Respiratory - Level 2.2 Flashcards
Definition of sarcoidosis?
o Multisystem inflammatory chronic condition characterised by non-caseating epithelioid granulomata
Epidemiology of sarcoidosis?
- Prevalence highest in Northern Europe, 20-40 years old, Afro-Caribbeans
Symptoms of sarcoidosis - pulmonary disease?
o Bilateral hilar lymphadenopathy, pulmonary infiltrates or fibrosis
o Dry cough, progressive dyspnoea, decreased exercise tolerance and chest pain
Symptoms of sarcoidosis - skin?
Papules on face
Maculopapular rash on body or extremities
Brownish-red infiltrative plaques on extremities or trunk
Erythema nodosum on legs
Lupus pernio – raised, hardened lesion affecting nose and cheeks (pathognomonic)
Symptoms of sarcoidosis - eye?
Granulomatous uveitis • Anterior & posterior Keratoconjunctivitis Dry eyes Glaucoma
Symptoms of sarcoidosis - neurological?
Bell’s palsy Lymphocytic meningitis Diabetes insipidus Neuropathy – numbness, weakness Pituitary dysfunction
Symptoms of sarcoidosis - CV?
Arrhythmias or heart failure in young patient – cardiomyopathy
Symptoms of sarcoidosis - others?
Lymphadenopathy Hepatomegaly, splenomegaly Hypercalcaemia and hypercalciuria, renal stones Bone marrow failure Lacrimal and parotid gland enlargement
Investigations in sarcoidosis?
o Bloods FBC (raised WCC, lymphopenia, anaemia) ESR raised U&E Ca (elevated) LFTs (deranged) Serum ACE raised
o Tuberculin skin test (negative)
o If raised Ca:
24-hour urine collection – raised calcium
Imaging of sarcoidosis
CXR
• Bilateral hilar lymphadenopathy
CT
Further testing of sarcoidosis
o Lung function tests – restrictive pattern o ECG (arrhythmias or BBB) o Bronchoalveolar Lavage Raised lymphocytes, raised neutrophils with pulmonary fibrosis
Diagnostic testing of sarcoidosis
o Tissue Biopsy
Lung, liver, lymph nodes, skin
Non-caseating granulomas
Staging of sarcoidosis of CXR?
Staging on CXR
o Stage 0 =normal
o Stage 1= bilateral hilar lymphadenopathy (+/- paratracheal adenopathy)
o Stage 2 = Bilateral hilar adenopathy with pulmonary infiltrates
o Stage 3 = Parenchymal infiltrates without hilar adenopathy
o Stage 4 = Parenchymal involvement gives volume loss (fibrosis) and other features (calcifications, hilar retraction, cysts, bullae)
Management of sarcoidosis - acute sarcoidosis?
o Bed rest
o NSAIDs
o Remission is common
o If severe – oral prednisolone or IV Methylprednisolone
Management of sarcoidosis - chronic sarcoidosis - immunosuppression?
Prednisolone (0.5mg/kg/day) for 4-6 weeks, then lower dose over 6 months according to response and give for 6-24 months
• NOT INDICATED IN STAGE 1, 2, 3 IF ASYMPTOMATIC
• Indications
o Parenchymal lung disease (symptomatic, progressive, static)
o Uveitis
o Hypercalcaemia
o Neurological or cardiac involvement
• Oral bisphosphonates to minimise bone loss
Management of sarcoidosis - chronic sarcoidosis - other therapies?
- Methotrexate/azathioprine/hydroxychloroquine /ciclosporin/cyclophosphamide
- Anti-TNF alpha
- Rituximab
Management of sarcoidosis - chronic sarcoidosis - lung transplant?
Lung Transplant
• End stage lung disease
Management of sarcoidosis - chronic sarcoidosis - specific eye and skin features?
Eye – Topical prednisolone
Skin – Topical Triamcinolone or oral prednisolone
Management of sarcoidosis - chronic sarcoidosis - monitoring?
o Follow up every 3-6 months o Monitoring FBC DEXA every 2 years Serial lung function tests, CXR and ACE levels
Complications of sarcoidosis?
