Presentation of systemic lupus erythematosus (SLE) in emergency department: a case report
© Fernandes et al.; licensee BioMed Central Ltd. 2013
Received: 27 March 2013
Accepted: 24 April 2013
Published: 5 May 2013
Abrupt and life-threatening presentations in connective tissue diseases (CTD) are rarely reported. Their early recognition and specific management could change course disease. SLE is a multisystem inflammatory disease that is often difficult to diagnose in the emergency department (ED).
A 26-year-old woman presented to the ED with a 48 hour history of progressive dispnea, generalized edema and left lower chest pain with non-productive cough. On examination, patient was feeling very ill, afebrile, tachycardic, tachypneic and a peripheral oxygen saturation of 94% on 40% supplemented oxygen with raised jugular venous pressure was noted. Intermittently, she presented an obtunded state of consciousness. A large pericardial, pleural and abdominal effusion was confirmed and a broad differential diagnosis was made. The patient had a 6 months history of inflammatory polyarthralgias involving initially interphalangeal joints, evolving, sometime later, the knees and elbows bilaterally and she was started glucocorticoids. 12 days before admission, she had had symptoms of a urethritis episode. In the context of an immunosupressed patient, with initial focal urologic complains, evidence of multiorgan dysfunction and a picture resembling a distributive shock, dictated a low threshold for sepsis.
Separating an acute episode of SLE from sepsis, on emergency grounds, can even be the most challenging decision. In the ED, acute life-threatening and multisystemic conditions should arise the suspicion of autoimmune diseases, particularly SLE.
Abrupt and life-threatening presentations in connective tissue diseases (CTD) are rarely reported. Their early recognition and specific management could change course disease. SLE is a multisystem inflammatory disease that is often difficult to diagnose in the emergency department . To the emergencist, it`s important to consider SLE as possibility, when faced with a patient with symptoms and/or signs suggesting a multisystem disorder. Emergent complications of SLE are managed in the usual manner the most described being: strokes, acute myocardial infarctions, hemoptysis, respiratory distress and pulmonary emboli [2–4]. Other complications, such as pericardial tamponade , pulmonary hemorrhage, renal failure, or cerebritis should be managed with appropriate subspecialist consultation.
In this patient, SLE diagnostic criteria were accomplished when malar rash, polyserositis and positive immunology were summed to the previous described anamnestic features. All clinical and laboratory parameters pointed to a very active clinical process. As so, a true flare or a first full presentation of SLE were both tentative hypothesis, considering the short duration of the disease since she first call for medical attention and voluntary suspension of therapy. Such an explosive clinical presentation, in the context of an immunosupressed patient, with initial focal urologic complains, evidence of multiorgan dysfunction and a picture resembling a distributive shock, dictated a low threshold for sepsis and the decision to treat with antimicrobials until infection was surely excluded. This option was supported by generalized knowledge that, in sepsis, the prognosis varies with the elapsed time and the precocity and aggressivity of the initial measures. Separating an acute episode of SLE from sepsis, on emergency grounds, can even be the most challenging decision. We have also seen such a dramatic clinical picture in young people, particularly women, with catastrophic antiphospholypid syndrome, which led us to consider it in this patient. In retrospect, the full immunossupression with glucocorticoids aborted the potential negative consequences of the active SLE, and allowed a conservative approach to a potential lifethreatning consequence of a large pericardial effusion [5, 6]. It remain unanswered if it was a real flare or the full expression of a progressing lupus, and perhaps it would turn an academic question in face of pressing clinical situations like this one, where treating with a structured reasoning, considering acute presentations of connective tissue disease and its complications will be more important and rewarding.
In the emergency department, acute life-threatening and multisystemic conditions should arise the suspicion of autoimmune diseases, particularly SLE. Circulating immune complexes and autoantibodies with multiples specificities, diffusely deposit and attack a multitude of organic targets, activating complement and mounting a full inflammatory response, provide a substract for the proteiform clinical e laboratorial expression of this disease. Usually, albeit impressive and frightening, the different manifestations can be successfully managed by adequate, early and full immunossupression .
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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