Question 9 from the first paper of 2005 asked the candidates to list the clinical features and manifestations of SLE. Specific focus was on the influence of these various manifestations on the course of ICU stay, and how you might go about managing them.

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
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

Good, J. T., et al. "Lupus pleuritis. Clinical features and pleural fluid characteristics with special reference to pleural fluid antinuclear antibodies."CHEST Journal 84.6 (1983): 714-718.

HELLMANN, DAVID B., MICHELLE PETRI, and Q. U. I. N. N. WHITING-O'KEEFE. "Fatal infections in systemic lupus erythematosus: the role of opportunistic organisms." Medicine 66.5 (1987): 341-348.

Urbanus, Rolf T., Ronald HMW Derksen, and Philip G. de Groot. "Current insight into diagnostics and pathophysiology of the antiphospolipid syndrome."Blood reviews 22.2 (2008): 93-105.

Kokori, Styliani IG, et al. "Autoimmune hemolytic anemia in patients with systemic lupus erythematosus." The American journal of medicine 108.3 (2000): 198-204.

Giannouli, Stavroula, et al. "Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment." Annals of the rheumatic diseases65.2 (2006): 144-148.

Van Steenbergen, Werner, et al. "‘Lupus’ anticoagulant and thrombosis of the hepatic veins (Budd-Chiari syndrome): Report of three patients and review of the literature." Journal of hepatology 3.1 (1986): 87-94.

Sultan, S. M., Y. Ioannou, and D. A. Isenberg. "A review of gastrointestinal manifestations of systemic lupus erythematosus." Rheumatology 38.10 (1999): 917-932.

Cameron, J. Stewart. "Lupus nephritis." Journal of the American Society of Nephrology 10.2 (1999): 413-424.

Bruce, Ian N., et al. "Risk factors for coronary heart disease in women with systemic lupus erythematosus: the Toronto Risk Factor Study." Arthritis & Rheumatism 48.11 (2003): 3159-3167.

Sanders, E. A. C. M., and L. A. H. Hogenhuis. "Cerebral vasculitis as presenting symptom of systemic lupus erythematosus." Acta neurologica scandinavica 74.1 (1986): 75-77.

Jacobson, Edwin J., and Michael J. Reza. "Constrictive pericarditis in systemic lupus erythematosus. Demonstration of immunoglobulins in the pericardium."Arthritis & Rheumatism 21.8 (1978): 972-974.

Busteed, S., et al. "Myocarditis as a prognostic indicator in systemic lupus erythematosus." Postgraduate medical journal 80.944 (2004): 366-367.

Turner-Warwick, Margaret, and Deborah Doniach. "Auto-antibody studies in interstitial pulmonary fibrosis." British medical journal 1.5439 (1965): 886.

Asherson, R. A., et al. "Pulmonary hypertension in systemic lupus erythematosus." British medical journal (Clinical research ed.) 287.6398 (1983): 1024.

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.

Martin, L., et al. "Upper airway disease in systemic lupus erythematosus: a report of 4 cases and a review of the literature." The Journal of rheumatology19.8 (1992): 1186-1190.

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.