Question 9

List the clinical features associated with Systemic Lupus Erythematosus, and outline how they would influence your management of a patient in Intensive Care.

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College Answer

SLE is a chronic inflammatory disease, presumably auto-immune, which occurs predominantly in women, and can affect almost all organ systems. These can masquerade as many different conditions, and can make workup very complex. Clinical features (and examples of ways they would influence management) include:

• Fatigue (common and debilitating)- care with differential diagnosis

• Fever (episodic and related to activity of disease)- need to exclude sepsis, potential for un-necessary antibiotics

• Arthritis (painful, migratory and asymmetrical; rarely deforming)- care with positioning, may need analgesia

• Skin changes (butterfly rash, and hair loss)- care with handling

• Raynaud’s phenomenon- caution with vasoconstrictors, pulse oximeters, arterial lines

• Renal dysfunction (usually glomerulonephritis)- avoid nephrotoxins, adjust drug doses

• Pleurisy and pleural effusions- need to diagnose, exclude other conditions

• Pericarditis and Libman-Sacks (verrucous) endocarditis- may require TOE or surgery

• Increased incidence of coronary artery disease- need to be aware of problem in otherwise young females without risk factors!

• Delerium, psychosis and seizures- complex management and diagnostic problem

• Thrombosis in association with anti-phospholipid antibodies- need to diagnose, and may need treatment for pro-coagulant state

• Abnormal haematology (leukopenia, anaemia, thrombocytopenia)- may need further investigation

• Lymphadenopathy and splenomegaly- may make suspicious of alternative disease process; may ned further investigation

• Auto-immune disease and immunosuppressive therapy (eg. corticosteroids, cyclophosphamide)- at particular risk of infections in immunosuppressed. Early and aggressive workup and initial treatment may be required. Aware of potential for adrenal suppression.

Discussion

This question would benefit from a structured tabulated answer.

Manifestations of Systemic Lupus Erythematosus
and their influences on ICU management
Topic Area	Manifestations	Influence on ICU management
Airway	Cricoarytenoiditis	Need for intubation Difficult intubation Risk of post-extubation stridor
Respiratory	Pulmonary fibrosis	Impaired gas exchange Restrictive ventilatory defect
	Pleuritis	Need to test pleural fluid for ANA
	Pulmonary hypertension	Hypoxia Hypothension in response to high PEEP Right heart failure
Circulatory	Myocarditis	Need for inotropes, IABP or ECMO Potential need for RV biopsy for diagnosis
	Constrictive pericarditis	Obstructive cardiac failure Danger of colchicine therapy Need for cardiothoracic surgical release
	Increased risk of coronary artery disease	MI is higher on the list of differentials for hemodynamic instability, even in young females
Neurological	Cerebral vasculitis	A differential diagnosis of seizures and decreased level of consciousness Impaired blood brain barrier
Endocrine	Cushing syndrome due to chronic steroid use	Hyperglycaemia Hyponatremia Fluid overload
Renal	Lupus nephritis	Pre-existing renail impairment Susceptibility to nephrotoxins is increased
Gastrointestinal	Gastrointestinal tract vasculitis (and/or ulceration)	Vigilant monitoring for GI haemorrhage Malabsorption of feeds may occur
Hepatic	Hepatic vein thrombosis due to APLS	A differential for ascites and hepatic failure in SLE patients Anticoagulation will be required
Haematological	Anaemia of chronic disease	Impaired haemopoietic response to haemorrhage Increased transfusion requirements
Haematological	Haemolytic anaemia	MAHA needs to be considered in the anaemic SLE patient Vasulitic and haemolytic screening needs to be performed
	Antiphospholipid syndrome	Risk of bleeding is increased Risk of thrombosis is increased Risks of anticoagulation need to be balanced with benefits
Infectious	Immunossuppression, and the risk of sepsis	Need to consider opportunistic organisms in the selection of antibiotic cover

References

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Nanke, Y. U. K. I., et al. "Cricoarytenoid arthritis with rheumatoid arthritis and systemic lupus erythematosus." The Journal of rheumatology 28.3 (2001): 624-626.

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Cervera, Ricard, et al. "Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients." Medicine 82.5 (2003): 299-308.

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