Respiratory System Disorders 4
Lecture 26
Pathology and Clinical
Science 1 (BIOC211)
Department of BioscienceText Reference:
Porth’s Pathophysiology: Concepts of Altered Health States
Sheila C. Grossman & Carol Mattson Porth.
Ninth Edition.
Copyright © 2014 Lippincott, Williams & Wilkins Publishers, Inc.
© endeavour.edu.au
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SESSION LEARNING
OUTCOMES o This session explains the aetiology, pathophysiology,
clinical features, investigations and management of
diseases of the respiratory system.
o It aims to understand the following respiratory infections
• Lung cancers
• Interstitial pulmonary disease
• Sarcoidosis
• Lung diseases caused by organic and inorganic dusts
• Alveolitis
• Vascular diseases
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TUMOURS OF THE BRONCHUS
AND LUNG
Introduction
• Lung cancer is the most common cancer
worldwide (1.2 million new cases in 2000 & 18%
of cancer death)
• Tobacco use is the major preventable cause
• Majority of tumours in the lung are primary
bronchial carcinoma
• The lung is also a common site of secondary
metastatic carcinoma
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PRIMARY TUMOURS
OF THE LUNGAetiology
• Cigarette smoking most common and though to be responsible for 90% of lung carcinomas
• Risk proportional to amount smoked and tar content
• Passive smoking though to be a factor in 5% of lung cancers though difficult to quantify
• Other risk factors – exposure to naturally occurring radon, atmospheric pollution and occupation dealing with some industrial products e.g. asbestos, cadmium
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BRONCHIAL CARCINOMA
Pathophysiology
o Arise from either bronchial epithelium or mucous gland
o Common cell types are squamous 35%,
adenocarcinoma 30%, small cell and large cell
o Onset of symptoms is dependent on cell types &
position
within the bronchus
o Can directly involve the pleura or by lymphatic spread
o Blood-borne metastasis occur mainly to liver, bone,
brain, adrenals and skin
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SMALL CELL CARCINOMA
http://medicalassessmentonline.com/john/CancerLungSmallCellcancer2014.jpg
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BRONCHIAL CARCINOMA
Clinical features
• Cough (dry or with sputum)
• Haemoptysis
• Bronchial obstruction
– complete or partial
– with or without infection
• Breathlessness
• Pleural pain
• Symptoms due to blood-borne metastasis
• Non-metastatic extrapulmonary manifestations
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BRONCHIAL CARCINOMA
Investigations
• Chest X ray
• CT
• Bronchoscopy and biopsy
Management
• Surgical resection
• Radiotherapy
• Chemotherapy
• Palliative
Prognosis
• Very poor (5 year survival less than 6%)
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BRONCHIAL CARCINOMA
radiology.med.sc.edu/BronchialCA.htm
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BRONCHOGENIC
CARCINOMA
http://www.meddean.luc.edu/lumen/MedEd/medicine/pulmonar/images/path4/sld37.jpg
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SECONDARY TUMOURS
OF THE LUNGo Blood-borne metastatic pulmonary
deposits from breast, kidney, uterus, ovary,
testes and thyroid
o Deposits are usually multiple and bilateral
o There are respiratory symptoms e. g.
breathlessness
o Diagnosis is made by radiological
examination
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INTERSTITIAL & INFILTRATIVE
PULMONARY DISEASESDiffuse parenchymal lung disease (DPLDs)
• Heterogeneous group of conditions associated with diffused thickening of the alveolar walls with inflammatory cells and exudates
• Include
–Acute respiratory distress syndrome
–Granulomas ( e.g. sarcoidosis )
–Alveolar haemorrhage and fibrosis
• Lung disease alone or part of systemic connective tissue disorder
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SARCOIDOSIS o Multisystem granulomatous disorder
o More commonly seen in colder parts of Northern Europe
Aetiology – uncertain
Pathology
• Granulomas mostly in mediastinal and superficial lymph nodes, lungs, liver, spleen, skin, eyes, parotid glands and phalangeal bones
Clinical features
• Asymptomatic
• Lofgren’s syndrome in young women
• Respiratory symptoms
• Skin lesions
• Lymphadenopathy
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From Davidson’s Principles & Practice of Medicine (22st ed., p. 714) by B.R. Walker,
N.R. Colledge, S. H Ralston & I.D. Penman. 2014. Edinburgh. Churchill, Livingstone
Elsevier.
