Respiratory (2) Flashcards
Define idiopathic pulmonary fibrosis
A chronic, progressive, interstitial restrictive lung disease of an unknown cause which results in fibrosis and thickening of interstitial lung tissue and dyspnoea.
Summarise the aetiology of idiopathic pulmonary fibrosis
Unknown cause
Occurs in genetically predisposed patients
Type II pneumocytes overproliferate, causing increased numbers of myofibroblasts which produce collagen in the interstitium
Risk factors: Age 50-70 Male Smoking Occupational dust, metal or wood exposure GORD Diabetes Family history
Summarise the epidemiology of idiopathic pulmonary fibrosis
More common in males
More common with increasing age - age at presentation 60-70 years old
Familial form presents younger - 55 to 60 y/o
What are the presenting symptoms of idiopathic pulmonary fibrosis?
Progressive symptoms Dry, non-productive, irritative cough - does not respond to anti-tussives Dyspnoea on exertion NO WHEEZE Weight loss Fatigue Malaise
What are the signs on physical examination of idiopathic pulmonary fibrosis?
Clubbing
Cyanosis
Dry late-inspiratory bibasilar fine crackles
Signs of right heart failure in advanced disease
What are the appropriate investigations for idiopathic pulmonary fibrosis?
CXR:
Usually normal at presentation.
Early disease may show ground glass shadowing.
Late stage disease shows reticulonodular shadowing and signs of cor pulmonale.
High-resolution CT: more sensitive in early disease the an CXR. Reticular opacities, honeycombing, increased thickness of interstitial space
Pulmonary Function Tests: Restrictive features - decreased FEV1, FVC, TLC, lung compliance, normal or increased FEV1/FVC
ANA, anti-CCP and Rheumatoid Factor: 1/3 of patients are positive for ANA or RF
Bronchoalveolar Lavage: exclude infections and malignancy
Lung Biopsy: gold standard for diagnosis BUT may not be appropriate - fibrosis, areas of normal surrouded by scar tissue, honeycombing
Define lung cancer
Primary malignant neoplasm of the lungs
Can be small cell or non-small cell
Small cell/oat cell carcinoma - malignant epithelial tumour arising from cells lining the lower respiratory tract
Non-small cell: Adenocarcinoma Squamous cell carcinoma Large cell Bronchial carcinoid
Summarise the aetiology of lung cancer
Small cell carcinoma:
Highly associated with smoking
From small, immature neuroendocrine cells
Aggressive, rapidly metastasising
Adenocarcinoma:
Most common type in female non-smokers
Squamous cell:
Highly associated with smoking
Other risk factors: FHx Radon exposure Asbestos Air pollution Ionising radiation
Summarise the epidemiology of lung cancer
Adenocarcinoma is most common type in non-smokers
Adenocarcinoma - 35%
Squamous cell carcinoma - 30%
Small cell carcinoma - 30%
Large cell carcinoma - 15%
Non-small cell 3 times more common in men
What are the presenting symptoms of lung cancer?
Local symptoms:
Cough
Dyspnoea
Weight loss, fever, fatigue, night sweats
Infiltration into blood vessels - haemoptysis
Symptoms from compression of SVC - facial swelling, dyspnoea
Symptoms from compression of laryngeal nerve - hoarse voice
Symptoms from compression of brachial plexus - pain in shoulder and arm
Small cell:
Symptoms from SIADH
Symptoms of due to ACTH paraneoplastic Cushing’s - weight gain, fatigue
Symptoms of Lambert-Eaton syndrome - weakness which improves with use
Squamous cell carcinoma:
Symptoms of hypercalcaemia due to paraneoplastic PTHrp release - abdominal pain, confusion, bone pain, constipation
What are the signs on physical examination of lung cancer?
