What anaesthetic considerations are required for a patient with sarcoidosis (including pulmonary and cardiac involvement, hypercalcaemia, chronic steroid therapy, and airway disease) pre‑operatively, intra‑operatively, and post‑operatively?

Anesthetic Management of Sarcoidosis

For patients with sarcoidosis undergoing surgery, the anesthetic approach must prioritize comprehensive preoperative cardiopulmonary assessment, meticulous intraoperative hemodynamic and oxygenation control, and extended postoperative monitoring with aggressive respiratory support to prevent life-threatening cardiac arrhythmias, respiratory failure, and hypercalcemic crisis.

Preoperative Assessment and Optimization

Cardiac Evaluation

• Screen all sarcoidosis patients for cardiac involvement regardless of symptoms, as cardiac sarcoidosis is present in 25% of patients but only 5% are symptomatic, yet it can cause sudden death 1
• Obtain baseline ECG, echocardiography, and consider cardiac MRI or PET scan if any suspicion of cardiac involvement exists 1
• Maintain sinus rhythm and control ventricular rate aggressively, as atrial contribution to ventricular filling is critical in patients with diastolic dysfunction 2
• If atrial fibrillation develops acutely perioperatively, perform immediate synchronized cardioversion if hypotension occurs 2

Pulmonary Assessment

• Obtain pulmonary function tests (FVC, MIP, MEP, PCF) to quantify restrictive lung disease from pulmonary fibrosis 3
• At least 90% of sarcoidosis patients have lung involvement, though many are asymptomatic 1
• Assess for pulmonary hypertension, which can complicate advanced disease 1, 4

Airway Evaluation

• Perform detailed airway examination for potential difficult intubation due to laryngeal or upper airway granulomatous involvement 5
• Sarcoidosis can cause airway obstruction from granulomas in the larynx, trachea, or bronchi 1

Metabolic Screening

• Measure serum calcium in all patients, as hypercalcemia occurs in 2-63% of sarcoidosis patients depending on measurement frequency 6
• Check for hypercalciuria, which is twice as prevalent as hypercalcemia and can cause nephrolithiasis 6
• Hypercalcemia results from uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages in granulomas 6

Steroid Management

• Continue chronic corticosteroid therapy perioperatively and provide stress-dose steroids (hydrocortisone 100 mg IV every 8 hours) to prevent adrenal crisis 7
• Most patients requiring treatment are on long-term corticosteroids, which remain the most effective therapy despite side effects 7

Intraoperative Management

Anesthetic Technique Selection

• Use total intravenous anesthesia (TIVA) with propofol and remifentanil as primary agents 3
• Avoid succinylcholine due to risk of hyperkalemic cardiac arrest and rhabdomyolysis in connective tissue disease 3
• Use non-depolarizing muscle relaxants (rocuronium or vecuronium) if neuromuscular blockade is required 3
• Consider regional anesthesia when feasible for peripheral procedures, as it reduces systemic stress response 2

Hemodynamic Goals

• Maintain mean arterial pressure within 10-20% of baseline to ensure adequate organ perfusion, particularly renal perfusion to prevent sarcoid renal crisis 3
• Avoid systemic hypotension, which can precipitate myocardial ischemia in patients with cardiac sarcoidosis or coexisting coronary disease 2
• Use phenylephrine or norepinephrine to treat hypotension rather than preload-reducing agents like nitroglycerin 2
• Titrate intravascular volume carefully using central venous pressure monitoring or transesophageal echocardiography to maintain adequate cardiac output without excessive left atrial pressure 2

Oxygenation and Ventilation

• Maintain SpO2 ≥95% continuously to prevent hypoxemia-related complications in patients with restrictive lung disease 3
• Use protective lung ventilation strategies with tidal volumes of 6 mL/kg, PEEP, and alveolar recruitment maneuvers if mechanical ventilation is required 2
• Monitor for adequate ventilation and avoid hypercapnia, which can worsen pulmonary hypertension 8