Sarcoidosis
A multisystem
disease
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SARCOIDOSIS
Investigation
• Blood tests, liver function tests
• Chest X ray
• Bronchoscopy and biopsy
Management
• Majority – spontaneous remission
• NSAIDs and corticosteroids
Prognosis
• Overall mortality low 1-5%
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Sarcoidosis
http://nikon2.magnet.fsu.edu/galleries/pathology/images/sarcoidosis/sarcoidosis20x02large.jpg
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LUNG DISEASES DUE TO
ORGANIC DUSTS
o Disease results from a local immune response to animal proteins or fungal antigens in mouldyvegetable matter
o Some examples
• Farmer’s lung (mouldy hay, straw, grain)
• Bird fancier’s lung (avian excreta, feathers)
• Malt worker’s lung
• Cheese worker’s lung (mouldy cheese)
o Most commonly presented as hypersensitivity pneumonitis
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HYPERSENSITIVITY PNEUMONITIS
(EXTRINSIC ALLERGIC ALVEOLITIS)
Pathogenesis
• Inhalation of certain types of organic dusts → diffuse immune complex reactions (Type III & type IV) in the walls of alveoli and bronchioles
• Chronic forms may lead to fibrosis
Clinical features
• History of exposure
• Flu-like symptoms – headache, myalgia, malaise, fever, dry cough, breathlessness
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HYPERSENSITIVITY PNEUMONITIS
(EXTRINSIC ALLERGIC ALVEOLITIS)
Investigations
• Chest X ray
• HRCT
• Pulmonary function tests
• Blood test – presence of antibodies to offending antigen
Management
• Removal of antigen or dust masks with appropriate filters
• Steroids
• Oxygen therapy in severely hypoxic patients
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ALVEOLITIS
http://www.humpath.com/IMG/jpg/acute_alveolitis_acute_pneumonia_a.jpg
Alveolitis as seen in acute bacterial pneumonia
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LUNG DISEASES DUE TO
INORGANIC DUSTSo Specific pathological changes in the lungs due to
exposure to dusts, fumes or noxious substances
o Generally prolonged exposure to inorganic dusts leads to diffuse pulmonary fibrosis (pneumoconiosis)
o Damage is from the inflammatory and fibrotic response to the dust
o Generally a long period of exposure is required
o Exposure can lead to other lung diseases
o Need a detailed occupational case history
o Diagnose by history, radiological and pulmonary function abnormalities
o No specific treatment is available for this group of diseases
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LUNG DISEASES DUE TO
INORGANIC DUSTSCoal Workers Pneumoconiosis - Inhalation of coal dust
o Simple - does not progress after exposure is stopped
o Progressive - massive Fibrosis
• Disease is disabling, can shorten life expectancy and can progress even after exposure is stopped
• Associated with chronic bronchitis, cough and sputum
Silicosis - Inhalation of silica dust
o Clinical features similar to coal workers pneumoconiosis
Asbestosis - Inhalation & exposure to asbestos dust
o Cause laryngeal carcinoma plus pleural and lung pathologies
o Decreases lung volumes and restricts ventilation of the lungs
o Usually progresses very slowly
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COAL MINERS PNEUMOCONIOSIS
Stevens & Lowe page 209
http://img.medscapestatic.com/pi/meds/ckb/09/38609tn.jpg
http://img.wikinut.com/img/3kh89at852q2nsi_/jpeg/180x300/Coal-Miners.jpeg
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SILICOSIS
https://o.quizlet.com/5bhRnQLyjE6kdorTKl9xFA_m.png
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ASBESTOS BODIES
Stevens & Lowe page 210
http://2.bp.blogspot.com/-
_3zIIyiJjCU/TtpbfbPvLRI/AAAAAAAAAMc/KqLCZic6r1s/s1600/asbes.jpg
http://upload.wikimedia.org/wikipedia/commons/7/71/Lung_asbestos_bodies.jpg
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From Essential Pathology. (3rd ed., p. 330) by Rubin E.. 2001. Philadelphia. Lippincott,