Wheeze, crackles, reduced breath sounds, dullness to percussion
Weight loss
Horner’s syndrome due to pancoast tumour - miosis, anhydrosis, ptosis
Signs of Cushing’s if paraneoplastic small cell
SVC compression: facial congestion, distension of neck veins, upper limb oedema
Brachial Plexus: wasting of small muscles of hand
Hypertrophic Osteoarthropathy - clubbing, painful swollen wrist/ankles (periosteal new bone formation) - adenocarcinoma
Signs of metastases:
Hepatomegaly
Supraclavicular lymphadenopathy
What are the appropriate investigations for lung cancer?
CXR - coin lesion, single or multiple pulmonary nodules, Lobar Collapse, Pleural Effusion, Mediastinal or Hilar Fullness
Small cell and squamous cell likely central mass
CT chest, liver, adrenals - non-calcified nodule, useful for staging
Bronchoscopy and biopsy
Sputum cytology - malignant cells
FBC - may show anaemia
U&E’s - elevated calcium due to PTHrp paraneoplastic squamous cell carcinoma
LFTS (normal/elevated if lone, increased ALP in bone metastases)
Isotope bone scans
Define extrinsic allergic alveolitis
An excessive immune response to inhaled antigens, usually from occupational exposures to organic dusts, leading to inflammation of the alveoli and distal bronchioles.
Summarise the aetiology of extrinsic allergic alveolitis
Inhalation of antigenic dusts (microbes, animal proteins) induce a hypersensitivity response in susceptible individuals
Farmer’s lung - inhalation of thermophilic actinomycetes from damp, moudly hay
Pigeon fancier’s lung - inhalation of bird poo and feathers
Malt worker’s lung - mouldy barley containing aspergillus
Exposure to inhalation of actinomycetes from mouldy sugar cane
Humidifier lung: water containing bacteria and Naegleria (amoeba)
Risk Factors:
Smoking
Viral infection
Exposure to: avian protein, mould and bacterial antigen
Summarise the epidemiology of extrinsic allergic alveolitis
UNCOMMON (2% occupational lung disease)
50% reported cases affect farm workers
6-21% of pigeon breeders found to develop extrinsic allergic alveolitis
What are the presenting symptoms of extrinsic allergic alveolitis?
Acute: (present 4-12 hours after exposure) Dyspnoea Fever Dry cough Chest tightness Headache Malaise Myalgia
Chronic: Increasing dyspnoea Decreased exercise tolerance Fatigue Weight loss
What are the signs on physical examination of extrinsic allergic alveolitis?
Acute:
Pyrexia
Rapid shallow breathing
Inspiratory crepitations
Chronic:
Fine inspiratory crepitations
Clubbing
What are the appropriate investigations for extrinsic allergic alveolitis?
FBC - increased WBC, anaemia
ESR - elevated
Albumin - low in chronic disease
CXR - diffuse infiltrates, fibrosis, may be normal in acute episode
CT chest - ground-glass opacities
Bronchoalveolar lavage - fluid shows lymphocytosis and increased mast cells, positive antibody
Inhalation challenge - shows immunological response to causative antigen
Pulmonary function test - restrictive lung disease (low FEV1, low FVC, decreased TLC, increased FEV1/FVC)
Lung biopsy - granulomas, lymphocyte infiltration in alveolar wall - in patients with lack of exposure history or atypical presentation
Define pneumoconiosis
A group of interstitial fibrosing lung diseases which are caused by chronic exposure and inhalation of metal, mineral and dust particles.
Summarise the aetiology of pneumoconiosis
Caused by inhalation of particles of coal dust, silica and asbestos
Risk Factors:
Occupational exposure (coal mining, quarrying, iron & steel works, industry, plumbers)
High Cumulative dose of inhaled silica or coal
Cigarette Smoking
Tuberculosis (cofactors that contribute)
Summarise the epidemiology of pneumoconiosis
Silicosis - sand blasting, silica miners
Coal worker’s pneumoconiosis - coal miner
Asbestosis - shipworker, roofing, insulation
Incidence is increasing in developed countries due to delayed disease following exposure
What are the presenting symptoms of pneumoconiosis
Can be asymptomatic and picked up on routine CXR
Dyspnoea on exertion
Dry cough
Black sputum in coal workers pneumoconiosis
Pleuritic chest pain in Asbestosis
What are the signs on physical examination of pneumoconiosis?
