Intrestitial lung disease 9 5-2016
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Transcript of Intrestitial lung disease 9 5-2016
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Interstitial Lung Disease &Pulmonary Infection
Dr. Vinita Singh
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CASE-1
A routine chest X- ray performed on an asymptomatic adult man who works at sandblasting reveals a fine nodularity in the upper zone of the lung and “eggshell” calcification of the hilar lymph nodes.
The Pt. S.calcium level is 9.8m/dl total protein is7.2g/dl.
He denies any H/O drug use or ciggarette smoking.
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Cond,
A biopsy from lung reveal birefringent particles within macrophages and fibrosis of lung. what is the material ?
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CASE -2
A 65yr old man who just retired after having worked for many years as a shipyard worker presents with increasing shortness of breath. Pertinent medical history is that he has been a long time smoker.
A CT Scan of his chest reveals thick , pleural Plaques on the surface of his lungs.
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Cond,
The Dumbbell – Shaped structures were found to stain blue with a prussian blue stain . What are these structures ?
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IMPORTANT CAUSES OF ILD
1. Primary or idiopathic• Idiopathic Pulmonary Fibrosis• Nonspecific interstitial pneumonia (NSIP)• Organising pneumonia (OP)• Respiratory bronchiolitis (RB)• Diffuse alveolar damage (DAD)• Desquamative interstitial pneumonia (DIP)• Lymphocytic interstitial pneumonia (LIP)
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Secondary Causes1.Infectious• Tuberculosis• Bacterial• Fungal• Parasitic• Viral2. Noninfectious• Hypersensitivity pneumonitis• Pneumoconiosis• Drug induced• Radiation induced• Malignancies
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Association with diseases of unknown aetiology
• Sarcoidosis• Connective tissue disorders• Systemic sclerosis• Rheumatoid arthritis• Dermatomyositis,Polymyositis• SLE• Chronic eosinophilic pneumonia
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ILD is a heterogeneous syndrome with the following common clinical features:
1. Exertional dyspnea
2. Bilateral diffuse infiltrates on chest radiographs
3. Physiological abnormalities with a restrictive lung defect,
decreased diffusing capacity (DLco) and abnormal alveolar-
arterial oxygen gradient (PAO2 – PaO2) at rest or with exertion.
4. Absence of pulmonary infection and neoplasm.
5. Histopathology with varing degrees of fibrosis and inflammation
with or without evidence of granulomatous or secondary
vascular changes in the pulmonary parenchyma.
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Diffuse Parenchymal Lung Disease (DPLD)
DPLD of known cause, eg, drugs or association, eg,
collagen vascular disease
Idiopathic interstitial
pneumonias
Granulomatous DPLD, eg, sarcoidosis
Other forms of DPLD, eg, LAM,
HX, etc
Idiopathic pulmonary
fibrosisIIP other than idiopathic
pulmonary fibrosis
Desquamative interstitial pneumonia
Acute interstitial pneumonia
Nonspecific interstitial pneumonia (provisional)
Respiratory bronchiolitis interstitial lung disease
Cryptogenic organizing pneumonia
Lymphocytic interstitial pneumonia
ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.
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PATHOLOGY
• Pulmonary interstitium is the anatomical space between the alveolar and the capillary basement membranes.
• Contains mesenchymal and connective tissue cells and extra cellular matrix composed of collagen, elastin and proteoglycans.
• Involvement of interstitium+ adjoining alveolar epithelial+ Capillary endothelial cells.
• Disease encroaches alveolar spaces involving acini, terminal bronchioles and overlying pleura.
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Inhaled environmental agents(fumes, dust, smoke)
Alveolar epithelial cell injury
Wound healing (inflammation, coagulation, epithelial/endothelial
repair)
Pulmonary fibrosis
Normal Chronic airflow
obstruction
Genetic predisposition
Delivery &
persistence
Biochemical
Immunologic Fibrotic
Four proposed mechanisms and potential variations in lungresponses to inhaled agents
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Recent Hypothesis:• Inflammatory hypothesis• Epithelial Cell Apoptosis• Angiogenesis• Abnormal Matrix Turnover• Th1 versus Th2 Cytokines• Growth Factor Production• Altered Fibroblast Phenotypes• Myofibroblast Recruitment and Maintenance
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Thannickal VJ, et al. Annu Rev Med. 2004;55:395-417.