Williams & Wilkins
INORGANIC
DISEASE
COMPARISON
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PULMONARY VASCULAR
DISEASE
Venous thromboembolism (VTE)
• Majority ( 75% )of pulmonary emboli arise from lower limb DVT
• Risk factors – surgery, pregnancy, cardiopulmonary disease, lower limb problem, malignant disease
• Pathophysiology
– Based on size of the embolus
– Acute massive, acute small or medium, chronic multiple micro emboli
– Causes blockage or blood vessels in the lungs preventing the effective exchange of gases
– Leads to hypoxia and hypercapnia
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VENOUS THROMBOEMBOLISM
(VTE)
Clinical features
• Depend on size of embolism and underlying
disease
• Range from small emboli with few or no
haemodynamic consequences to
cardiovascular collapse
• Usually chest pain, dyspnoea, haemoptysis,
fainting
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VENOUS THROMBOEMBOLISMInvestigations
• Chest X-ray
• ECG
• Arterial blood gas analysis
• Ventilation perfusion scanning
• Echocardiography
• Pulmonary angiograph
Management
• General measure – pain relief, oxygen, resuscitation
• Anticoagulants
• Thrombolytic therapy
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PULMONARY
EMBOLISM
From Porth’s Pathophysiology: Concepts of Altered Health
States. (9th ed., p. 984), by
Sheila C. Grossman & Carol Mattson Porth.
Philadelphia, U.S.A. Walters Kluwer Health - Lippincott,
Williams & Wilkins
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DISEASES OF THE PLEURA
Pleurisy
o Term for any disease that involves the
pleura and leads to pleural pain or friction
• Common in pulmonary infarction, TB or
Tumour
• Clinical features are dependent on the
nature of the disease causing the pain
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EMPYEMA
o The presence of pus in the pleural space (Thin like serous fluid or Incredibly thick)
o Generally unilateral – involving the whole or part of a lung
o Always secondary - generally to infection of the lung
o Bacterial pneumonia and TB
Pathophysiology
o The pleura becomes covered with a thick shaggy inflammatory exudate
o The pus is generally under pressure and can erupt into a bronchus if not treated
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EMPYEMA
Clinical Features
o Persistent recurrent pyrexia in a patient already diagnosed with lung infection particularly if antibiotic treatment is being given
Treatment and Management
o Dependent of the underlying cause
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EMPYEMA
http://healthmediconline.com/wp-content/uploads/2011/05/empyema.jpg
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SPONTANEOUS PNEUMOTHORAXPresence of air in the pleural space
Causes
o Rupture of a sub pleural emphysematous bulla or pleural bleb
o Rupture of a sub pleural tuberculoses focu
Pathophysiology
o Closed - No communication between the pleura and lungs with deflation
o Open - Communication between the lungs and the pleural space generally through a bronchus
o Tension / Valvular
• Communication present but small and only allows movement of air in one direction
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SPONTANEOUS PNEUMOTHORAX
Clinical Features
o Onset generally rapid, may present with infection
o Pain or tightness on the affected side of the chest
aggravated by deep breathing and dependent on the size of the pneumothorax and the type
o Breathlessness and cyanosis
o Hyper-resonance on percussion and increased vocal resonance
Treatment and Management
o Bed rest
o Infection must be countered in open pneumothorax
o Valvular pneumothorax can be extreme resulting in death within minutes though generally time for medical intervention
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PNEUMOTHORAX
From Porth’s Pathophysiology: Concepts of Altered Health
States. (9th ed., p. 966), by
Sheila C. Grossman & Carol Mattson Porth.
