Pathology of the respiratory system Markéta Hermanová Structures of upper and lower respiratory tract Structure and nomenclature of the lower RT Histology of respiratory tract 02_06 Cellular components of bronchial mucosa 02_07 The respiratory membrane respmemb Most common signs and symptoms of pulmonary diseases nCough nDyspnoea nAbnormal sputum nChest pain nHemoptysis nCyanosis nDigital clubbing nAltered breathing patterns Clinical problem Respiratory tract (RT) disease Sputum -Clear or mucoid -Purulent -With blood Excess secretion of mucus in asthma, chronic bronchitis Inflammatory exudate from RT infection Cardiac failure, pulmonary infarction, ulceration of respirátory mucosa, tumor Breathlessness Asthma, chronic bronchitis, emphysema, pneumonia, penumothorax, pulmonary embolism Chest pain Pleuritis, pulmonary embolism Finger clubbing Thickening and widening of terminal phalanges in lung cancer, bronchiectasias Headache Cerebral metastases from lung cancer Hemoptysis RT infection, pulmonary embolism, lung cancer, bronchiectases, tbc Cough Reflex response to presence of mucus, exudate, tumor or foreign material Ankle swelling Right heart failure due to pumonary hypertension Confusion Hypoxaemia due to respiratory cause Pathological basis of respiratory signs and symptoms Sign or symptom Pathological basis Wheezing -On inspiration -On expiration Narrowing of the proximal airways (e.g. by tumor) Distal bronchial narrowing (e.g. In asthma) Dyspnoea Decreased oxygen in blood (impaired alveolar gas exchange, left heart failure, anaemia) Cyanosis Increased non-oxygenated haemoglobin (e.g. circulatory bypassingof lungs in congenital heart diseases, impaired alveolar gas Exchange) Pleuritis pain Pleural irritation due to inflammation, infarction, tumor Pleural effusion -Transudate (low protein) - -Exudate (high protein) Cardiac failure, hypoglobulinaemia (e.g. in cirrhosis, nephrotic syndrome) Pleural inflammation, tumors. Clubbing Lung carcinoma, pulmonary fibrosis, cirrhosis, inflammatory bowel diseases Weight loss Tbc, tumors Major aetiological factors of respiratory diseases Aethiological factor Disease Genetic Cystic fibrosis Alpha-1 antitrypsin deficiency Some asthma Environmental -Smoking - - - - -Air pollution - - -Occupation - - - - -Infection Lung cancer Chronic bronchitis Emphysema Susceptibility to infection Chronic bronchitis Susceptibility to infection Pneumoconiosis Asbestosis Mesothelioma Lung cancer Influenza, measles, bacterial pneumonias, tbc. Respiratory infections – upper respiratory tract (URT) nRhinitis -Common cold -viral and bacterial infections, allergic reaction (hay fever) -Nasal polyp = chronic hyperplastic rhinitis n nSinusitis -Inflammation of paranasal sinuses; acute or chronic -Complicated by cranial osteomyelitis, meningitis or cereebral abscess n nWegener granulomatosis -autoimmune disease, vasculitis involving also URT (+ necrotising vasculitis in lungs and glomerulonephritis) - nOtitis media -Infection of middle ear, often assoc. with URT infection - n Respiratory infections – upper respiratory tract (URT) nLaryngitis -Viral or bacterial -Diphteria – was a common and most serious bacterial cause n nEpiglottitis -Cause by Haemophilus influenzae -Swollen epiglottis causes airway obstruction (intubation necessary, tracheostomy) n Respiratory infections – lower respiratory tract (LRT) nBronchitis -Cough, dyspnoe, tachypnoe, sputum -Acute or chronic (cough and sputum 3 month in 2 years, often in smokers) -Viral or bacterial (Haemophilus influenzae, Streptococcus pneumoniae) n nBronchiolitis -Usually primary viral infection in infants (RSV), causes dyspnoe and tachypnoe -May be secondary to other inhaled irritants, also a part of systemic disease (by pathologic immune reaction) - n Respiratory infections – lower respiratory tract (LRT): Pneumonia nAlveolar inflammation nBronchopneumonia and lobar pneumonia n n pneumonie -Patchy consolidation -Centred on bronchioles or bronchi -Purulent inflammation -Often in infancy or old age -Often secondary -Affects segments or whole lobe -Uncommon in infancy and old age -Males more than females -90 % due to Streptococcus pneumoniae -Cough and fever with sputum -Fibrinous inflammation - Healing complicated by fibrotisation Pulmonary infections nFactors which favor the pneumonia -Chronic diseases -Immunologic defficiency, immunosuppressive treatment -Unusual virulent infections, nosocomial infections -Loss or suppression of the cough reflex (neuromuscular