- Pulmonary – Infection, PE, fibrotic lung disease, pulmonary hypertension
- Cardiac – arrhythmias, HF, SCD
- Ocular – Uveitis, conjunctivitis
- Neurological – Nerve damage, TIA/CVA, CNS impairment
- ENT – salivary gland dysfunction, nosebleeds, obstruction
- Others – lymphoma, hypercalcaemia, renal stones, arthritis
Prognosis of sarcoidosis?
- 60% with thoracic sarcoidosis resolve over 2 years
- 20% respond to steroids
o If not, improvement unlikely
Definition of cystic fibrosis?
- Autosomal recessive condition causing a defective CFTR protein on chromosome 7
- CFTR is a cAMP dependent chloride channel found in membrane of cells
Pathology of cystic fibrosis?
- Leads to excessively thick mucus in many body systems
o Reduction in airway surface liquid layer and impaired ciliary function
o Chronic endobronchial infection ensues
o Thick meconium is produced
o Pancreatic ducts also become blocked by thick secretions
o Abnormal sweat glands
Epidemiology of cystic fibrosis?
- Incidence of 1 in 2500 live births with a carrier rate of 1 in 25
- Average life expectancy is mid-30s
Symptoms of infancy of cystic fibrosis?
- Meconium ileus (1 in 10)
- Prolonged neonatal jaundice
- Failure to thrive
- Recurrent chest infections
o Cough, purulent sputum, chest deformity
o Initially S.aureus, H.inflenziae
o Pseudomonas aeruginosa, burkholderia cepacia colonisations - Malabsorption (ADEK), steatorrhoea – failure to thrive
Symptoms of young child of cystic fibrosis?
- Bronchiectasis
- Rectal Prolapse
- Nasal Polyp
- Sinusitis
Symptoms of older child of cystic fibrosis?
- Allergic bronchopulmonary aspergillosis
- Diabetes Mellitus
- Cirrhosis/Portal Hypertension
- Intestinal Obstruction
- Pneumothorax
- Sterility in males – congenital absence of vas deferens
Signs of cystic fibrosis?
- Evidence of malnutrition, poor weight gain
- Hyperinflation of the chest
- Coarse inspiratory crackles
- Expiratory wheeze
- Finger clubbing
Testing of cystic fibrosis - in newborns?
- Screened for in Guthrie test
Testing CF - in everyone - when to test?
o FHx, congenital intestinal atresia, meconium ileus, symptoms of intestinal obstruction, faltering growth, undernutrition, recurrent LRTIs, chronic cough, chronic sinus disease, obstructive azoospermia, rectal prolapse
Testing CF - in everyone - what test to perform?
o Sweat Test (children and young people) >60mmol/L
Measuring sodium and chloride concentrations in sweat
o Gene Testing (Adults)
Testing CF - in everyone - when to refer to specialist?
o Positive or equivocal sweat test
o Assessment suggests CF but results normal
o Gene testing reveals 1 or more gene mutations
Management of cystic fibrosis - reviews?
o Annual review
Includes pulmonary (O2 sats, CXR, FBC, aspergillus serology and serum IgE, respiratory samples, LFTs), nutrition, liver disease, complications assessment, DM test
o Routine Reviews - O2 sats, secretion sample, LFT with spirometry Weekly in 1st month Monthly between 1-12 months Every 6-8 weeks between 1-5 years Every 8-12 weeks >5 Every 3-6 months as adults
Management of cystic fibrosis - prevention of cross infection?
o Specialist clinics are organised to prevent-cross infection – separate people individually during clinic & keep people in separate clinics depending on bacteria grown
o In-patients – individual rooms with en-suite facilities
Management of cystic fibrosis - exercise/physio?
