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Leila PACES > RESP ILD/CF/BRONCHIECTASIS/COPD/ASTHMA > Flashcards

RESP ILD/CF/BRONCHIECTASIS/COPD/ASTHMA Flashcards

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1
Q

Significant negatives when presenting an ILD case

A
  • Signs of aetiology
  • Resp distress
  • Evidence of pulmonary HTN
  • Complications of immunosuppression
  • Scars (surgical/ radiotherapy)
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2
Q

Causes of pulmonary fibrosis

A

Upper zone:
TB
Ank Spond/ psoriasis (sernonegative)/ ABPA
Pneumoconiosis (sillicosis, coal workers lung, berylliosis)
EAA (bird fancier’s, mushroom worker etc)

Mid zone:
sarcoid

Lower zone:
Toxins/drugs: amiodarone/NF/MTX/chemo
Asbestosis
IPF
Rheumatological/autoimmune (RA, SLE, DM/PM, Scleroderma, CTDs)
Vasculitis

Other systemic: NF, Tuberous sclerosis

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3
Q

DDx clubbing + creps

A

ILD
Bronchiectasis
CF
Lung abscess
Lung ca

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4
Q

DDx creps

A

ILD
Bronchiectasis
Pneumonia
Pulmonary oedema

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5
Q

Unilateral fine crackles and contralateral thoracotomy scar with normal breath sounds

A

Single lung tx in patient with pulmonary fibrosis

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6
Q

Ix in suspected pulmonary fibrosis

A

Bedside
Obs
Sputum MCS
ABG (T1RF)
Pulmonary function tests
ECG - Signs of right heart strain

Bloods
CRP, ESR, Rf, ANA, ANCA, anti-dsDNA, ACE, Serum precipitins (for EAA)

Imaging
CXR
HRCT

Special tests
Spiro
BAL
Lung biopsy
Echo

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7
Q

Why do BAL in iLD?

A

determine if any infection prior to starting immunosuppression

If lymphocytosis then indicates a better response to steroids and a better prognosis

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8
Q

Lung function test results in pt with iLD

A

FEV1/FVC > 0.8 (restrictive)
Low TLC
Reduced transfer factor

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9
Q

ILD + Pacemaker

A

Amiodarone

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10
Q

Prognosis of ILD

A

Depends on aetiology
If immunosuppression responsive then 80% 5 year survival
If honeycombing on cT and no response to immunosuppression then 80% 5 year mortality

High risk of bronchogenic carcinoma

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11
Q

What did the PANTHER study reveal

A

That there is increased mortality when combining steroids and azathioprine - no longer used

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12
Q

Mx of iLD

A

MDT (Referral to ILD service), Resp, resp physio, resp CNS, dietitian, PT/OT, GP, palliative care

Conservative
Smoking cessation
Vaccination (5 yearly pneumococcal, annual influenza)
Stop offending agent if occupational
Resp physio/ pulm rehab

Medical
LTOT
Pirfenidone/nintedanib
Steroids depending on aetiology
Mx of any R sided heart failure

Surgical
Lung tx (single or double)

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13
Q

Lymphadenopathy in ILD pt

A

Sarcoidosis
Bronchogenic carcinoma

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14
Q

LTOT criteria

A

Pao2 < 7.3kPa or <8Kpa if established cor pulmonale

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15
Q

Causes of restrictive lung defect

A

Pulmonary fibrosis
Kyphoscoliosis
Obesity hypoventilation syndrome
Neuromuscular disorders

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16
Q

Causes of obstructive lung disease

A

COPD
Asthma
Bronchiectasis
Bronchiolitis obliterans

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17
Q

Right ventricular hypertrophy finding on ECG

A

Prominent R wave in V1

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18
Q

Asymmetric causes of lung fibrosis

A

Old TB (treated - thoracotomy or phrenic nerve crush)
Lung malignancy (radiotherapy marks/ lymphadenopathy/ small muscle wasting of hands)

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19
Q

5 manifestations of rheumatoid lung

A
  1. Pleural effusions
  2. Pulmonary nodules
  3. Fibrosis
  4. Caplan syndrome
  5. obliterative bronchiolitis
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20
Q

What is Caplan syndrome

A

Coal worker pneumoconioses + rheumatoid arthritis

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21
Q

Why may you get a hoarse voice in pulmonary HTN?