Crackles
Wheeze
Clubbing
Coal-worker’s pneumoconiosis and silicosis: Decreased breath sounds
Asbestosis: End-inspiratory crepitations & Clubbing
General: Cyanosis, Barrel Chest, Weight Loss
Signs of:
Pleural effusion or right heart failure (cor pulmonale)
Rheumatoid arthritis or scleroderma (uncommon complications of silica/coal exposure)
Renal Failure (may be presenting condition)
What are the appropriate investigations of pneumoconiosis?
CXR:
Micronodular mottling
Silicosis - upper lobe eggshell calcification
Asbestosis - pleural and subdiaphragmatic plaques
Spirometry - restrictive lung disease - decreased FEV1, FVC, TLC, increased or normal FEV1/FVC
CT Scan: fibrotic changes can be visualised early, upper zone interstitial fibrosis (progressively involves the whole lung)
Bronchoscopy: allows visualisation and bronchoalveolar lavage
Define aspergillus lung disease
Lung disease associated with Aspergillus fungal infection (usually caused by aspergillus fumigatus)
Summarise the aetiology of aspergillus lung disease
Inhalation of the Aspergillus spores can produce 3 different clinical pictures:
Aspergilloma: growth of an A. fumigates mycetoma ball in a pre-existing lung cavity i.e. post TB, old infarct or abscess
Allergic Bronchopulmonary Aspergillosis: colonisation of airways by aspergillus leads to IgE &IgG mediated immune responses. The release of proteolytic enzymes, mycotoxins and antibodies leads to airway damage and central bronchiectasis. Usually occurs in asthmatics.
Invasive Aspergillosis: invasion of aspergillus into lung tissue and fungal dissemination. Occurs in immunosuppressed patients i.e. neutropenia, aids, steroids
Risk factors: Allogenic stem cell transplantation Prolonged severe neutropenia (>10 days) Immunosuppressive therapy Aplastic anaemia
Summarise the epidemiology of aspergillus lung disease
Uncommon
Clinical incidence is < 1 in 100 hospital admissions
More common in males and immunocompromised
What are the presenting symptoms of aspergillus lung disease?
Aspergilloma: asymptomatic, haemoptysis (can be very significant), cough, weight loss, lethargy
Allergic bronchopulmonary aspergillosis: difficulty in controlling asthma, recurrent episodes of pneumonia with wheeze, cough, fever and malaise
Invasive aspergillosis: facial pain, dyspnoea, rapid deterioration, septic picture, headache, congestion or sinus tenderness, Intracranial space occupying lesion: seizure, altered mental status and cranial nerve palsy
What are the signs on physical examination of aspergillus lung disease?
Aspergilloma: tracheal deviation if large
Allergic Bronchopulmonary Aspergillosis: dull affected lung, dull breath sounds, wheeze
Invasive Aspergillosis: cyanosis
What are the appropriate investigations for aspergillus lung disease?
Aspergilloma:
CXR: round opacity may be seen with crescent of air surrounding it (usually upper lobe)
CT/MRI: if CXR does not clearly delineate opacification Sputum cultures may be -ve if no communication between colonised cavity & bronchial tree
Allergic Bronchopulmonary Aspergillosis:
Immediate skin test reactivity to Aspergillus antigens
Eosinophilia, ↑ total serum IgE, ↑ specific serum IgE & IgG to A.fumigates
CXR: transient patchy shadows collapse, distended mucus filled bronchi (bronchiectasis), Signs of complications: fibrosis in upper lobes and bronchiectasis
CT: lung infiltrates, central bronchiectasis
Lung Function Test: reversible airflow limitation, reduced lung volume/gas transfer
Invasive Aspergillosis:
Detection of Aspergillus in cultures OR histological examination
Bronchoalveolar lavage fluid or sputum may be used diagnostically
Chest CT: nodules surrounded by ground glass appearance ‘halo sign’ (haemorrhage into tissue surrounding area of fungal invasion
