Rheum

Question 1

What is rheumatoid arthritis?

Answer 1

common chronic inflammatory arthritis
autoimmune

Question 2

What are the major clinical features of rheumatoid arthritis?

Answer 2

MSK - articular stiffness and swelling including C-spine instability
Lungs – dyspnoea – diffuse interstitial fibrosis or effusion
Heart – chest pain from pericarditis

Question 3

What are the clinical features on exam of rheumatoid arthritis?

Answer 3

o Hands – symmetrical small joint synovitis
o Neck – cervical spine tenderness/↓rotational movement (atlanto-axial instability affects 25%)
o Lung – effusion, fibrosis, infection, nodules
o Heart – pericardial rub (pericarditis), diastolic murmur (AR)

Question 4

What Ix are useful in rheumatoid arthritis?

Answer 4

o Cervical spine x-rays - Distance between posterior aspect of C1 arch & anterior aspect of odontoid peg should be no more
than 3mm in adults
o Bloods – Hb, platelets
o CXR
o ECG
o Echocardiogram – pericardial effusion, AR

Question 5

What are the anaesthetic issues in rheumatoid arthritis?

Answer 5

• Resp disease - restrictive lung disease
• Cardiac disease incl pulm HTN secondary to pulmonary fibrosis
• Atlanto-axial instability
• Steroid dependence and need for supplementation

Question 6

What is Ankylosing Spondylitis?

Answer 6

autoimmune non-rheumatic arthropathy

Characterised by:
o Involvement of spine, especially sacroiliac joints
o Asymmetrical peripheral arthritis & synovitis
o Absence of rheumatoid nodules or detectable circulating rheumatoid factor

Question 7

What are clinical features on history of Ankylosing Spondylitis?

Answer 7

o Persistent pain & morning stiffness (worse at rest but improves with
exercise) in lower spine + sacroiliac joints
o Visual impairment, photophobia, eye pain
o Fatigue, weight loss, low-grade fever

Question 8

What are clinical features on exam of Ankylosing Spondylitis?

Answer 8

o Back + sacroiliac joints:
- Loss of lumbar lordosis + thoracic kyphosis
- Severe flexion deformity of lumbar spine

o Lungs: decreased chest expansion (<5cm), upper lobe fibrosis
o Heart: signs of aortic regurgitation (1%)

Question 9

What are Ix for Ankylosing Spondylitis?

Answer 9

o FBC – mild normochromic anaemia
o Spine x-ray – bamboo spine, erosion of sacroiliac joints
o ECG – conduction defects
o Echocardiography – aortic regurgitation
o RFTs – restrictive defect

Question 10

What are the anaesthetic issues with Ankylosing Spondylitis?

Answer 10

• Potentially difficult airway
  o Limited mouth opening with temporomandibular joint involvement → difficult laryngoscopy & LMA insertion
  o Stiff & deformed spinal column prevents appropriate cervical spine motion for intubation → need fiberoptic or video laryngoscope assistance
  o Excessive neck extension in patients with chronic cervical kyphosis can cause vertebrobasilar insufficiency & neurological injury
  o AFOI – safest option
• Restrictive lung disease from costochondral rigidity & fixed flexion deformity of thoracic spine
• Technically difficult neuraxial blockade – use paramedian approach
• Aortic regurgitation – avoid sudden increases in SVR

Question 11

What is scleroderma?

Answer 11

connective tissue disease associated with excess production & deposition of collagen, glycosaminoglycans + fibronectins within connective tissues

Question 12

What are some of the clinical features of scleroderma?

Answer 12

o Oesophageal hypomotility
o Prominent skin sclerosis - limited mouth opening and neck movement
o Pulm Fibrosis
o Pulm HTN (if severe)
o Renal impairment
o Pericarditis
o Dilated cardiomyopathy

Question 13

What are some features on exam of scleroderma?

Answer 13

o General appearance – ‘bird-like’ facies, weight loss
o Hands - Calcinosis, atrophy distal tissue pulp (Raynaud’s), sclerodactyly,
telangiectasia
o Head - Alopecia, reduced opening mouth
o Heart – pulmonary hypertension, pericarditis, heart failure
o Lungs – fibrosis

Question 14

What are some Ix for scleroderma?

Answer 14

o Blood – ANA positive (40%)
o ECG – look for signs of pericarditis, arrhythmias or conduction disturbances
o CT chest – pulmonary fibrosis
o Echocardiogram – pericardial effusion indicated by presence of echolucent area
adjacent to cardiac structures, abnormal right heart chamber size + motion
indicates pulmonary hypertension, note RVSP
o RFTs – isolated low DLCO + reduced lung volume are common early, abnormal results likely due to
interstitial fibrosis or pulmonary hypertension due to vasculopathy

Question 15

What are some anaesthetic issues with scleroderma?

Answer 15

o Difficult peripheral IV access
o Difficult airway management
o Aspiration risk due to oesophageal dysmotility
o Restrictive lung disease
o Myocardial fibrosis - risk of cardiac failure, conduction defects
o Renal impairment
o Raynaud’s - avoid vasoconstrictors and if severe avoid radial artery for art line

Question 16

Whats SLE?

Answer 16

Chronic multisystem inflammatory disease characterised by autoantibody production

Question 17

What are some clinical features of SLE?