A

Compression of left RLN by dilated main pulmonary artery

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22
Q

What features of JVP seen in tR?

A

Giant V waves due to increased atrial filling volumes

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23
Q

Gold standard diagnosis of pulm HTN

A

Right heart catheterisation

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24
Q

Definition of pulmonary HTN

A

Pulmonary artery pressures > 25mmHg

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25
Clinical signs of pulmonary HTN
Flapping tremor of CO2 retention, bounding pulse Polcythaemia and plethoric face Raised JVP with prominent V waves of TR Left parasternal heave Palpable P2 Wide splitting of S2 Loud P2 PSM at LLSE loudest on inspiration EDM in pulmonary area on inspiration (Graham Steelle - functional PR) Hepatomegaly Sacral and ankle oedema
26
ECG changes in pulm HTN
RAD Prominent P waves (p pulmonale) RBBB Prominent R wave in V1I
27
Ix in pulm HTN
ABG ECG CXR Echo (peak tricuspid regurgitant velocity estimates systolic pulmonary resistance) Right heart catheterisation CTPA or V/Q scan - looking for chronic thrombi US abdomen - portal HTN
28
Common causes of cor pulmonale
COPD ILD OSA Hypoventilation disorders i.e. OSA, OHS, neuromuscular disorders, kyphoscoliosis
29
Why may a patient with pulmonary HTN have a central tunnelled catheter or a hand held nebulizeR?
Administration of continuous IV epoprostenol and nebuliser for iloprost
30
Complications of pulmonary HTN
Death from right heart failure Arrhythmias Dilated main pulmonary artery - hoarse voice but can also cause external compression of left anterior descending artery -> angina/ventricular arrhythmias, can also compress left main bronchus causing localised bronchiectasis
31
Who is pulmonary hypertension classified
using WHO clinical classification
32
WHO clinical classification of pulmonary HTN (groups 1-5)
Group 1: Pulmonary arterial HTN (idiopathic, drugs and toxins, CTDs, congenital heart disease, portal HTN) Group 2: Left heart disease Group 3: lung disease (COPD, ILD, OHS, Sarcoid) Group 4: CTEPH Group 5: miscellaneous
33
Treatment of Group 1 PH
Pulnonary vasodilators: Phosphoesterase 5 inhibitors (sildenafil) Endothelin receptor antagonists Prostacyclin analogues (epoprostenol) Double lung transplant
34
Treatment of group 4 PH
Lifelong anticoagulation, pulmonary endarterectomy
35
What test is important in young female pts with pH
Pregnancy test (v high mortakity)
36
Loud s1 in in a pt with PH
Mitral stenosis
37
Causes of PH
Primary Secondary - Congenital heart disease (intracardiac shunts: ASD, VSD, PDA) - Left sided heart disease (MS, MR, AS, AR) - Chronic hypoxia CTEPH, COPD, OSA, ILD, bronchiectasis
38
Bronchiectasis peripheral inspection
Sputum pot Oxygen Nebuliser Hickmann/PICC Line Airway clearance device Cachectic? Tachypnoeic Clubbed? Yellow nails? Young female? (CF)
39
What to look out for on examination of the chest of a patient with suspected bronchiectasis
Hickmann line Thoracotomy scars/ chest drain scars Lymphadenopathy ?DEXTROCARDIA ?Cor Pulmnale Coarse wet crackles which can vary with coughing
40
What may localised vs diffuse crackles suggest in a bronchiectasis patient?
Localised: post-infecitous ie TB or foreign body Diffuse - systemic disease
41
Where else may you want to examine in suspected broncheictasis patient?
Nose for polyps Abdomen ?situs invertus or any scars from IBD, hepatosplenomegaly from amyloidosis
42
DDx bronchiectasis
ILD with infection Lung abscess Lung malignancy
43
features of resp failure
tachypnoea cyanosis oxygem requirement co2 retention flap
44
why may a bronchiectasis patient have hepatosplenomegaly
