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RESPIRATORY -----, TRACT

Respiratory infections 59 Tu bercu losis 60 Obstructive lung disease 1 61 Obstructive lung disease 2 62 Restrictive lung disease 1 63 Restrictive lung disease 2 64 Sarcoidosis 65 Lung tumours 66 Pleura 67

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Cilia, mucous blanket covering mucosa, cough reflex, alveolar macrophages, IgA, cell mediated immuni

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Acute bronchitis

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Bronchitis Pneumonias mainly specific to immunosuppressed
Chronic bronchitis Clinical features

See obstructive airwa s disease

Nocardia Actinom ces

Intense suppuration with abscess formation

Chronic pneumonias
Granulomatous

Bronchiolitis

Intra-alveolar exudate, consolidation Lobar pneumonia - part or all of lobe, streptococcus pneumoniae 90%, klebsiella Anatomic distribution Bronchopneumonia - (commonest form) patchy distribution generally involving more than one lobe pneumococcus, staph aureus, haemophilus influenzae Pre-antibiotic era, whole or almost whole of lobe Con estion - vascular con estion and oedema Red hepatisation - neutrophils, red cells and fibrin Gre hepatisation - fibrinous exudate Resolution - exudate di ested, architecture left intact Consolidation in patches in one or several lobes most frequently bilateral and basal Focal suppurative exudate filling bronchi, bronchioles and adiacent alveoli

Special pneumonias (Non-immunosuppressed hosts)

Acute bacterial pneumonia

Extremely variable - moderate chest symptoms to fulminant life threatening infection in immunocompromised Acute fever, headache, malaise Cou h with minimal sputum Prognosis - uncomplicated cases good, may have bacterial superinfection

Bronchopneumonia

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Complications

Abscess formation Em ema Fibrosis Septicaemia, meningitis, arthritis, infective endocarditis

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SYSTEMATIC PATHOLOGY
6% of deaths worldwide Only a small proportion of those who are infected develop active disease Poverty, crowding, chronic debilitating disease, HIV infection Mycobacterium hominus - most cases
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Epidemiology Aetiology
Mycobacterium bovis Mycobacterium avium intracellulare

Transmission from active cases by inhalation of airborne or anisms Contaminated milk intestinal and oropharyngeal tuberculosis Less virulent 10 to 30% of AIDS patients with tuberculosis

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Haematogenous spread Meninges, kidneys, adrenals, bones, fallopian tubes

Isolated organ tuberculosis

Tissue destruction mediated lar ely b host response Initial non-specific immune response

Pathogenesis

After 2 to 3 weeks delayed hypersensitivity granulomatous response Caseating destructive response to tubercle bacilli

Diagnosis

Identification of tubercle bacillus in sputum or tissue biopsy

Acid fast bacilli on Ziehl-Neelsen stain Culture PCR

Secondary tuberculosis
Localised - as m tomatic Insidious symptoms - malaise weight loss, anorexia, fever, . ni ht sweats

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Primary tuberculosis

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Limitation of airflow Ratio of FEV1 to FVC is decreased

Definition

Localised obstructive airways disease

Permanent dilatation of bronchi and bronchioles due to destruction of the muscle and elastic supporting tissue, resulting from or associated with chronic necrotisin infections Bronchial obstruction - forei n bodies Congenital or hereditary conditions - cystic fibrosis, Kartagener's s ndrome Necrotising or suppurative neumonia Obstruction Chronic ersistent infection

Episodic reversible bronchospasm due to exaggerated bronchoconstrictor res onse to various stimuli Bronchial beta-receptors may be insensitive to catecholamines partially constricted bronchi which do not dilate well to beta-a onists

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Some forms mediated by type I and t e III h ersensitivit reactions Ectopic asthma - type 1 hypersensitivity reaction, raised serum I E Non-atopic asthma - respiratory tract infections especially in chronic bronchitis, ma be due to irritation in unusuall reactive airwa s