Philadelphia, U.S.A. Walters Kluwer Health - Lippincott,
Williams & Wilkins
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CONGENITAL DISORDERS OF
THE DIAPHRAGM
Diaphragmatic Hernias
o Defects of the diaphragm allowing herniation of the abdominal viscera. More commonly found towards the posterior aspect
Eventration of the diaphragm
o Abnormal elevation or bulging of one hemidiaphragm
o Usually asymptomatic
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ACQUIRED DISORDERS
OF THE DIAPHRAGMDiaphragmatic Paralysis
• Generally through phrenic nerve damage to one hemi-
diaphragm
Clinical Features
• Loss of ventilatory capacity (20%)
• Generally asymptomatic in a healthy individual
Causes
• Bronchial carcinoma
• Injury or disease of the cervical vertebrae including birth
injuries, surgery and aneurysms
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DEFORMITIES OF
THE CHEST WALL
Thoracic Kyphoscoliosis
Abnormalities of alignment of the dorsal spine
Causes
• Congenital abnormality
• Vertebral Disease - TB, osteoporosis, ankylosing spondylitis
• Trauma
• Neuromuscular disease - Poliomyelitis
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KYPHOSIS
https://uprightdoctor.files.wordpress.com/2011/07/kyphosis-adult-hyper.jpg
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DEFORMITIES OF
THE CHEST WALL
Clinical Manifestations (if severe)
• Restriction and distortion of the chest wall
• Misdistribution of the ventilation and blood flow to the lungs
• Can develop
– Type II respiratory failure - Pulmonary hypertension
– Right ventricular Failure - Survival beyond middle age uncommon
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Readings and ResourcesResources:
o Set Textbooks:
Colledge, N.R., Walker, B.R. & Ralston S.H. (2014). Davidson’s Principles and Practice of Medicine, (22nd ed.). Edinburgh.
Churchill Livingstone.
Grossman, S.C. & Porth, C.M. (2014). Porth’s Pathophysiology: concepts of altered health states, (9th ed.). Philadelphia,
U.S.A. Walters Kluwer Health - Lippincott, Williams & Wilkins.
o Additional textbooks:
Davies, A. & Moores, C. (2010). The respiratory system: basic science and clinical conditions, (2nd ed.). Edinburgh. Churchill,
Livingstone, Elsevier.
Field, M., Pollock, C., Harris, D. (2010). Systems of the Body: The Renal System; Basic Science and Clinical Conditions. (2nd
ed.). United Kingdom: Churchill Livingstone.
Jamison, J.R. (2006) Differential Diagnosis for Primary Care: a handbook for health care practitioners. (2nd ed.). Edinburgh.
Churchill Livingstone.
Lee, G. & Bishop, P. (2013). Microbiology and Infection Control for Health Professionals, (5th ed.). Frenchs Forest, NSW.
Pearson Education.
McCance, K.L. & Huether, S.E. (2014). Pathophysiology: the biological basis for disease in adults and children, (7th ed.). St.
Louis, MO. Elsevier.
Murphy, K. (2011). Janeway’s immunobiology, (8th ed.). New York. Garland Science.
Noble, A., Johnson, R. & Bass, P. (2010). The cardiovascular system: basic science and clinical conditions, (2nd ed.).
Edinburgh. Churchill, Livingstone, Elsevier.
Pagana, K.D. & Pagana, T.J. (2013). Mosby’s diagnostic and laboratory test reference, (11th ed.). St. Louis, MO. Elsevier.
Smith, M.E. & Morton, D.G. (2010). The digestive system: basic science and clinical conditions, (2nd ed.). Edinburgh.
Churchill, Livingstone, Elsevier.
VanMeter, K.C. & Hubert, R. (2014). Gould’s pathophysiology for health professions, (5th ed.). St. Louis, MO. Elsevier.
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