Respiratory - Level 2.2 Flashcards

1
Q

Definition of sarcoidosis?

A

o Multisystem inflammatory chronic condition characterised by non-caseating epithelioid granulomata

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2
Q

Epidemiology of sarcoidosis?

A
  • Prevalence highest in Northern Europe, 20-40 years old, Afro-Caribbeans
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3
Q

Symptoms of sarcoidosis - pulmonary disease?

A

o Bilateral hilar lymphadenopathy, pulmonary infiltrates or fibrosis
o Dry cough, progressive dyspnoea, decreased exercise tolerance and chest pain

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4
Q

Symptoms of sarcoidosis - skin?

A

 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)

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5
Q

Symptoms of sarcoidosis - eye?

A
	Granulomatous uveitis
•	Anterior & posterior
	Keratoconjunctivitis
	Dry eyes
	Glaucoma
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6
Q

Symptoms of sarcoidosis - neurological?

A
	Bell’s palsy
	Lymphocytic meningitis
	Diabetes insipidus
	Neuropathy – numbness, weakness
	Pituitary dysfunction
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7
Q

Symptoms of sarcoidosis - CV?

A

 Arrhythmias or heart failure in young patient – cardiomyopathy

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8
Q

Symptoms of sarcoidosis - others?

A
	Lymphadenopathy
	Hepatomegaly, splenomegaly
	Hypercalcaemia and hypercalciuria, renal stones
	Bone marrow failure
	Lacrimal and parotid gland enlargement
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9
Q

Investigations in sarcoidosis?

A
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

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10
Q

Imaging of sarcoidosis

A

 CXR
• Bilateral hilar lymphadenopathy
 CT

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11
Q

Further testing of sarcoidosis

A
o	Lung function tests – restrictive pattern
o	ECG (arrhythmias or BBB)
o	Bronchoalveolar Lavage
	Raised lymphocytes, raised neutrophils with pulmonary fibrosis
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12
Q

Diagnostic testing of sarcoidosis

A

o Tissue Biopsy
 Lung, liver, lymph nodes, skin
 Non-caseating granulomas

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13
Q

Staging of sarcoidosis of CXR?

A

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)

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14
Q

Management of sarcoidosis - acute sarcoidosis?

A

o Bed rest
o NSAIDs
o Remission is common
o If severe – oral prednisolone or IV Methylprednisolone

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15
Q

Management of sarcoidosis - chronic sarcoidosis - immunosuppression?

A

 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

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16
Q

Management of sarcoidosis - chronic sarcoidosis - other therapies?

A
  • Methotrexate/azathioprine/hydroxychloroquine /ciclosporin/cyclophosphamide
  • Anti-TNF alpha
  • Rituximab
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17
Q

Management of sarcoidosis - chronic sarcoidosis - lung transplant?

A

 Lung Transplant

• End stage lung disease

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18
Q

Management of sarcoidosis - chronic sarcoidosis - specific eye and skin features?

A

 Eye – Topical prednisolone

 Skin – Topical Triamcinolone or oral prednisolone

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19
Q

Management of sarcoidosis - chronic sarcoidosis - monitoring?

A
o	Follow up every 3-6 months
o	Monitoring
	FBC
	DEXA every 2 years
	Serial lung function tests, CXR and ACE levels
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20
Q

Complications of sarcoidosis?

A
  • 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
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21
Q

Prognosis of sarcoidosis?

A
  • 60% with thoracic sarcoidosis resolve over 2 years
  • 20% respond to steroids
    o If not, improvement unlikely
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22
Q

Definition of cystic fibrosis?

A
  • Autosomal recessive condition causing a defective CFTR protein on chromosome 7
  • CFTR is a cAMP dependent chloride channel found in membrane of cells
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23
Q

Pathology of cystic fibrosis?

A
  • 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
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24
Q

Epidemiology of cystic fibrosis?