Can cause secondary amyloidosis
45
percussion note and vocal fremitus in bronchiectasis
usually normal
46
causes of bronchiectasis
Idiopathic Inherited: CF, PCD/Kartagener's, yellow nail syndrome Immunodeficiency: AIDS, hypogammaglobulinaemia Infective: TB, childhood measles/pertussis Post-obstruction: foreign body, lung ca Hypersensitivity: ABPA, IBD, RA
47
What investigations would you do for bronchiectasis?
Bedside Obs (Temp) Sputum -> MCS and AFBs and cytology PEFR ECG ABG Urine dip for proteinuria ?amyloidosis Bloods FBC, UE, LFT, CRP Immune screen: Serum immunoglobulins, ANA, Rf, anti-CCP HIV test Aspergillus Sweat test/genetic screening for cF Nasal biopsy/cilia studies if ?Kartageners Imaging (Diagnose extent and distribution) CXR HRCT Special tests Spirometry bronchoscopy
48
Management of patients with bronchiectasis
MDT: Resp, resp CNS, physio, dietitian, PT/OT, Psychologist, GP Conservative Smoking cessation, annual flu vaccine/ 5 yearly pneumococcal, resp physio and active cycle breathing with postural drainage Medical Mucolytics, hypertonic saline nebs treat current and colonised infections (nebulised, oral or IV) Anti-inflammatories - azithromycin three times/week LTOT Surgical Lobectomy/pneumonectomy Single or double lung transplant Bronchial artery embolisation if massive haemoptysis
49
Complications of bronchiectasis
Pulmonary: Massive haemoptysis Lung infections, abscesses and empyemas Extra pulmonary: cor pulmonale Extra-pulmonary Secondary amyloidosis Anaemia of chronic disease
50
management of yellow nail syndrome
high dose vitamin E
51
DDx yellow nail syndrome
psoriasis fungal nail infection
52
Major pathogens associated with bronchiectasis
Pseudomonas Haemophilus Streptococcus Others S.aures Moraxella ABPA Burkholderia (inCF)
53
general tx for ABPA
Steroids
54
general mx for hypogammaglobulinaemia
IVIg
55
Kartagener's syndrome triad
PCD Situs invertus Chronic sinusitis/bronchiectasis
56
Difference between PCD and karatgener's
Kartagener's is a subset of PCD which is an umbrella term for a set of disorders involving mucociliary clearance
57
PCD inheritance mode
AR
58
CF genetics
Autosomal recessive mutation in CFTR gene (delta F508) on chromosome 7
59
Investigations TO DIAGNOSE CF
Sweat test (chloride and sodium) Genetic testing for common mutations Immune reactive trypsinogen in neonates
60
Investigations in CF
Sputum MCS ABG Obs + temp PEFR ECG Bloods FBC, UE, LFT + bilirubin, CRP, Vitamin D, bone profile, blood glucose, faecal elastase (pancreatic exocrine function), vitamins ADEK Genetic, sweat test CXR HRCT DEXA scan Fertility testing
61
In which conditions can you get false -ve on sweat test
Addison's Hypothyroidism
62
Mean survival in cF
40 years but rising (gene therapy is in development)
63
Poor prognostic factor in CF
Colonisation with burkholderia cepacia
64
CF pathophysiology
Blockage by thickened secretions: Bronchioles -> bronchiectasis pancreatic ducts -> loss of exocrine and endocrine function gut -> DIOS Seminal vesicles -> male infertility fallopian tubes -> reduced female fertility Gall stones + cirrhosis Osteoporosis
65
CF management
MDT Conservative Vaccination Resp physio, active cycle breathing, postural drainage (nebs + mucolytics) Dietary changes Psychological support and genetic counselling Avoid segregation with other CF patients Medical Nebulisers Prophylactic abx Pancrease (Fat soluble vitamin supplements) Mucolytics (DNAse) CFTR modulators - Trikafta Surgery Double lung transplant
66
What is DNAse
A mucolytic agent used in CF patients
67
What is pancrease (CREON)?
Fat soluble vitamin supplements
68
What is Trikafta?
A CFTR gene modulator

Leila PACES (11 decks)

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