Acute Systemic lupus erythematosus (Acute SLE) is a multisystem autoimmune disease caused by autoantibodies and the deposition of immune complexes.
The disease affects primarily women and has a higher prevalence among African Americans. A series of clinical findings is associated with acute SLE.
These include a characteristic malar rash, photosensitivity, arthritis, serositis, and a discoid rash. In addition, involvement of the renal, neurologic, and hematologic systems present with system-specific symptoms.
The presence of autoantibodies (e.g., anti-Sm, anti double-stranded DNA) can also assist in making the diagnosis. When a patient exhibits four or more of these criteria the diagnosis can be made with a sensitivity and specificity of 75% and 95%, respectively.
SLE can present with an acute or subacute onset. Most patients undergo some elements of acute exacerbation and remission during the natural course of the disease. This is important because the goals of acute SLE treatment include mainly the control of symptoms and prevention of the worsening of organ failure.
Treatment usually involves a number of systemically administered medications. For minor disease, arthritis and pain can be treated with nonsteroidal antiinflammatory medications or COX-2 inhibitors. Antimalarial agents (chloroquine, quinacrine) can also benefit patients with generalized symptoms.
For severe systemic disease, the use of steroids is the mainstay of treatment. These drugs can be administered in high doses, with attempts to reduce the dose at frequent intervals once symptoms are under control. Cytotoxic agents are used for aggressive disease including renal manifestations.
It is important that intensive care unit (ICU) clinicians understand whether the presentation of a worsening clinical condition in an acute SLE patient in the ICU results from the adverse effects of the medications used to treat the disease, an acute lupus flare, or a separate and unrelated condition.
For instance, in severe cases of acute SLE, the medications used for immunosuppression increase the possibility for severe sepsis and septic shock. It is important to remember that the mechanism of action of these drugs is aimed at the cellular component of the immune system and complement.
Therefore, patients presenting with sepsis are likely to be infected with organisms that are usually prevented by the appropriate functioning of these systems. Broad-spectrum antimicrobials should be used to treat sepsis in these patients, but consideration of other organisms (viruses, fungi) should be considered if patients do not improve quickly.
In addition, chronic steroid use has a number of complications that may contribute to problems in the ICU patient with acute SLE when used. On a chronic basis, steroids are associated with hypertension, sometimes refractory to treatment.
Steroids also suppress adrenal function, thereby making the patient susceptible to acute stressors like surgery or shock. This condition can manifest by refractory hypotension and needs to be addressed preoperatively and during the ICU course with maintenance steroids.
Steroids lead to hyperglycemia, which has recently been shown to be an independent predictor of mortality in ICU patients; thus, strict glucose control in these patients is mandatory. Steroids also contribute to the onset of peptic ulcers in the ICU and poor wound healing.
Therefore, there needs to be strict attention to both prophylaxis for ulcer disease and frequent repositioning to prevent skin breakdown and decubitus ulcer formation.
The patient with acute SLE may experience problems related to the disease itself when hospitalized in the ICU. The multisystem nature of the disease often creates problems that make caring for these patients difficult. Patients can exhibit pulmonary or cardiovascular disease.
They can have pulmonary infiltrates, effusions, and fibrosis that necessitate mechanical ventilation. Pericarditis and valvular dysfunction can lead to shock and poor cardiac output.
Patients may become hyper-coagulable, making them susceptible to central neurologic thrombosis or myocardial ischemia.
Renal insufficiency exacerbated by the renal stressors of the ICU may cause progressive renal failure that makes fluid and electrolyte management in the ICU difficult and may require dialysis.
Bone marrow suppression may create anemia that will alter oxygen-carrying capacity or thrombocytopenia that can lead to bleeding. Both of these conditions may require ongoing transfusions.