AGEGENETIC FACTORS
ENVIRONMENTAL FACTORSNATURE OF INJURY
– Etiologic agent– Recurrent vs single
– Endothelial vs epithelial
Histopathologic Pattern
DIP RB-ILD LIP COP NSIP AIP UIPInflammation Fibrosis
LUNG INJURY
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PATTERNS ON CHEST X-RAY
LINEAR
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reticular
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NODULAR
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RETICULONODULAR
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Normal Lung- cut surface and pleura smooth and homogenousIPF- cut surface demonstrates patchy involvement of lung with fibrous scarring around dilated airspaces forming a honey comb pattern
IPF
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CASE-3
A 24yr old African American woman presents with nonspecific symptoms including fever malaise. A chest X –ray reveals enlarged hilar lymph nodes ( potato nodes) . serum calcium level is found to be elevated What is the probable diagnosis ?
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CASE -4
A 61yr old man presents with increasing shortness of breath .
A chest X- ray reveals diff. Pulmonary infiltrate. transbronchial biopsy reveals fibrosis of the walls of the alveoli, many of which contain sheets of “ desquamated’’ cells.
What is the probable diagnosis ?
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CASE -5 A 37yr old woman presents with acute onset of a productive cough, fever, chills and pleuritic chest pain.
A chest X –ray reveals consolidation of the entire lower lobe of her right lung . Histologic examination of lung tissue reveals multiple suppurative , neutrophil – rich exudates filing bronchi , bronchioles and alveolar spaces.
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Cont’
1. What is the probable diagnosis? 2. What is the etio – pathogenesis ?
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Etiology• Decreased resistance - General/immune• Virulent infection - Lobar pneumonia• Defense Mechanisms
In the normal respiratory system there are a number of important defense mechanisms that protect the lung from infection. These include:– Reflex closure of the vocal cords – Cough reflex – Mucociliary clearance – Macrophage activity and immune competence.
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• An increased risk of bacterial infection is associated with impairment of the defense mechanism, as in any of these clinical situations:– Loss of consciousness – Immunodeficiency state – Pulmonary edema – Neutropenia – Chronic airway obstruction– Viral infection.
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• ExudateThe exudate in bacterial pneumonia is typically composed of varying proportions of:– edema fluid – red blood cells – leukocytes (principally neutrophils) – fibrin
• The cellular exudate in acute bacterial pneumonia is in the alveolar spaces and distal bronchioles though in severe cases the major airways may also be filled with purulent secretion.
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Types• Viral• Bacterial• Mycoplasmal• Fungal
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The Pneumonia Syndromes• Community-acquired
acute pneumonia– Streptococcus
pneumonia– Haemophilus influenza– Moraxella catarrhalis– Staphylococcus aureus– Legionella
pneumophilia– Klebsiella– Pseudomonas
• Community-acquired atypical pneumonia– Mycoplasma– Chlamydia– Legionella – Viruses (RSV,
parainfluenza & influenza, adenovirus)
• Nosocomial pneumonia– Gram negative rods– Staphlyococcus aureus
• Aspiration pneumonia– Anaerobic oral flora– Amniotic fluid– Gastric content– Chemicals
• Chronic pneumonia– Nocardia– Actinomyces– Granulomatous
• Necrotizing pneumonia– Anaerobic– Staphlyococcus aureus– Klebsiella– Streptococcus pyogens
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Several possible routes of infection of the lung exist:– Aspiration of contaminated secretions--
most common – Inhalation of infected airborne droplets – Bacteremia – Direct extension of an acute
inflammatory process from an adjacent organ or structure.
Routes of Infection
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Etiopathogenesis• Causes of bacterial pneumonia can be categorized
as extrinsic and intrinsic. • Extrinsic factors : infection with respiratory
pathogens. Exposure to pulmonary irritants or direct pulmonary injury causes noninfectious pneumonitis.– Infectious agents responsible for bacterial
pneumonias include S. pneumoniae and H. influenzae; Klebsiella, Staphylococcus, and Legionella species; and gram-negative organisms.