Regular exercise improves lung function and fitness
o Individualised exercise programme and review
CT scan for under 12s or if exacerbation does not respond to treatment
Physiotherapy
o Airway clearance techniques taught to patient and carers
o Postural drainage, active cycle breathing or forced expiratory techniques
Management of cystic fibrosis - if lung disease?
o Nebulised rhDNAse (Dornase alpha) as first-line
o Add hypertonic sodium chloride if needed
o If not able to use – mannitol dry powder
Management of cystic fibrosis - if deteriorating lung function or repeated exacerbations?
o Azithromycin long-term at immunomodulatory dose
o If deteriorating – stop azithromycin and start oral prednisolone
Management of cystic fibrosis - treating infections?
o Aspergillus fumigatus – sustained antifungals only if declining pulmonary status and chronic complex
o Burkholderia cepacian – IV Antibiotic eradication, seek microbiology advice
o H.influenzae – Oral antibiotic (IV if unwell)
o S.Aureus - Oral flucloxacillin prophylaxis needed
o Pseudomonas aeruginosa – oral/IV Abx then sustained inhaled Abx (colistimethate sodium)
Management of cystic fibrosis - nutrition?
o Increase calorie intake by increasing portion size/high calorie foods (150% of normal)
If not adequate – oral nutritional support or enteral feeding
o Fat-soluble vitamins - ADEK
Management of cystic fibrosis - pancreatic insufficiency?
o Test with stool elastase estimation
o Offer oral pancreatic enzyme replacement therapy (Pancreatin)
o Omeprazole helps absorption
Management of cystic fibrosis - distal intestinal obstruction syndrome?
o Manage in specialist CF centre
o Oral or IV fluids offered to ensure adequate hydration
o Meglumine amidotrizoate with sodium amidotrizoate solution (oral or via enteral tube)
o 2nd line – polyethylene glycol orally or via enteral tube
o Surgery is last resort if prolonged treatment with polyethylene glycol solution not effective
Management of cystic fibrosis - abnormal LFTs?
o Liver US and ursodeoxycholic acid
Management of cystic fibrosis - diabetes?
o Test annually from 10 years old with CGM, serial glucose testing over days or OGTT
Management of cystic fibrosis - osteoporosis?
o DEXA scan if high-risk – long-term steroids, lung disease, frequent IV Abx, undernutrition, post-menopause
Complications of CF?
- Underweight
- Meconium ileus (1 in 7)
- Fat-soluable deficiencies (ADEK)
- Male infertility
- Reduced femal fertility
- Nasal polyps and sinusitis
- Chronic liver disease
- Diabetes
- Osteoporosis
Epidemiology of inhaled foreign body?
- 75% occur in children <3
- Higher risk in learning difficulties, swallowing difficulties and boys
Risk factors of inhaled foreign body?
o Decreased consciousness o <4 or >70 o Male o Cerebrovascular disease o Dementia
Symptoms of inhaled foreign body?
o Unilateral wheeze suggest partial obstruction of main or distal bronchi
Signs of inhaled foreign body?
o Unilateral wheeze suggest partial obstruction of main or distal bronchi
Investigations of inhaled foreign body?
- CXR o Can only see radio-opaque foreign bodies - Flexible bronchoscopy o Removal - If fails, rigid bronchoscopy - CT scan
Management of inhaled foreign body - mild?
o Encourage coughing
Management of inhaled foreign body - severe?
Encourage coughing Lean patient forwards External Manoeuvres Infants (<1 years old) • Back blows – 5 to interscapular region • Chest thrusts – like chest compressions during CPR • Check in mouth and see if removable • If not – alternate two manoeuvres Adults and children - 5 BB then 5 ATs • Back blows – to interscapular region with heel of hand • Abdominal thrusts – stand behind, put both arms around upper abdomen, clench one fist and grasp with other, pull sharply inwards and upwards If unconscious: • Lower patient to floor • Call ambulance • Begin CPR o Removal with flexible/rigid bronchoscopy or surgery
Complications of inhaled foreign body?
- Pneumonia
- Atelectasis
- Asphyxia
- Pneumomediastinum
- Bronchospasm
- Bronchiectasis
Definition of tuberculosis?
An infection caused by bacteria of the Mycobacterium tuberculosis complex, predominantly Mycobacterium tuberculosis and more rarely Mycobacterium
- bovis
Spread of tuberculosis?