Answer 17

o Cardiovascular – conduction abnormalities, non-infective endocarditis, accelerated
atherosclerosis + CAD, myocarditis, pericarditis, heart failure
o Respiratory – pleuritis, pleural effusions, pneumonitis, restrictive lung disease, pulmonary hypertension
o Renal – nephritis, oedema, dialysis
o CNS – psychosis, seizures, peripheral neuropathy
o Hematological – anaemia, leucopenia, thrombocytopenia, thrombosis

Question 18

What are some clinical features on exam of SLE?

Answer 18

o General inspection – Cushingoid appearance, weight, mental state
o Chest – pericarditis, pleural effusion, pleurisy, pulmonary fibrosis, collapse or infection

Question 19

What Ix for SLE?

Answer 19

o Bloods – electrolytes, urea, Cr, Hb, WCC, platelets, APTT
o Antibodies - Anti-double-stranded DNA (anti-dsDNA) is highly specific (70% positive), Anti-nuclear antibody (ANA)
o ECG – silent ischaemia, conduction abnormalities
o Echocardiography – valvular dysfunction, Libman-Sacks endocarditis
o CXR – pleural effusion, pneumonitis, subglottic stenosis
o RFTs

Question 20

What are some anaesthetic issues for SLE?

Answer 20

• Cardiac, resp or renal impairment
• Haematologic – severe thrombocytopenia
• Need for steroid replacement
• Risk of atlanto-axial instability
• Careful positioning

Question 21

What is Marfan’s syndrome?

Answer 21

Autosomal dominant, multisystem connective tissue disease

Question 22

What are some clinical features of marfan’s syndrome?

Answer 22

Cardiac - dilated aorta, valvular heart disease (MR from MVP, AR)

Resp - pneumothorax, Restrictive defect due to pectus excavatum/kyphoscoliosis, Emphysema, bronchogenic cysts & honeycomb lung lead to pneumothorax

Skeletal – high arched palate

Question 23

What are some clinical features on exam of marfan’s syndrome?

Answer 23

o Cardiovascular - Aortic dilation, dissection or rupture with associated AR
- Valve dysfunction/prolapse – MR resulting from MVP is common, look for AR
- Heart failure, pulmonary hypertension
- Conduction abnormalities

o Respiratory
- Restrictive defect due to pectus excavatum/kyphoscoliosis
- Emphysema, bronchogenic cysts & honeycomb lung lead to pneumothorax

o Skeletal – high arched palate, long tubular bones → tall stature, pectus excavatum, kyphoscoliosis, joint hyperextensibility

Question 24

What are some Ix of marfan’s syndrome?

Answer 24

o ECG – bundle branch block
o Echocardiography – MVP, MR, tricuspid valve prolapse, AR, dilatation of aortic root
o CXR – apical bullae, pneumothorax, cardiomegaly, pulmonary oedema
o TOE, chest CT/MRI or aortography for suspected dissection

Question 25

What are some anaesthetic issues with Marfan’s syndrome?

Answer 25

• cardiorespiratory abnormalities - aortic dilatation, valve dysfunction, conduction abnormalities, pulm HTN, restrictive lung defect
• Proper positioning & limb support to avoid joint trauma/dislocation
• Prepare for a potentially difficult intubation due to high arched palate, jaw protrusion, teeth crowding & cervical spine bony/ligamentous abnormalities

Question 26

What is Ehler’s-Danlos Syndrome?

Answer 26

Heterogeneous group of inherited connective tissue disorders characterized by joint hyperlaxity, skin hyperextendability & tissue fragility

generally abnormal collagen synthesis resulting in reduced strength of collagen in numerous tissues

Six major types, with EDS types I (classic) + III (hypermobility) accounting for 90% of all cases

Question 27

What are the clinical features of Ehler’s-Danlos Syndrome?

Answer 27

o Joint hypermobility
o Skin hyperelasticity, fragility + bruising
o Impaired wound healing & inappropriate scarring
o multiple aneurysms (rarely of aorta) & rupture + dissection of arteries,

Question 28

What are anaesthetic issues with Ehler’s-Danlos Syndrome?

Answer 28

o Careful positioning to prevent damage to delicate skin & avoid joint dislocations
o Adequate venous access to cope with potential heavy blood loss
o Local anesthesia may have an insufficient effect
o Avoid TMJ dislocation/subluxation & cervical spine subluxation
o Peak airway pressure must be kept as low as possible to reduce risk of pneumothorax
o Avoid wide swings in BP to reduce risk of hemorrhage & aneurysm rupture

Question 29

What is sarcoidosis?

Answer 29

Chronic multisystem granulomatous disease characterised histologically by presence of nonspecific, non-caseating granulomas with unknown aetiology

Question 30

What are clinical features of sarcoidosis?

Answer 30

Lung – airway granulomas, restrictive (+/- obstructive) disease, alveolar fibrosis, pulmonary hypertension

Heart – heart block, pericardial disease, restrictive cardiomyopathy

Renal impairment

Spleen + Liver – splenomegaly, hepatomegaly, progressive liver disease leading to portal hypertension

Question 31

What are anaesthetic issues of sarcoidosis?

Answer 31

• Avoid GA & use local/regional where possible if impaired respiratory function
  o Tolerate apnoeic episodes poorly due to ↓FRC & low O2 stores – GA, supine position + mechanical ventilation further ↓FRC
  o Poor lung compliance needs ETT & ventilation with low airway pressure to minimise barotrauma
• Appropriate steroid cover if needed