– Aspiration and inhalation of aerosols containing the bacterial pathogen are the most common modes of infection.
– Some bacteria, such as Staphylococcus species, may spread to the lungs hematogenously.
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• S. pneumoniae is the most common cause of bacterial pneumonia.
• Pneumonia from H influenzae often is associated with debilitating conditions such as asthma, COPD, smoking, and a compromised immune system.
• K. pneumoniae may cause a severe necrotizing lobar pneumonia in patients with chronic alcoholism, diabetes, or COPD.
• S. aureus pneumonia is observed in those who abuse intravenous drugs. – S. aureus generally occurs in hospitalized patients
and patients with prosthetic devices; it spreads hematogenously to the lungs from contaminated local sites. This pathogen also is an important cause of pneumonia following infection with influenza A.
• L. pneumophila infections occur either sporadically or as local outbreaks.
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• Gram-negative pneumonias are observed in individuals who are immunocompromised or hospitalized. –Causative organisms include
Escherichia coli and Pseudomonas, Enterobacter, and Serratia species. Residents of chronic care facilities are at risk for gram-negative pneumonia.
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• Intrinsic factors : related to the host's immune response, the presence of comorbidities, and other risk factors:– Loss of protective reflexes allows
aspiration of oropharyngeal flora into the lung.
• Aspiration is facilitated by altered mental status from intoxication, deranged metabolic states, neurological causes (eg, stroke), and endotracheal intubation.
– Local lung pathologies (eg, tumors, chronic obstructive pulmonary disease [COPD], bronchiectasis).
– Smoking impairs the host's defense to infection by a variety of mechanisms.
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• Aspiration pneumonia is observed in individuals with altered sensorium (eg, seizures, alcohol intoxication, drug intoxication) or CNS impairment (eg, stroke). – The stomach or oropharyngeal contents
are aspirated.
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Complications of Pneumonia• Destruction of lung tissue from infection
(leading to bronchiectasis)• Organization of the exudate• Abscess formation • Spread of the infection to the pleural
cavity (empyema) • Sepsis & Pyemia• Respiratory failure• Acute respiratory distress syndrome• Superinfection with gram-negative
organisms• Death
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1. Pneumonia
•1.1. Bronchopneumonia
•1.2. Lobar pneumonia
•1.3. Viral (Atypical) pneumonia
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Bronchopneumonia
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Bronchopneumonia
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1.2. Lobar Pneumonia• Fibrinosuppurative consolidation – whole
lobe• Rare (due to antibiotic treatment)• ~95% - Strep. pneumoniae types 1,3,7&
2• Four stages (Laennec,1838) :
– Congestion & edema (1 to 2 days)– Red Hepatization (2-4 days )– Gray Hepatization (4 to 8 days)– Resolution (1 to 3 weeks).
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• Congestion & Edema: This stage is characterized
histologically by:– vascular engorgement, – intra-alveolar fluid, – small numbers of neutrophils, – often numerous bacteria. – Grossly, the lung is heavy and
hyperemic.
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• Red hepatization: – Vascular congestion persists, – Extravasation of red cells into
alveolar spaces,– Increased numbers of neutrophils
and fibrin. – The filling of airspaces by the
exudate leads to a gross appearance of solidification, or consolidation, of the alveolar parenchyma.
– A dry, granular, dark-red lung surface on gross appearance• This appearance has been likened
to that of the liver, hence the term "hepatization".
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• Gray hepatization: –As pneumonia progresses over 2-3
days, erythrocytes are lysed with persistence of the neutrophils and fibrin and, epithelial cells degenerate
–The alveoli still appear consolidated, but grossly the color is paler and the cut surface is drier.
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• Resolution: – The exudate is digested by
enzymatic activity, and cleared by macrophages or by cough mechanism.
– Dying pneumococci release a preformed toxin, further contributing to this damage.
– The pneumococci are opsonized by leukocytes and begin to be cleared.
– Resolution results in the formation of jellylike yellowish-colored exudates.
– Absorption of these exudates is remarkably efficient, with little organization or permanent scaring.
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Lobar Pneumonia
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Lobar Pneumonia – Gray hepatization