- Spread via respiratory route
Definition of active disease of tuberculosis?
o Symptomatic or progressive disease in organs (pulmonary most common), can involve CNS, lymph nodes, bones, joints, GI, GU, skin
Definition of latent disease of tuberculosis?
o Persistent immune response with no evidence of active TB (asymptomatic and not infectious)
Epidemiology of tuberculosis?
- Declining incidence and mortality in developed country
- 10 million people developed it in world
- Common in India, Pakistan, Romania, Bangladesh and Somalia
Risk factors of tuberculosis?
o Being born in high prevalence areas o Diabetes, HIV, chronic kidney disease o Close-proximity o Immunodeficiency o Deprived social areas
Symptoms of tuberculosis?
o The majority of patients are asymptomatic, and disease remains latent
o Lung lesion plus spread via lymph system called Ghon complex
o Fever, night sweats
o Anorexia and weight loss
o Cough
Productive, with SOB, haemoptysis
Extrapulmonary symptoms of tuberculosis?
Lymphadenopathy (painless swelling with rubbery texture)
Arthralgia, back pain
Abdominal or pelvic pain, constipation, bowel obstruction
Sterile pyuria
Headache, vomiting
Signs of tuberculosis?
o Failure to thrive, anorexia
o May affect gut, skin and lymph nodes and caseate forming a cold abscess
Abdo pain, bowel obstruction, sterile pyuria, headache
Investigations of tuberculosis - primary care - latent TB?
Mantoux test
• Purified protein derivative of tuberculin intradermal injection
• Positive test indicates infection or previous BCG vaccine
If positive – consider Interferon-gamma test (Quantiferon TB Gold)
Investigations of tuberculosis - primary care - active TB?
Chest X-ray
• Consolidation, cavities, miliary spread (fine white dots), calcification
• Hilar lymphadenopathy
Three sputum samples for microscopy, culture & sensitivity for Acid-Fast Bacilli on Ziehl-Neelsen Stain
• If unable to perform, gastric washing on three consecutive mornings required to culture acid-fast bacilli
Investigations of tuberculosis - primary care - active non-respiratory TB?
CXR
Samples – sputum, pleura, pleural fluid, urine, ascites, bone marrow, lymph node excision, CSF
Screening of TB - when?
People in contact with active pulmonary or laryngeal TB (household members, close contacts)
Immunocompromised
New entrants to UK from high prevalence countries
New NHS employees working with patients and clinical specimens
Screening of TB - What?
Mantoux Test
• Tuberculin skin test infected intradermally, inspected for signs of local skin reaction after 2-3 days
• Positive if >5mm or more
Interferon gamma release assay (Quantiferon TB Gold)
• Blood test detecting WBC response to TB antigens
If positive - CXR
Management of latent TB infection?
o Offer HIV, HepB, HepC testing before treatment
Medical therapy
3 months isoniazid (with pyridoxine) with rifampicin OR
6 months of isoniazid (with pyridoxine)
Management of active TB infection - referral?
o Refer to specialist if suspected TB or know TB not completed treatment as planned
CXR
Sputum sample for microscopy culture and histology
• 3 spontaneously produced deep-cough sputum samples, preferably 1 EMU
• If not – induced sputum or bronchoscopy and lavage
Management of active TB infection - assessment in hospital?
NAAT test for Mycobacterium tuberculosis
Other sampling techniques if needed (sputum induction, bronchoscopy and lavage, gastric aspiration, CT)
Biopsy and needle aspiration – DO NOT PLACE IN FORMALIN WHEN SENDING FOR CULTURE (JUST IN SALINE)
Management of active TB infection - specialist management - baseline bloods?