Bronchiectasis

Aspirin induced - mechanism of induction by aspirin unknown. Recurrent rhinitis, nasal I s and urticaria

Asthma
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Occupational asthma - combination of type I and type III hypersensitivity reactions induced b an anti en inhaled at work Allergic bronchopulmonary aspergillosis inhalation of Aspergillus fumigatus spores induce a type I and then a type III h ersensitivit reaction

Persistent cou h

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Episodic dyspnoea, cough and wheezin 5% adults, 10% children Status asthmaticus - severe asthma not responsive to thera

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Definition

Permanent enlargement of airspaces distal to the terminal bronchioles with destruction of their walls Common Heavy cigarette smokers Men> women

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Persistent productive cough for at least 2 consecutive months in at least 2 consecutive ears

Definition Excess protease or elastase activity unopposed by appropriate anti-protease regulation Centriacinar Proximal part of acini-respiratory bronchioles Acini are uniforml enlar ed

Bronchial mucous gland hypersecretion and hypertrophy and surface epithelium metaplasia caused b irritants

Panacinar

Emphysema Chronic bronchitis

Classification Distal acinar

Prominent productive cou h Ma develop outflow obstruction May be accompanied by hypercapnia, hypoxaemia and c anosis 'blue bloaters' Cor pulmonale

Proximal part of acinus normal only distal part involved occurs adjacent to areas of fibrosis or atelectasis more striking subplerual spontaneous pneumothorax

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Adult respiratory distress syndrome Causes (ARDS): shock, trauma, infections, gas inhalation, Disseminated intravascular coagulation (DIC) . Reduced lung compliance (increased lung stiffness) FVC and FEV1 reduced - FEV1/FVC near normal Aetiolo Drug and toxin reactions Cytotoxic drugs ego busulphan and bleomycin -> low grade alveolitis and interstitial fibrosis Para uat - similar to ARDS Radiation pneumonitis High dose radiation -> diffuse alveolar damage Lower doses -> progressive ulmona fibrosis Diffuse intrapulmonary haemorrhage Goodpasture's syndrome - antibody acts on alveolar membranes -> pulmonary haemorrhage Idiopathic pulmonary haemosiderosis - (rare) in children, intra-alveolar haemorrhage Insult to alveolar - capillary walls
-> diffuse alveolar damage.

Definition

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Acute interstitial lung diseases

Affect ability of lung to act as bellows Severe obesity, kyphoscoliosis, neuromuscuiar disorders affecting respiratory muscles eg. Guillain-Barre syndrome

Neutrophils and macrophages im ortant

Extrapulmonary

disorders

Diffuse alveolar damage, alveolar exudation, hyaline membranes and type II pneumocyte hyperplasia Short history dyspnoea, tachypnoea and res irato distress Prognosis - 50% die within first few days. Most of rest have full resolution. Some -> ulmona fibrosis

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Pathology
Interstitial fibrosis, lymphocytic and macrophage infiltrate, microc st formation.
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Slowly increasing dyspnoea, cough and finger clubbing - over months or ears

Clinical

Fibrosing alveolitis
Inhaled dusts either mineral or or anic Reaction may be inert, fibrous, aller ic or neoplastic Distribution of lung disease depends on ph sical properties of the dust Anthracosis - coal dust pigment in macrophages in lun - as m tomatic Simple coalworkers pneumoconiosis aggregates of dust laden macrophages, no scarring but local dilatation of res irato bronchioles Complicated coalworkers pneumoconiosis large irregular nodules with scarring. Associated emphysema, scarring and lung destruction -> honeycomb lung and res irato failure or cor ulmonale. / Caplan's syndrome - co-existence of coalworkers pneumoconiosis and rheumatoid arthritis -> development of ulmona nodular lesions ears

Extrinsic allergic alveolitis

Clinical features

Coalworkers neumoconiosis

Pneumoconioses

Langerhans cell ranulomatosis

Sarcoidosis
Silicosis Pro Diffuse ulmona interstitial fibrosis Asbestosis

See sarcoidosis map

Asbestos bodies - iron coated asbestos fibres

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Multisystem disease of unknown cause characterised by non-caseating granulomas in many tissues and organs Incidence worldwide

