Scleroderma

Learning objectives

Describe scleroderma
Recognize the symptoms and signs of scleroderma
Anesthetic management of a patient with scleroderma

Definition and mechanisms

Scleroderma or systemic sclerosis (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis, resulting in hardening and tightening of the skin, and progressive end-organ dysfunction
SSc is characterized by increased synthesis of collagen (leading to sclerosis), damage to small blood vessels, activation of T lymphocytes, and production of altered connective tissue

Signs, symptoms, and complications

Cardiovascular: Raynaud’s phenomenon, healed pitting ulcers on the fingertips, skin and mucosal telangiectasis, palpitations, irregular heart rate, and fainting due to conduction abnormalities, hypertension, and congestive heart failure (CHD)
Pulmonary: Progressive worsening of dyspnea, chest pain (due to pulmonary hypertension), and dry, persistent cough due to interstitial lung disease (ILD)
Digestive: Gastroesophageal reflux disease (GERD), bloating, indigestion, loss of appetite, diarrhea alternating with constipation, sicca syndrome and its complications, loosening of teeth, and hoarseness (due to acid reflux)
Musculoskeletal: Joint, muscle aches, loss of joint range of motion, carpal tunnel syndrome, calcinosis, and weight loss
Genitourinary: Erectile dysfunction, kidney failure
Other: Facial pain due to trigeminal neuralgia, hand paresthesias, headache, stroke, and fatigue

Risk factors

Family history
Certain genetic factors
Gender: Occurs more in women than in men
Exposure to silica, viruses, medications, and drugs

Treatment

There is no treatment that can cure or stop the overproduction of collagen, which is characteristic of SSc. Treatment mainly focuses on controlling symptoms and preventing complications.

Raynaud’s phenomenon: Vasodilators (e.g., calcium channel blockers, α-blockers, serotonin receptor antagonists, angiotensin II receptor inhibitors, statins, local nitrates, or iloprost)
Digital ulcers: Phosphodiesterase-5-inhibitors or iloprost
Prevention of new digital ulcers: Bosentan
Malnutrition: Tetracycline antibiotics
Interstitial lung disease: Cyclophosphamide, azathioprine with or without corticosteroids
Pulmonary arterial hypertension: Endothelin receptor antagonists, phosphodiesterase-5-inhibitors, and prostanoids
Gastroesophageal reflux disease: Antacids or prokinetics
Kidney crises: Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists

Management

Preoperative risk identification and stratification

Airway
- Microstomia
- Limited cervical extension
Neurological
- Peripheral neuropathies (paresthesias, numbness)
- Autonomic dysfunction risk factors (orthostatic hypotension)
Pulmonary hypertension risk factors (dyspnea, increased PASP, RVSP)
- Progressive cardiomyopathy (TTE)
- Conduction abnormalities (ECG)
ILD risk factors
- DLCO <50%
- CRP ≥5 or ESR ≥20
- Decreased 6-minute walk test
Sleep-disordered breathing risk (apnea, daytime somnolence)
- STOP-Bang questionnaire
- Sleep study findings
Esophageal dilation: GERD symptoms
Malnutrition
- Pseudo-obstruction
- Presence of sarcopenia
Venous thromboembolic disease risk factors
- SSc diagnosis <12 months
- Atrial fibrillation
- CHD
Anemia: Preoperative complete blood count

Intraoperative risk mitigation

Difficult airway preparedness
Aspiration chemoprophylaxis
Appropriate padding and positioning
Availability of ultrasound for difficult intravenous access
Cardiac monitoring: Arterial line, possible TEE, PA catheter
Lung protective ventilation strategy
Minimize adverse drug interactions
Ensure adequate prophylactic anticoagulation

Postoperative complication surveillance

Sleep-disordered breathing: Continuous postoperative pulse oximetry
Increased myocardial infarction: Continuous postoperative telemetry
ILD deterioration: Presence of increased oxygen requirements >24 hrs after surgery; consider early workup for postoperative pneumonia or exacerbation of ILD
Venous thromboembolic disease: Surveillance in surgery, trauma, and those administered glucocorticoids
SSc renal crisis: Refractory HTN, encephalopathy, CHF

Keep in mind

Patients with SSc often have well-compensated mechanisms for their underlying disease, which may be unmasked with anesthetic interventions
Anticipate a difficult airway and the potential risk of aspiration
SSc patients are at risk for postoperative myocardial infarction, and prolonged mechanical ventilation may result in substantial morbidity, specifically in patients with interstitial lung disease