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KEY POINTS

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  • Most ICU admissions for rheumatology patients are prompted by infection.

  • New-onset rheumatic diseases rarely prompt ICU admission in the absence of a revealing prodrome.

  • In most patients without a previously established collagen vascular disease, suspected vasculitis will be explained by an alternative diagnosis.

  • Serologic assessment of critically ill patients is a double-edged sword providing both enlightenment and misleading shadows. All serologic testing must be interpreted with a thorough understanding of the patient’s clinical condition.

  • Inability to assign specific diagnostic labels to patients with severe life-threatening autoimmune or inflammatory disease should not delay therapeutic intervention.

  • Not all ischemic skin lesions that appear to be vasculitis are. Vasculopathies of various causes should always be part of the differential diagnosis.

  • Empiric trials with corticosteroids can be a rational approach to patient care when such trials are carried out appropriately and infection and malignancy have been excluded.

  • Acute organic brain syndrome without focal neurologic deficits or evidence of systemic vasculitis is unlikely to be due to vasculitis.

  • Fever in patients with systemic autoimmune diseases should be presumed to be infectious if accompanied by chills, leukocytosis with a left shift, or hypotension.

  • Patients who have been treated with significant doses of corticosteroids within the past year may require empiric replacement therapy during critical illness or surgical procedures until adrenal insufficiency can be excluded.

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Approximately 10% to 25% of all rheumatic disease patients visiting the emergency department require hospitalization and up to one-third of these patients will require intensive care.1 Patients with rheumatic diseases admitted to the medical ICU most often have problems not directly related to their primary illness. Sepsis, massive gastrointestinal bleeding, and myocardial infarcts may arise secondary to treatment. The major direction of care in these circumstances often comes from the intensivist. Circumstances do arise that require the unique insight of the experienced clinical rheumatologist, who at times must direct the management of a disease-specific complication. Just as often the rheumatologist is asked to address a diagnostic dilemma spawned by puzzling clinical and laboratory data. This chapter addresses the more common issues that prompt the rheumatologist and the intensivist to collaborate.

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SYSTEMIC LUPUS ERYTHEMATOSUS

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FEVER: IS IT THE LUPUS?
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Fever in the patient with lupus presses the clinician for an urgent answer to the question: Is this caused by lupus activity or infection? Fever is a common finding in active systemic lupus erythematosus (SLE) occurring in up to approximately 50%.2 It may respond to the usual antipyretics or require corticosteroids. Single-daily-morning dose prednisone may not control late afternoon or evening fevers and may require the use of split-dosing. Leukocytosis and increased bands on peripheral smear are strong presumptive evidence for infection, as is the presence of shaking chills. Complement proteins or components, including C3 and C4, are acute-phase reactants and usually rise with infection. Low levels of complement occur in some but not all patients with active lupus. ...

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