Temperature Management

• Prevent hypothermia aggressively by maintaining normothermia throughout the procedure, as temperature extremes can trigger vasospasm and worsen Raynaud's phenomenon in patients with systemic involvement 3
• Use forced-air warming devices and warmed intravenous fluids 3

Positioning and Skin Protection

• Pad all pressure points meticulously, as skin may be fragile and prone to breakdown from chronic steroid use 3
• Avoid excessive trauma during positioning that could cause skin injury 3

Monitoring

• Use standard ASA monitoring with continuous ECG, blood pressure, pulse oximetry, and capnography 2
• Consider invasive arterial blood pressure monitoring for major procedures or patients with significant cardiac involvement 2
• Avoid drugs that cause histamine release (morphine, atracurium, mivacurium) as they may trigger vasospasm 3

Postoperative Management

Immediate Recovery

• Admit all sarcoidosis patients to ICU or high-dependency unit for continuous cardiopulmonary monitoring for at least 24-48 hours, even after seemingly uncomplicated procedures 3
• Extubate only when fully awake with complete reversal of neuromuscular blockade verified 2
• Place patients in semi-upright position for extubation and recovery 2

Respiratory Support

• Continue SpO2 monitoring continuously for minimum 24 hours postoperatively 3
• Monitor for delayed respiratory complications from restrictive lung disease 3
• If hypercapnic respiratory failure develops (pH <7.35), initiate non-invasive ventilation (NIV) with bilevel positive airway pressure immediately 8
• Target oxygen saturation 88-92% using controlled oxygen therapy to prevent worsening hypercapnia 8

Aggressive Respiratory Physiotherapy

• Implement multimodal respiratory physiotherapy combining deep breathing exercises (30 deep breaths per hour while awake), early mobilization, and bronchial drainage techniques 2, 8
• Progress from bed mobility to sitting, standing, and walking within 24 hours 8

Pain Management

• Use regional analgesia techniques (paravertebral block or epidural) as first-line combined with scheduled acetaminophen and short-course NSAIDs 2, 8
• Reserve opioids exclusively for breakthrough pain, not as primary analgesics, to minimize respiratory depression 8
• Adequate pain control is essential to enable effective respiratory physiotherapy 8

Metabolic Monitoring

• Monitor serum calcium levels, especially if patient had preoperative hypercalcemia 6
• If corrected total calcium rises above 3 mmol/L or becomes symptomatic, initiate glucocorticosteroid treatment to inhibit macrophage 1α-hydroxylase activity 6
• Ensure adequate hydration with urine output >2 liters/day 6

Steroid Coverage

• Continue stress-dose steroids until patient is stable and tolerating oral intake, then taper to maintenance dose 7
• Consider prophylactic antibiotics for Pneumocystis pneumonia if receiving ≥20 mg methylprednisolone equivalent for ≥4 weeks 2
• Provide proton pump inhibitor for GI prophylaxis with prolonged steroid use 2

Critical Pitfalls to Avoid

• Do not discharge patients to regular ward—they require extended monitoring even after uncomplicated procedures due to risk of delayed cardiac arrhythmias and respiratory decompensation 3
• Do not use uncontrolled high-flow oxygen, which worsens hypercapnia and increases mortality 8
• Do not delay NIV initiation while waiting for chest X-ray if severe acidosis (pH <7.25) is present 8
• Do not rely on incentive spirometry alone without combining it with deep breathing exercises, early mobilization, and NIV when indicated 8
• Avoid inadequate pain control, which prevents effective respiratory physiotherapy and increases pulmonary complications 8
• Do not assume absence of cardiac involvement based on lack of symptoms—25% have cardiac sarcoidosis but only 5% are symptomatic 1

Criteria for Escalation of Care

Proceed to intubation and invasive mechanical ventilation if:

• Worsening acidosis despite NIV 8
• Deteriorating mental status 8
• Hemodynamic instability 8
• Inability to clear secretions 8
• Patient intolerance of NIV interface 8
• Multi-organ failure 8