Encourage adherence to treatment
Stop smoking
FBC, U&E, LFTs
Management of active TB infection - specialist management - drug therapy?
o 2 months Rifampicin Isoniazid (with pyridoxine) Pyrazinamide Ethambutol o After 2 months – 6 months Rifampicin Isoniazid (with pyridoxine) • If active CNS TB – 10 months
Regimen of rifampicin in TB?
o 600-900mg PO 3x/week
o SE – LFT rise (stop if bilirubin rise), platelets low, orange discolouration to urine, tears and contact lenses, inactivation of pill
o Reduce dose if eGFR<50
Regimen of isoniazid in TB?
o 15mg/kg PO 3x/week
o Pyridoxine given to prevent neuropathy
o Avoid tyramine rich food (cheese, salami, pickled herring, Bovril, Marmite)
o SE – LFT rise, WCC low, neuropathy
Regimen of pyrazinamide in TB?
o 2.5g PO 3x/week
o SE – hepatitis, arthralgia (CI: acute gout)
Regimen of ethambutol in TB?
o 30mg/kg PO 3x/week
o SE - Reversible ocular toxicity (colour vision and acuity problems)
Management of active TB infection - specialist management - drug therapy if multiple drug resistent TB?
• Need prolonged treatment as risk of multiple drug resistance 18-24 months with at least 6 drugs
Prevention of TB - what and when is given?
Vaccination BCG
Neonates – in increased risk of TB before discharge from hospital
• If born in area with high incidence of TB, 1 or more parents or grandparents born in high-incidence country or FHx of TB within 5 years
Healthcare workers
Close Contacts if Mantoux negative:
• Not been vaccinated (no documentation or scar)
• <35 or >35 and healthcare workers with contact with patients/materials
Complications of TB?
o Transmission to others
o Cor pulmonale, bronchiectasis
o Death
o Drug Resistance
Definition of obstructive sleep apnoea?
- Intermittent closure/collapse of pharyngeal airway causing apnoeic episodes during sleep
- These are terminated by partial arousal – leading to light sleep
- Cycle repeats itself many time throughout night
Epidemiology of obstructive sleep apnoea?
- Up to 3% of people
Risk factors of obstructive sleep apnoea?
o Obese o Male o Middle-aged o Neck circumference >43cm o FHx o Smoking o Alcohol before bed o Sleeping supine o Hypothyroidism
Risk factors in children of obstructive sleep apnoea??
o Adenotonsillar hypertrophy
o Obese
o Down’s syndrome, neuromuscular disease, Prader-Willi syndrome
Symptoms of obstructive sleep apnoea?
o Loud snoring o Daytime somnolence o Poor sleep quality – feeling unrefreshed on waking o Morning headache o Decreased libido o Cognitive performance decreased
Signs of obstructive sleep apnoea?
o Enlarged tonsils
o Nasal blockage
o Signs of COPD, respiratory failure, cor pulmonale
Investigations of obstructive sleep apnoea?
- Epworth sleepiness questionnaire – assess severity of symptoms
o Refer if >10
Management of obstructive sleep apnoea - general measures?
o Weight reduction
o Avoid tobacco and alcohol
o Sleep on side
Management of obstructive sleep apnoea - driving advice?
Continue to drive:
• If investigated for, or diagnosed but no symptoms of daytime sleepiness
Inform DVLA but continue to drive if:
• Using CPAP or intra-oral device – if compliant and symptoms controlled
Stop Driving:
• Diagnosed and symptoms impair driving
Management of obstructive sleep apnoea - referral?
o To sleep centre – confirm diagnosis using sleep studies/secondary care treatments
Urgently if hazardous occupation, driver, signs of respiratory/heart failure, COPD
Routinely if Epworth scale >10
Management of obstructive sleep apnoea - specialist investigations?
Polysomnography – records sleep and breathing patterns
Limited sleep study – at person home or sleep centre
Diagnosed if >5 episodes of apnoea per hour sleep
Severity:
• Mild 5-14, Moderate 15-30, Severe >30
Management of obstructive sleep apnoea - non-surgical management?
CPAP via nasal mask during sleep (moderate to severe disease)
• Lifelong
Intra-oral devices (mandibular advancement)
o If mild with normal daytime alertness
Management of obstructive sleep apnoea - surgical management?
o Surgical relief of pharyngeal obstruction – usually in children
Tonsillectomy, uvulopalatopharyngoplasty, tracheostomy
Complications of obstructive sleep apnoea?
o Pulmonary hypertension o Type 2 respiratory failure o Stroke o RTAs o Hypertension
