Hematuria in a patient with class IV lupus nephritis

A 49-year-old Hispanic female with a past medical history notable for systemic lupus erythematosus (SLE) and biopsy proven lupus nephritis, hypertension, and peptic ulcer disease was referred for evaluation of hematuria. The patient also reported recent h/o arthralgias, some worsening of her existing malar and discoid rashes, and intermittent fevers. She denied any history of gross hematuria, dyspnea, chest pain, abdominal pain, fatigue, oral ulcers, Raynaud's phenomenon, or edema. The patient was seen by her nephrologist and found to have 4+ protein and 2+ blood on urinalysis with urine sediment revealing moderate dysmorphic red cells and no casts. Her first kidney biopsy revealed diffuse proliferative lupus nephritis World Health Organization class IV. At this time, the patient had undergone treatment with monthly cyclophosphamide infusions along with steroids for 6 months. Her renal function revealed a creatinine clearance of 75 ml/min. Urinalysis revealed only few red blood cells with no casts, with resolution of her proteinuria. The frequency of cyclophosphamide pulses was decreased to every 3 months for the next 12 months, which was the dose at the patient's current presentation. Her medications included cyclophosphamide 0.85 g/m(2) every 3 months, prednisone 15 mg p.o. q.d., plaquenil 200 mg p.o. b.i.d., lisinopril 40 mg p.o. q.d., nifedipine XL 60 mg p.o. q.d., and calcium/multivitamin supplements. Her family history was negative for hematuria, proteinuria, or any other known renal disease. The patient was originally from El Salvador and had been in the United States for 11 years. She denied tobacco consumption and drank alcohol occasionally. Physical examination revealed a middle-aged female with a Cushingoid appearance. Her weight was stable at 162 lbs. Her blood pressure was 130/70 mmHg; she was afebrile. Skin examination revealed increased erythematous lesions in a malar distribution, along with a circular, erythematous, maculopapular rash throughout the proximal regions of her upper extremities and the upper front and back of her thorax in a shawl type of pattern. Musculoskeletal examination revealed no evidence for active synovitis. Lungs were clear to auscultation and her abdomen was benign. Her heart examination revealed a regular rate and rhythm with an I/VI systolic ejection murmur at the left sternal border, with no rubs or gallop. Extremities revealed trace pedal edema bilaterally. Laboratory values revealed a blood urea nitrogen and creatinine of 23 and 0.7 mg/dl, respectively; white blood cells 5.87; hemoglobin 11.0 g/dl; hematocrit 33.9; platelets 298,000; electronic spin resonance 81; double-stranded DNA 475 IU (Normal -0-25 IU); C3 52(Normal - 90-180 mg/dl); C4 16 (Normal - 10-40mg/dl), and CH50 of 62 (Normal - 150-250U/ml). Twenty-four hours protein collection revealed 1.075 g of proteinuria. Owing to persistent activity of her sediment as well as abnormal serologic tests (Table 1), the frequency of the cyclophosphamide infusions were increased from every 3 months to every month, and a repeat biopsy was performed in December of 1998.