disorders, chest pain, drugs; could cause aspiration of gastric content,…) -Injury of mucociliary apparatus (smoking, viral disease, genetic disorders, inhallation of hot or corrosive gases,…) -Pulmonary congestion and edema -Accumulation of secretion (bronchial obstruction, mucoviscidosis,…) - - Implications for therapists nCareful hand hygiene n nAdequate hydratation and airway clearance technique n(deep breathing, coughing and ventilation-perfusion ventilatory support and supplemental oxygen) n nProper positioning Pneumonia syndromes nCommunity-acquired acute pneumonia n(streptococcus pn., haemophilus inf., Moraxella cat., Staphylococcus a., Legionella pn., Klebsiella pn., Pseudomonas ae.,….) nCommunity-acquired atypical pneumonia n(Mycoplasma pn., Chlamydia spp., Coxiella burnetti, viruses,…) nNosocomial pneumonia n(G- rods (Klebsiella spp., Serratia marcescens, E. coli, Pseudomonas spp.,…)) nAspiration pneumonia n(mixture od anaerobic and aerobic flora) nChronic pneumonia n(Nocardia, Actinomyces, granulomatous (mycobacterium tbc, atypical mycobacteria, Histoplasma capsulatum, Coccidioides immitis, Blastomyces dermatidis) nNecrotizing pneumonia and lung abscess n (mixture of aerobic and anaerobic flora, Staphylococcus a., Klebsiella pn., Streptococcus pyogenes and pneumoniae) nPneumonia in the immunocompromised host n (CMV, Pneumocystis c., Mycobacterium avium intracellulare, aspergillus, Candida a. and others listed above,…), n Bronchopneumonia brpn1 brpn2 copy Atypical pneumonias (usually interstitial) nInfective nIn non-immunosuppressed hosts -Viral and mycoplasma pneumonia -Legionnaires´disease (Legionella pneumoniae) - nNon-infective -Idiopatic interstitial pneumonitis and fibrosis (v.s. autoimmune) -Cryptogenic organizing pneumonia = bronchiolitis obliterans -Aspiration pneumonia (primarily non interstitial, often with lung abscess) -Eosinophilic (v.s. allergic) and hypersensitivity pneumonitis -Pulmonary involvement in collagen vascular diseases n(systemic lupus erythematodes, rheumatoid arthritis, progressive systemic sclerosis n(scleroderma), dermatomyositis-polymyositis, mixed connective tissue disease) - n Alveolar walls = interstitium in lungs…..in interstitial inflammation the inflammatory infiltrate withihn the alveolar walls…complication of alveolocapillary gas exchange In immunosuppressed hosts -Pneumocytis carinii -Fungi (e.g. Candida albicans, Aspergillus) -Viruses (CMV, HSV, varicella zoster) TBC of the lungs tbc1 tbc2 primary tbc secondary tbc miliary tbc nObstructive pulmonary diseases n n(increase in resistence to airflow due to partial or complete obstruction at any level) n nChronic bronchitis nEmphysema nAsthma nBronchiectasia n nChronic bronchitis + emphysema = chronic obstructive pulmonary disease (COPD) nRestrictive pulmonary diseases n n(reduced expasion of lung parenchyma with decreased total lung capacity) n nChest wall disorders in presence of normal lungs (kyphoscoliosis, pleural diseases, severe obesity, neuromuscular disorders such as poliomyelitis) nAcute or chronic interstitial and infiltrative diseases Emphysema lung13 10x20x2 -Abnormal enlargement of alveolar airspaces -Usually seen in smokers in association with chronic bronchitis. -Risk factors: smoking, alpha-1antitrypsin deficiency (inhibitor of proteases) Bullous emphysema Rupture causing pneumothorax and collapse of the lung emfyzém1 Asthma bronchiale asma -Reversible small airways obstruction characterised by bronchospasm, inflammation and oedema. Paroxysmal attacks -Overdistended lungs, mucous plugs in bronchi, mucous gland and smooth muscle hypertrophy astma Asthma bronchiale nExtrinsic, atopic n (IgE-mediated external allergens) n nIntrinsic, non-atopic, idiopathic n (secondary to infection?) n nAspirin – induced n nAllergic bronchopulmonary aspergillosis n nOccupational n n(Exercise – induced) n n n Bronchiectasis: permanent dilatation of bronchi and bronchioles image00110 kopie -Results from pulmonary inflammation and scarring due to infection, bronchial obstruction or lung fibrosis (e.g. after radiotherapy) - -Secondary inflammationlead to further destruction of airways - -Chronic cough with dyspnoe and production of copious amount of foul-smelling sputum - -Complications: pneumonia, abscess, amyloid, pulmonary fibrosis, cor pulmonale - -Arise from: genetic conditions (cystic fibrosis), childhood infections, chronic lung infections (tbc), radiotherapy in lung and breast cancer, distal to bronchial