List the clinical features associated with Systemic Lupus Erythematosus, and outline how they would influence your management of a patient in Intensive Care.
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.
This question would benefit from a structured tabulated answer.
|Topic Area||Manifestations||Influence on ICU management|
|Increased risk of coronary artery disease||
|Endocrine||Cushing syndrome due to chronic steroid use||
|Gastrointestinal||Gastrointestinal tract vasculitis
|Hepatic||Hepatic vein thrombosis due to APLS||
|Haematological||Anaemia of chronic disease||
|Infectious||Immunossuppression, and the risk of sepsis||
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.