Distintictive

ranulomatous response

Epidemiology

Pathogenesis

Cell mediated immune response No proven causative agent

Any organ Many asymptomatic Bilateral hilar adenopathy on chest X-ray Respiratory symptoms - d spnoea, cou h Constitutional symptoms - fever, fatigue, weight loss, anorexia, ni ht sweats Unpredictable course 20% permanent lung dysfunction or visual impairment, 10 to 15% progressive pulmonary fibrosis and cor pulmonale Lymph nodes - most cases, hilar and paratracheal commonly enlarged, discrete non-caseating granulomas, Schaumann bodies, asteroid bodies

Clinical course

Lungs - common site, granulomas throughout parenchyma, diffuse interstitial fibrosis

Pathology

Skin - 33 to 50%, subcutaneous nodules, erythematous plaques Eye, lacrimal glands, salivary glands - iritis, iridocyclitis Spleen and liver - 75% splenomegaly 20%, hepatomegaly 33% Myocardium - sudden death

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SYSTEMATIC PATHOLOGY
Cigarette smoking Radioactive Asbestos Dusts ego arsenic, vinyl chloride Radon gas Cough Haemoptysis Arise from bronchial Polypoid mucous glands lesions Adenomas Dyspnoea _.atelectasis lumen or sessile bronchial blockage of bronchial ores
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May cause obstruction

Hoarseness - recurrent laryngeal nerve involvement Recurrent pneumonia blockage of bronchial lumen

Coin lesion on X-ray Circumscribed, Nodules of cartilage hard, white lining

Raised hemidiaphragm (on X-ray) phrenic nerve involvement Superior vena cava syndrome

with bronchial

Horner's syndrome Clinical presentation Pleural effusion

More common than primary May arise from blood or lymphatic spread

Lymph nodes, bone, brain,liver and adrenals

Discrete nodules scattered throughout both lungs Lymphangitis carcinomatosa - diffuse involvement of lymphatics by carcinoma Pulmonary metastases from carcinomas, sarcomas and lymphomas Commonest primary sites of carcinomas - breast, kidney and GI tract

Secondary tumours

Primary lung carcinoma

Paraneoplastic syndromes: commoner with small cell carcinoma except where indicated

Hypercalcaemia - parathyroid related hormone - squamous carcinoma Cushings syndrome secretion of ADH syndromes

Inappropriate Neuromuscular

Finger clubbing, hypertrophic pulmonary osteoarthropathy Haematologic - migratory thrombophlebitis, nonbacterial endocarditis, DIC - adenocarcinomas myc amplification and inactivation of p53 and Rb - small cell carcinoma k-ras - 30% adenocarcinomas Most including some adenocarcinomas arise at the hilum, from bronchi Form masses invading lung parenchyma into

Rare Increased incidence in AIDS

Sarcomas

B cell phenotype Arise from bronchus and bronchiole associated Iymphoia tissue Lymphomatoid granulomatosis - rare, T cell lymphoma, destroys blood vessels

Primary pulmonary lymphomas

Other primary malignant tumours

Adenocarcinoma may also occur at the lung periphery - bronchioalveolar Non- small cell carcinoma: Squamous carcinoma Adenocarcinoma (bronchioalveolar) Large cell Small cell carcinoma Poor - patients present with late onset Non-small cell better than small cell

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Common Connective tissue disease e . rheumatoid disease

Inflammation
Maybe accompanied by effusion

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fibrous tumour

Sharp localised chest pain worse on res iration

. Most often secondary deposits from lun ,breast or ovarian carcinomas

Tumours

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Asymptomatic patches of thickened fibrotic pleura on the diaphragm and posterior thoracic wall H aline acellular connective tissue

Pleural plaques

Asbestos exposure

Diffuse pleural fibrosis and pleural effusions Mesothelioma

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