatosis
Well defined cystic spaces in the lung parenchyma
CXR:
Alveolar pattern
Interstitial pattern
combination of alveolar - interstitial patterns
CLASSIFICATION OF DIFFUSE
Pulmonary Diseases
IDIOPATHIC PULMONARY FIBROSIS
Cryptogenic Fibrosing Alveolitis
Rare Disorder
Diffuse Fibrosis throughout the Lung fields
Late middle age
Most common cause of chronic diffuse interstial Lung disease
Some studies indicate that Hepatitis C virus infection is an etiologic factor
PATHOGENESIS
Complex
Macrophages activated by several factors
Production of growth factors e.g. Fibronectin, platelet-derived growth factor (PDGF), TGT-B, IGF-I
Deposition of collagens type I and III
CLINICAL FEATURES
Progressive Breathlessness
Frequent Coughing
Fatigue
Anorexia
Weight loss
Arthralgias
PHYSICAL FINDINGS
Cyanosis
Tachypnea at rest
Clubbing of the fingers and toes
(without hypertrophic osteoarthropathy)
Dry crackles, or coarse crackles on inspiration (Auscultation), heard at the Lung bases
Accentuated pulmonic second sound
Signs of RHF
IMAGING
CXR- Diffuse reticular or reticulonodular markings (lower Lung zones)
High Resolution CT- “Ground Glass” appearance of the lower Lung fields (stage of alveolitis)
Nodular Infiltrates
Honey Combed or Swiss Cheese appearance (end stage disease)
Iinear opacities
Ring shaped opacities
Lung field contracted
LABORATORY EXAMINATION
ESR elevated
Circulating immune - complex titers 50%
Serum immunoglobins
Cryoimmunoglobulins
RF (Rhematoid factors) 30%
ANA (Antinuclear antibody) 35%
LUNG FUNCTION TESTS
Advanced Disease
TLC
VC
RV
FEV1/FVC Ratio-Normal or increased
DLCO - Reduced by 30% - 50%
Bronchoscopy
Sarcoidosis - Yield 80%
BAL Useful information about cells+proteins
LUNG BIOPSY
Histologic Evaluation
Good Microbial Cultures
Immunofluorescence and eleetnn-microscope studies
Analysis of inorganic substances
DIAGNOSTIC APPROACH
Occupational and environmental history
Prior chest films review
check for multi system disease process, new medication, neurologic status (aspiration and infection)
Chest X-ray / HRCT chest scan
Pulmonary function test / DLCO
Arterial Blood gases (desatuiration with exercise)
Fiberoptic bronchoscopy (1st invasive procedure)
Transbronchial biopsy / BAL
Imaged thorascopic or open lung biopsy
TREATMENT
Oral prednisone 1mg / kg / d * 8 week maintenance level 0.25 mg / kg / day * 6 months
Immosuppression with cyclophosphamide if disease is progressive (Addition) dose 1mg / kg / day
Pulsed doses of cyclophosphamide given biweekly
Azathioprine
Colchicine 0.6 mg / day (inhibit macrophage produced fibroblast growth factors)
Discontinue cigarette smoking
Supplemental oxygen therapy
Diureties
Bronchodilator’s
Narcotic containing antitussive medication
Management of infection during immno suppression
Prophylactic use of pneumococcal and influenza vaccines
Lung transplantation
IDIVIDUAL FORMS OF ILD
ILD associated with collagen vascular disorders
Systemic Lupus Erythematosus
Half of the patients with SLE ultimately develop overt lung disease
Pleuritis, pleural effusion, Acute pneumouitis (most frequent)
Chromic progressive ILD (uncommon)
abnormal DLCO
Lymphocytic alveolitis (better response to treatment)
End
RHEUMATOID ARTHRITS
Pleural disease (effusion and subpleural noduls)
Parenchymal nodular infiltrates
Diffuse interstitial fibrosis
ILD can develop before joint disease becomes evident (in men)
High titers of rheumatoid factor
Broncholitis obliterans with organizing puemonia have been reported
Patient who receives methotrexate or gold must be differentiated
Penicillamine therapy broncholitis obliterans
ANKYLOSING SPONDYLITIS
Bilateral upper lobe fibrosis
Fibrocavitary disease, may develop late in the course
SYSTEMIC SCLEROSIS
Radiographic evidence of lung involvement
Cutaneous scleroderma can involve the anterior chest wall and abdomen restrictive lung disease
SJOGREN’S SYNDROME
SYNDROMES OF ILD WITH PULMONARY HEMORRIAGE
Recurrent hemoptysis, dysprea and hypoxemia with diffuse alveolar opacities on chest radiography
SLE, wegener’s granulomatosis, behcet’s disease, allergic churg- strauss granulomatosis, Henoch- schonlein purpura, mixed cryoglobinemia
Serologic test ANA, Anti GBM antibody and complement
Renal biopsy and lung biopsy may be repaired for definite diagnosis
e-g
GOOD PASTUR’S SYNDROME
Pulmonary hemorrhage and glumerulonephritis are the features
Anti bodies to renal glomeraular and lung alveolar basement membranes
IDIOPATHIC PULMONARY HEMOSIDEROSIS
Diagnosis of exclusion
Lung biopsy is necessary (rule out) inflammatory injury)
Children and young adults are usually affected
Clinical course fulminant mild
Glucocorticoid treatment to control bleeding acutely
PULMONARY ALVEOLAR PROTEINOSIS
Alveoli are filed with grammar material that stains with periodic acid schiff reagent (PAS)
(Primary VS secondary)
secondary can be associated with inhaled dust exposure (silica and aluminium) malignancy, and chronic infection
intraalveolar material surfactant phospholid, LDH, proteins and Igs
whole lung lavage provides long term benefits
LYMPHOCYTIC INFILTRATIVE DISORDERS
Behave as low grade lymphoma
diffuse interstitial infiltration with lymphocytes and plasma cells
Autoimmune disease or dysproteinemia exists
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