obstruction, immunodeficiency, aspergillosis Implications for the therapists nPulmonary rehabilitation nSmoking cessation, physical activity n n(breathing exercises, airway clearance techniques, physical training, program to improve posture and conditioning of respiratory musculature) n n Pulmonary edema (congestion) nHemodynamic 1.Increased pulmonary venous pressure – venostatic edema (left sided heart failure, mitral stenosis or incompetence, volume overload, pulmonary vein obstruction) 2.Decreased oncotic pressure (hypoalbuminemia, nephrotic syndrome, liver disease, protein loosing enteropathies) 3.Lymphatic obstruction n nDue to microvascular injury (alveolar injury; leakage of fluids and proteins into interstitium and later into alveoli) 1.Infections (pneumonia, septicemia), shock, trauma 2.Inhalation of toxic gases 3.Liquid aspiration 4.Drugs, chemicals 5.Radiation n nUndetermined origin 1.High altitude 2.CNS trauma Pulmonary edema 9x40 9x400 - working with a client already dianosed with PE – sitting position preferred.. Atelectasis; collapse nAtelectasis (primary; neonatal; immature neonates, lack of surfactant) n nCollapse (secondary, previously inflated lungs) = acquired atelectasis 1.Obstruction (resorption of the air after the blockage) 2.Compression (hydrothorax, pneumothorax, pleural exsudate) 3.Contraction atelectasis (fibrotic changes prevent full expansion) Acute respiratory distress syndrome (in adults - ARDS) – diffuse alveolar damage (DAD) – shock lung: Acute respiratory failure after a systemic or pulmonary insult nDiffuse pulmonary infections, sepsis nGastric aspiration nChemical injury (heroin, methadon, barbiturate overdose, acetylsalicylic acid) nPhysical injury nHematologic conditions (DIK, multiple trasfusions) nPancreatitis nUremia nInhaled irritants (oxygen toxicity, smoke, toxic gases) nHypersenzitivity reaction (organic solvents, drugs – anticancer treatment) nCardiopulmonary bypass Pneumoconioses nNon-neoplastic lung reaction (usually occupational) to inhalation of organic (mineral dusts) as well as inorganic particulates and chemical fumes and vapors n nConsequences of inhalation of different materials: nInert (does not induce fibrotisation), e.g. simple coal workers pneumoconiosis (inhalation of coal dust, antracosis caused by inhaled carbon) n nFibrous, e.g. silicosis (silica inhalation), asbestosis resulting in lung fibrosis, respiratory insufficiency and cor pulmonale n!!!increased risk of mesothelioma and lung cancer in asbestosis!!! n nAllergic (inhalation of organic dusts, e.g. Farmer´s lung) Pulmonary silicosis Silica particles under polarized light Sarcoidosis -multisystem inflammatory disorder - -noncaseating granulomas; 90 % lung and hilar lymph nodes involvement - -idiopathic, unknown etiology; may represent a pathologic immune response in genetically predisposed individuals exposed to certain enviromental agents(e.g. mycobacteria, Rickettsia species, Propionibacterium acnes) n -other organs affected: spleen, liver, bone marrow, skin, eye and associated glands, salivary glands, muscle n Pulmonary embolism nHypercoagulable state -Primary (factor V Leiden, antiphospholipid syndrome,…) -Secondary (obesity, surgery, oral contraceptives, pregnancy,…) n n embolism Pulmonary hypertension nPulmonary blood pressure =1/8 of systemic blood pressure nPulmonary hypertension → cor pulmonale n nIN: nChronic obstructive and restrictive lung diseases nAntecedent congenital or aquired heart disease (venostasis before left heart) nRecurrent thrombemboli nAutoimmune disorders involving pulmonary vasculature nVenooclusive lung disease (musculoelastic thickening of subpleural and interlobular septal veins) nPrimary idiopathic pulmonary hypertension n n Tumors of the lung nMost common primary malignant tumor in the world; related to cigarette smocing, assoc. with occupational exposure to carcinogens, generally with poor prognosis n nEpithelial -Benign (adenomas) -Malignant -squamous cell carcinoma -adenocarcinoma -small cell (neuroendocrine) carcinoma - undifferentiared carcinomas-large cell) nMesenchymal nOthers nSecondary – metastatic tumors Small-cell lung carcinoma 1 1 1 14_02 1 Peribronchial and perivascular infiltration 2 Infiltration of lymph nodes in hilus 3 Bronchus 4 Vessels 1 1 2 3 4 Squamous cell lung carcinoma 1103S2 11_03 1. Tumor localized in the periphery 2. Central necrosis 1 2 1. Tumor in bronchus 2. Segmental bronchus 2 1 Complications of lung cancer ca plic Thank you for your attention….