Systemic Sclerosis
Patient (condition)
 
Aliases:
Scleroderma

Topic aliases are alternate phrasings for a particular topic.


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Anaesthetic overview -
Chronic collagen/connective tissue disease affecting the skin, joints & visceral organs. Anesthetic considerations are related predominantly to disease processes involving the lungs (interstitial fibrosis leading to restrictive lung disease with reduced gas exchange), heart (pulmonary hypertension, cor pulmonale, myocardial fibrosis leading to conduction defects), oesophagus (dysmotility increasing risk of gastric reflux), kidneys (renal vessel fibrosis leading to hypertension, proteinuria, chronic renal failure), and joints (difficult laryngoscopy, intra-operative positioning).

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Limited scleroderma, or limited systemic sclerosis, was previously known as CREST syndrome. This syndrome is characterised by the presence of Calcinosis, Raynaud's-disease, Esophageal dysmotility, Sclerodactily, and Telangtiectasia. refl.in/1p

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Nov 26, 2012
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Limited systemic vs diffuse systemic sclerosis?
Limited systemic scleroderma is associated more frequently with pulmonary hypertension (note this is in the absence of pulmonary fibrosis) refl.in/im.
Diffuse systemic sclerodema is more frequently associated with pulmonary fibrosis.

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Assess for evidence of and severity of pulmonary hypertension if long term or severe disease

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Assess baseline renal function if not recently investigated. Scleroderma may result in renal vessel fibrosis, proteinuria, and progressive renal failure.

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Sep 18, 2011
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Consider performing respiratory function tests to quantify severity of any restrictive or lung diffusion deficits which may influence decision to admit to a high dependency or intensive care unit.

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Enquire about the presence of other conditions associated with limited systemic sclerosis (previously known as CREST syndrome - Calcinosis, Raynaud's disease, Esophageal dysmotility, and Telangiectasia.)

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Aug 05, 2012
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Patient may be warfarinised if severe pulmonary hypertension is present. Consider this when planning neuraxial procedures.

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Insertion of radial arterial lines is relatively contraindicated if patient has Raynaud's syndrome as part of their spectrum of disease

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Carefully assess airway to determine the effect of any TMJ or cervical spine involvement on mouth opening or neck mobility

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Consider risk of intraoperative gastric reflux and pulmonary aspiration for patients with oesophageal dysmotility. If performing general anesthesia consider either rapid sequence induction or awake fiberoptic intubation.

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Pay particular attention to patient positioning as joint mobility may be limited

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