Question 22

A patient is admitted to the ICU with a history of systemic sclerosis.
Discuss the implications for intensive care management of the following aspects.
a) Cutaneous manifestations of systemic sclerosis. (20% marks)
b) Cardiovascular manifestations of systemic sclerosis. (40% marks)
c) Respiratory manifestations of systemic sclerosis. (40% marks)

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College answer

Aim: To explore how multisystem disease processes affect ICU treatment.
Key sources include: Clinical case mix. CanMEDS Medical Expert.
Discussion: This question mimics the miscellaneous multisystem disease topics on rheumatoid arthritis (2019.2 Q11) scleroderma, and obesity (2012.1 Q28, 2008.2 20.1, 2003.2 Q8). Candidates were asked to discuss the clinical implications for intensive care management. Candidates are encouraged to follow the directions of the question as candidates who did this received a passing mark. The unsuccessful candidates simply listed the abnormalities found in systemic sclerosis. This did not address the
question asked. In many instances the implications for intensive care management were missing or superficial. 

Answers demonstrating awareness of the ICU implications include but are not limited to the following examples:
Cutaneous: Potentially difficult intubation as limited mouth opening and limited soft tissue mobility. Fixed flexion deformities in advanced disease will increase pressure area risk.
Respiratory: Pulmonary fibrosis increasing barotrauma risk, 2’ to low compliance. Increased risk of right heart failure 2’ to chronic corpulmonalae requiring PEEP titration to avoid excessive RV afterload


This does indeed recall Question 8 from the second paper of 2003, which asked for the clinical manifestations of CREST syndrome. That potential list is rather long, and what appears to have happened is a restriction of that range of issues into just the respiratory, cardiovascular and cutaneous. 

Cutaneous manifestations and their implications

  • Limited neck extension and limited mouth opening: difficult intubation
  • Poor skin perfusion promotes pressure areas
  • Telangiectasia is present also on mucosal surfaces; there is an increased risk of bleeding from upper GI sites
  • Difficult vascular access: the skin, being very thick, makes it difficult to palpate vessels (veins and arteries both)

Cardiovascular manifestations and their implications

  • Cardiac problems:
    • Arrhythmias,
    • Hypertrophic cardiomyopathy (due to chronic hypertension)
    • Myocardial fibrosis (thus, restrictive diastolic failure)
    • Pericardial stricture (also restricts diastolic filling)
    • Right heart failure due to pulmonary hypertension 
  • Vascular problems
    • Poor distal perfusion of the extremities, leading to gangrene- as one might expect this is not improved by arterial cannulation.
    • Cerebral vasculitis is a differential for delirium
    • Renal artery stenosis is a differential for renal failure
  • Haemodynamic problems
    • These patients usually have chronic steroid use: need to consider adrenal insufficiency when they become shocked
    • Will rapidly become volume-overloaded because of diastolic dysfunction
    • RV can decompensate due to excessive volume

Respiratory manifestations and their implications

  • Mechanical ventilation is influenced by:
    • Pulmonary fibrosis
    • Restrictive lung disease
    • Pulmonary hypertension
  • All of these limit the range of volumes and pressures which can be safely used
  • SpO2 monitoring may be frustrated by poor end-digital perfusion
  • The rapidly fatal "scleroderma-pulmonary-renal syndrome (SPRS)" may develop, which manifests as a fulminant course of acute normotensive renal failure associated with diffuse alveolar hemorrhage.


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TUFFANELLI, DENNY L., and R. K. Winkelmann. "Systemic scleroderma: a clinical study of 727 cases." Archives of Dermatology 84.3 (1961): 359-371.

Silver, Richard M. "Clinical aspects of systemic sclerosis (scleroderma)." Ann Rheum Dis 50.suppl 4 (1991): 854-61.

Farber, Harrison W., Robert W. Simms, and Robert Lafyatis. "Analytic Review: Care of Patients With Scleroderma in the Intensive Care Setting." Journal of intensive care medicine 25.5 (2010): 247-258.

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Shalev, T., et al. "Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases." Clinical and experimental rheumatology 24.4 (2006): 380.

Janssen, Namieta M., Dilip R. Karnad, and Kalpalatha K. Guntupalli. "Rheumatologic diseases in the intensive care unit: epidemiology, clinical approach, management, and outcome." Critical care clinics 18.4 (2002): 729-748.