FRCA Notes

Systemic Sclerosis (Scleroderma)

This topic presumably falls under 'rheumatological disease' in the curriculum; it is a known viva topic.

Resources

  • Systemic sclerosis is a rare, auto-immune, inflammatory and progressive multisystem connective tissue disease
  • Patients are more likely to need surgery than the baseline population, and more likely to undergo high-risk surgery
  • European prevalence 7 - 33/100,000
  • 4x higher in females
  • Most commonly presents in 3rd - 5th decade of life
  • 10yr survival 66%

  • Female gender
  • African American or Native American ethnicity
  • Previous chemotherapy e.g. bleomycin, taxanes and gemcitabine
  • Previous radiotherapy
  • Exposure to toxins such as:
    • Silica or silicone (e.g. breast implants)
    • Gadolinium
    • Vinyl chloride

  • Probably initiated by environmental triggers in a genetically susceptible individual
  • Complex pathophysiology involving endothelial cells, fibroblasts and circulating immune cells & inflammatory mediators
  • Leads to:
    • Cell-mediated autoimmunity inc. IL-6
    • Small vessel fibroproliferative vasculopathy
    • Fibroblast dysfunction and abnormal collagen deposition

  • There is consequent:
    • Fibrosis of skin and internal organs
    • Ischaemia-reperfusion small-vessel injury

Airway

  • Potential difficult airway owing to:
    • Small mouth
    • Reduced neck mobility
    • Nasal/oral telangiectasia and bleeding

Respiratory

  • Pulmonary fibrosis/ILD (cause of death in 19% of patients)
    • 75% of patients have DLCO <80%
  • Sleep-disordered breathing (31%)
  • Cor pulmonale

Cardiovascular

  • Up to 50% of patients will have cardiac involvement within 5yrs of disease onset
  • Symptomatic cardiac involvement is associated with 75% 5yr mortality
  • Cardiovascular disease accounts for 28% of systemic scleroderma-associated mortality
  • May have normal cardiac investigations but still have occult disease e.g. of the cardiac conduction system
  • Higher risk of perioperative major adverse cardiac events inc. MI
  • Pulmonary hypertension, if present, is often rapidly progressive and causes a tripling of mortality risk
Cardiovascular features of systemic sclerosis
Hypertension
Dyslipidaemia
Arrhythmias
Coronary artery disease
Myocardial ischaemia
Cardiac failure
Pulmonary hypertension (64%)

Neurological

  • Stroke
  • Chronic pain from arthralgia, Raynaud's or back pain
    • Neuropathic in nature in 26%
  • Contractures from dermal thickening
  • Nerve entrapment

Renal

  • Scleroderma renal crisis was previously a major cause of mortality; this is attenuated by ACE-I
  • Associated renal impairment now only present in 6% of cases
  • CKD, if presence, is due to various causes:
    • ANCA-associated vasculitis
    • Isolated reduced GFR
    • Antiphospholipid-associated nephropathy
    • Intra-renal arterial sitffness
    • Proteinuria
    • Penicillamine-associated renal disease
    • NSAID-induced nephropathy
    • Pre-renal disease due to cardiac failure, pulmonary hypertension or hypovolaemia from diuretic use

Gastrointestinal

  • Hypotonic lower oesophageal sphincter
  • GORD
  • Oesophageal dysmotility and dysphagia
  • Gastric antrum vascular ectasia

Haematological

  • Anaemia
    • Of chronic disease
    • Microangiopathic haemolytic anaemia
    • Secondary to occult GI bleeding

  • Threefold increase in VTE risk

Cutaneous

  • Scleroderma (hard skin), with a spectrum of cutaneous disease:
    1. Limited cutaneous systemic sclerosis (70 - 80%; previously CREST syndrome) e.g. distal to knees or elbows
      • Anti-centromere antibodies
      • High risk of severe GI disease and pulmonary hypertension

    2. Diffuse cutaneous systemic sclerosis (20 - 30%)
      • Anti-scl70, anti-topoisomerase 1 or anti-U3-RNP autoantibodies
      • Higher risk of cardiac involvement, interstitial lung disease and renal disease

    3. Systemic sclerosis with coalescing or overlapping connective tissue disease (20%) e.g. SLE, polymyositis

    4. Systemic sclerosis without scleroderma (<5%)

  • Raynaud's phenomenon
  • Sclerodactyly (finger involvement)
  • Widespread telangiectasia
  • Calcinosis

  • No specific disease-modifying therapy
    • Tocilizumab (anti-IL-6 receptor mab) is promising
    • Immunosuppressive agents are used e.g. mycophenolate, methotrexate, cyclophosphamide, calcineurin inhibitors or rituximab

Management of organ dysfunction

Process Management
Interstitial lung disease Nintedanib
Tocilizumab
Pirfenidone
Immunosuppression
Pulmonary hypertension As standard
Cardiac disease Diuretics
Cardiac resynchronisation therapy
GORD PPI/antacids
Prokinetics
Renal disease ACE-I
Raynaud's Calcium channel blockers
Angiotensin receptor antagonists
Prostacyclins


