This 20 year old Caucasian woman, diagnosed at 8 years of age with systemic lupus erythematosus (SLE), presented to the hospital with a one month history of non-traumatic low back pain. She was significantly incapacitated and required crutches for ambulation. The pain had a sudden onset and woke her up from her sleep. There was no history of recent changes in her urinary or gastrointestinal functions. Prior to admission, she had been seen by a physician who prescribed ibuprofen (400 mg q6h), but with no relief. Other medications at the time of admission were prednisolone (60 mg/day) and furosemide (20 mg QD). She had increased her steroid dose on her own because of worsening arthralgias. She had not visited her rheumatologist for a considerable period of time and admitted poor compliance regarding scheduled medical appointments. Her knowledge of her medical condition was limited. The initial presentation during childhood was that of epistaxis secondary to thrombocytopenia. She had primary amenorrhea, with some breast development and minimal growth of axillary and pubic hair. In addition, the patient had a history of avascular necrosis of the hips and she had also developed cataracts.
The physical examination on admission revealed truncal obesity and cushingoid facies. The vital signs were T 37.8°C, P 104/min, R 20/min, BP 120/80 mmHg. She had a dry oral mucosa and supple neck. The lungs were clear. On auscultation she had normal S1 and S2, regular rhythm and no murmurs. The abdomen had striae. No organomegaly was noted. She had large hematomas on both shins. Her sensation and reflexes were intact, but her motor functions could not be explored due to pain.
Laboratory: WBC 8.5 (S 96%, L 3%), Hb/Hct 9.6/28, MCV 91, Plt 75, PT/PTT 14/36; Calcium 6.6, SGOT/SGPT 92/30, protein T/A 4.1/2.1, bilirubin T 0.3, BUN/creat 21/1.1, glucose 133. Arterial blood gas: pH 7.38, pCO2 32, pO2 80, HCO3 19, and base excess -4.1. In addition, her serology was positive for anti-double stranded DNA and anti-cardiolipin antibodies. The urine analysis showed trace protein, many bacteria, and amorphous crystals. ECG: sinus tachycardia, otherwise unremarkable. The chest-x ray had evidence for increased pulmonary vascular congestion. An x-ray of the spine demonstrated diffuse demineralization and loss of height of vertebral bodies.
The patient was evaluated by a rheumatologist who prescribed pain medication as needed (meperidine 75 mg IM, and acetaminophen/oxycodone 2 tab). In the hospital, prednisone was started at 30 mg/day (during the first 2 days) and then tapered over the following days. Calcium carbonate and vitamin D were also prescribed in view of the severe osteopenia. During the initial hospital days, the patient reported slight symptomatic improvement. A CT scan of the lower thoracic and lumbar spine revealed excessive epidural lipomatosis compressing cauda equina nerve roots. Diffuse osteopenia was again noted, in addition to multiple compressed vertebral bodies.
Several days after admission, the patient developed severe dyspnea in association with tachypnea (44/min) and tachycardia (140/min). Arterial blood gases (ABG) performed at that time revealed severe hypoxemia with respiratory alkalosis. She was intubated, and heparin was initiated (V/Q scan was reported as negative). In view of continuing clinical deterioration with worsening tachypnea (60/min) and tachycardia (150/min), the patient was admitted to the intensive care unit (ICU). Repeat chest-x-ray showed bilateral interstitial infiltrates and was reported consistent with adult respiratory distress syndrome (ARDS). Antibiotics were started to provide coverage for possible opportunistic pathogens. Bronchoalveolar lavage was performed and was reported positive for Pneumocystis carinii. A Swan-Ganz catheter was inserted revealing pressures within the normal range. The remainder of her hospital stay was characterized by a severe decrease in peripheral platelet numbers together with persistent fibrin degradation products (fDp) at > 1:20, falling fibrinogen levels and hem positive stools (signs of disseminated intravascular coagulation or DIC). Purpuric lesions and violaceous plaques developed on the trunk and extremities, with eventual evolution to vesicular lesions on breasts and abdomen. A series of chest-x-rays showed progressive worsening of the bilateral interstitial infiltrates. She became markedly hypotensive with a systolic blood pressure of 60 mmHg and a heart rate of 60 ppm. Shortly thereafter, she went into asystole. In accordance with the expressed wishes of the next-of-kin, no further measures were taken and the patient was pronounced dead.
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