A
  • Incidence of 1 in 2500 live births with a carrier rate of 1 in 25
  • Average life expectancy is mid-30s
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25
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
26
Symptoms of young child of cystic fibrosis?
- Bronchiectasis - Rectal Prolapse - Nasal Polyp - Sinusitis
27
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
28
Signs of cystic fibrosis?
- Evidence of malnutrition, poor weight gain - Hyperinflation of the chest - Coarse inspiratory crackles - Expiratory wheeze - Finger clubbing
29
Testing of cystic fibrosis - in newborns?
- Screened for in Guthrie test
30
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
31
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)
32
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
33
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 ```
34
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
35
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
36
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
37
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
38
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)
39
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
40
Management of cystic fibrosis - pancreatic insufficiency?
o Test with stool elastase estimation o Offer oral pancreatic enzyme replacement therapy (Pancreatin) o Omeprazole helps absorption
41
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
42
Management of cystic fibrosis - abnormal LFTs?
o Liver US and ursodeoxycholic acid
43
Management of cystic fibrosis - diabetes?
o Test annually from 10 years old with CGM, serial glucose testing over days or OGTT
44
Management of cystic fibrosis - osteoporosis?
o DEXA scan if high-risk – long-term steroids, lung disease, frequent IV Abx, undernutrition, post-menopause
45
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
46
Epidemiology of inhaled foreign body?
- 75% occur in children <3 | - Higher risk in learning difficulties, swallowing difficulties and boys
47
Risk factors of inhaled foreign body?
``` o Decreased consciousness o <4 or >70 o Male o Cerebrovascular disease o Dementia ```
48
Symptoms of inhaled foreign body?
o Unilateral wheeze suggest partial obstruction of main or distal bronchi
49
Signs of inhaled foreign body?
o Unilateral wheeze suggest partial obstruction of main or distal bronchi
50
Investigations of inhaled foreign body?
``` - CXR o Can only see radio-opaque foreign bodies - Flexible bronchoscopy o Removal - If fails, rigid bronchoscopy - CT scan ```
51
Management of inhaled foreign body - mild?
o Encourage coughing
52
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 ```
53
Complications of inhaled foreign body?
- Pneumonia - Atelectasis - Asphyxia - Pneumomediastinum - Bronchospasm - Bronchiectasis
54
Definition of tuberculosis?
An infection caused by bacteria of the Mycobacterium tuberculosis complex, predominantly Mycobacterium tuberculosis and more rarely Mycobacterium - bovis
55
Spread of tuberculosis?
- Spread via respiratory route
56
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
57
Definition of latent disease of tuberculosis?
o Persistent immune response with no evidence of active TB (asymptomatic and not infectious)
58
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
59
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 ```
60
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
61
Extrapulmonary symptoms of tuberculosis?
 Lymphadenopathy (painless swelling with rubbery texture)  Arthralgia, back pain  Abdominal or pelvic pain, constipation, bowel obstruction  Sterile pyuria  Headache, vomiting
62
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
63
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)
64
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
65
Investigations of tuberculosis - primary care - active non-respiratory TB?
 CXR |  Samples – sputum, pleura, pleural fluid, urine, ascites, bone marrow, lymph node excision, CSF
66
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
67
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
68
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)
69
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
70
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)
71
Management of active TB infection - specialist management - baseline bloods?
 Encourage adherence to treatment  Stop smoking  FBC, U&E, LFTs
72
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 ```
73
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
74
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
75
Regimen of pyrazinamide in TB?
o 2.5g PO 3x/week | o SE – hepatitis, arthralgia (CI: acute gout)
76
Regimen of ethambutol in TB?
o 30mg/kg PO 3x/week | o SE - Reversible ocular toxicity (colour vision and acuity problems)
77
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
78
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
79
Complications of TB?
o Transmission to others o Cor pulmonale, bronchiectasis o Death o Drug Resistance
80
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
81
Epidemiology of obstructive sleep apnoea?
- Up to 3% of people
82
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 ```
83
Risk factors in children of obstructive sleep apnoea??
o Adenotonsillar hypertrophy o Obese o Down’s syndrome, neuromuscular disease, Prader-Willi syndrome
84
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 ```
85
Signs of obstructive sleep apnoea?
o Enlarged tonsils o Nasal blockage o Signs of COPD, respiratory failure, cor pulmonale
86
Investigations of obstructive sleep apnoea?
- Epworth sleepiness questionnaire – assess severity of symptoms o Refer if >10
87
Management of obstructive sleep apnoea - general measures?
o Weight reduction o Avoid tobacco and alcohol o Sleep on side
88
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
89
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
90
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
91
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
92
Management of obstructive sleep apnoea - surgical management?
o Surgical relief of pharyngeal obstruction – usually in children  Tonsillectomy, uvulopalatopharyngoplasty, tracheostomy
93
Complications of obstructive sleep apnoea?
``` o Pulmonary hypertension o Type 2 respiratory failure o Stroke o RTAs o Hypertension ```