Perioperative management of the patient with systemic sclerosis


History and examination

  • Establish course, extent and severity of disease/organ system involvement
  • The frequency of Rheumatological review can be an indicator of disease severity
  • Elicit specifically clinical features of respiratory or cardiac involvement
  • Airway assessment as standard

Investigations

  • Bloods:
    • FBC - may show anaemia
    • U&E inc. magnesium - check for renal involvement and side-effects of management such as diuretic-induced hypomagnesaemia
    • LFTs - may be deranged by immunosuppressive agents
    • Clotting profile - prolonged APTT may suggest antiphospholipid antibodies
    • ESR & CRP - not usually raised so elevated levels may suggest intercurrent infection

  • Low threshold for respiratory investigations such as lung function testing, arterial blood gas, CXR or high-resolution CT
  • 12-lead ECG
  • Low threshold for more intensive cardiac investigations including TTE, cardiac MRI
  • Assessment of functional capacity
  • MUST scoring as prone to malnutrition due to upper GI issues ± dietician referral

Optimisation

  • Liaison with rheumatology regarding perioperative drug management and suitable review
  • Consider respiratory and/or cardiology input if indicated
  • An FVC <50% predicted is suggestive of difficulty in weaning off mechanical ventilation
  • Liaison between rheumatology and renal teams with respect to the risk/benefit of continuing/suspending pre-operative ACE-inhibitor therapy
  • Ensure antacid/PPI ± prokinetic premedication

Monitoring and access

  • AAGBI
  • Thick skin and contractures may make NIBP measurements unreliable
  • Raynaud's may may peripheral saturations monitoring unreliable
  • Peripheral venous access may be difficult; use ultrasound ± central venous access
  • Caution with radial arterial lines as may induce arterial vasospasm and compromise distal flow; brachial may be preferable

Anaesthetic technique

  • RA may be preferable to GA

  • GA carries risks including:
    • Potentially difficult airway including difficult facemask ventilation
    • High risk of aspiration, necessitating RSI technique
    • Haemodynamic instability due to effects of anaesthesia on top of depleted volume status, myocardial involvement, effects of anti-hypertensives ± pulmonary hypertension
    • Ventilatory difficulties relating to interstitial lung disease

  • A spontaneously ventilating technique may be preferable, especially in those with advanced interstitial lung disease
  • Use lung-protective ventilation, including permissive hypercapnoea
  • Consider targetting lower saturations e.g. >88%

  • Regional techniques are also not without issue:
    • Difficult positioning due to contractures
    • Difficult skin puncture
    • Exaggerated haemodynamic response to neuraxial techniques
    • Limited spread of LA in fascial planes due to soft tissue fibrosis
    • Failure and risk of need for GA
  • Careful titration via catheter-based techniques are preferable e.g. spinal catheter, CSE

Drug management

  • Avoid clonidine, non-selective β-blockers and dopamine agonists
    • May trigger Raynaud's phenomenon leading to digital ischaemia
  • Use indirectly acting vasopressors (ephedrine) preferentially over direct agents (phenylephrine, metaraminol) for the same reason

  • Try to dampen sympathetic responses e.g. to pain, anxiety as may worsen peripheral perfusion
  • Multi-modal analgesia is preferable although NSAIDs are generally contra-indicated

Care bundle

  • Meticulous asepsis due to immunosuppression
  • Appropriate peri-operative antibiotic prophylaxis, especially given immunosuppressed state
  • Temperature monitoring, maintaining warmth at all times
  • Use fluid warmers, under-mattress warmers, forced air blankets
  • Ensure digits and extremities are warmed, inspected regularly and saturations probe moved regularly to avoid digital ulceration
  • Meticulous positioning due to presence of contractures, low body mass and risk of nerve entrapment
  • Use methylcellulose eye drops as may have eyelid skin involvement and/or keratoconjunctivitis sicca
  • VTE prophylaxis

  • Level 2 or 3 post-operative care usually required
    • Unplanned post-operative critical care admission is usually due to respiratory issues (66%), which may be due to infection
    • Use of NIV may be preferable to invasive ventilation; the latter carries an 85% mortality
  • Mindful that advanced hand disease may preclude proper use of PCA

Scleroderma renal crisis

  • A severe complication which occurs in up to 10%
  • 25% will need RRT, of whom two-thirds will become dialysis-dependent
  • 1yr mortality is 30%

  • Presents with:
    • Rapid onset (malignant) hypertension (90%)
    • Oligo-/anuria
    • Encephalopathy: headaches, visual disturbance, cognitive disturbance, seizures
    • Cardiac failure
    • Thrombotic microangiopathy
    • Non-specific symptoms such as fatigue, malaise

  • Management is with ACE-I, treatment of hypertensive crisis, pulmonary vasodilators and consideration of plasma exchange
  • Recovery of renal function takes up to three years

Catastrophic antiphospholipid syndrome

  • A rare complication of those with antiphospholipid syndrome manifesting as accelerated multi-organ failure and carrying a 50% mortality
  • Features include:
    • Acute renal failure
    • Pulmonary vasculopathy
    • Cerebral vasculopathy
    • Coronary artery thrombosis
  • Management includes anticoagulation (seek haematology advice), immunosuppression, statins and consideration of plasma exchange