CPC: Case Study

Chief Complaint:
A 25-year-old female with systemic lupus erythematosis with new onset seizures.

History of Present Illness:
Patient is a 25-year-old female who moved to the United States from Nigeria four months ago. Three months ago, she presented to her physician at an outside hospital complaining of fatigue, and was found to be anemic with an elevated creatinine (4.3 mg/dl) and proteinuria (12.8 gm/24 hr). A renal biopsy demonstrated diffuse proliferative and membranous glomerulonephritis consistent with lupus nephritis (Figure 1). Her serology showed decreased complements and a positive anti double stranded DNA titer of 1:640. The patient was been treated with intravenous cyclophosphamide and prednisone with dramatic improvement in her renal function. She last received intravenous cyclophosphamide two weeks prior to admission at which time her creatinine was 1.3 mg/dl and her 24 hour urine had 1.4 gm protein.

On the 1/30/01, she developed tonic-clonic seizures and was brought to an outside hospital. Over 4 hours, she had 4 seizures that were controlled with benzodiazipines and phenytoin. A CT scan showed suggested occipital infarctions bilaterally and periventricular white matter changes (Figure 2). Initial LP showed 1 WBC, Glucose=49 (serum=150 mg/dl), Protein= 23 mg/dl.

Past Medical History:
Prior to her developing lupus, the patient had never had a serious illness. There was no prior history of hypertension or of diabetes. There is questionable history of removal of a benign ovarian cyst.

Social History
The patient lives with her husband in Baltimore County. She does smoke or use alcohol. She is presently unemployed. While in Nigeria, she worked as a sales person for a cellular phone company.

Allergies
No known drug allergies.

Medications
Prednisone, 60 mg, po qD.
Furosemide, 80 mg, po q.D.
Cyclophosphamide, 1000 mg, IVq 3 months
Epogen, subQ, qW

Review of Systems
The patient denies shortness of breath, chest pain, abdominal pain, nausea and vomiting. She does note a steady pain in her knees and ankles, which is worsened by weight bearing.

Physical Exam and Hospital Course
She was transferred to Johns Hopkins Hospital on 2/1/01. At that time her temperature was 99.1, BP= 150/100, HR=100, RR=16 with normal SaO2. Lung, abdomen, and cardiac examination was normal. She was somnolent and verbalizing slowly, was oriented to place and name but not date, she had the suggestion of a L visual field defect, did not look up but looked down normally, and one examiner thought she had mild R sided neglect. She moved all 4 extremities symmetrically and had no sensory defects. Reflexes were symmetric, her right toe went down and left toe went up during Babinski testing. Labs on admission were notable for BUN= 24, creatinine=1.4, Albumin= 1.9 mg/dl, hematocrit= 27%, WBC= 4800 (86% polys, 8% lymphocytes), platelets= 144,000, INR= 1.0, aPTT= 23.5 seconds. Urinalysis was normal except for 2+ protein.

She was treated with Solumedrol 1gm x 3 days followed by Prednisone 40 mg/day. Phenytoin was continued and she had no further seizures.

Over the next week, she remained oriented to person and place, not time. She also had a persistent headache and fevers from 38-39.50 C. A blood culture grew coagulase negative staphylococcus and enterococcus. Her central line was removed, she was started on Vancomycin and Gentamicin. Echocardiogram was normal and subsequent blood cultures were negative.

10 days after admission she was persistently febrile and had worsening mental status. She was stuporous and not following commands. She also developed a new peripheral L VIIth cranial nerve palsy. Blood and urine cultures were negative. An MRI showed "non-specific T2 abnormalities in the left posterior parietal lobe and basal ganglia" (Figure 3). A chest CT showed a "subtle mixed alveolar/interstitial nodular infiltrate diffusely in both lungs" (Figure 4). A lumbar puncture revealed an elevated opening pressure (not recorded) WBC= 300 (80% polys), RBC= 70, Protein= 145 mg/dl, Glucose= 10 mg/dl. Stains were negative for bacteria, mycobacteria, and fungi.

Figure 3: February 14, 2001
Figure 3: February 14, 2001

Figure 4

Over the next three days her mental status and respiratory status worsened. She had a respiratory arrest, was intubated and was placed on mechanical ventilation. Her pupils became fixed and unresponsive. An intraventricular drain was placed for hydrocephalus. Despite aggressive intensive care she expired 2 weeks later with multi-organ failure.

Questions to consider: