LAUSR

A 34year-old Hispanic woman with a past medical history of obesity presented with a 2-day history of epigastric abdominal pain accompanied by nausea and vomiting, arthralgias, periorbital edema and a flat, erythematous, pruritic bilateral rash on her legs and left arm. Rash had occurred on and off for over 2 years and began following pregnancy. She reported that it was usually precipitated by stress, occurred in random areas of her body, and disappeared after 3–4 days. She had hematuria and a “foamy” appearance to her urine. Family history is positive for SLE in her cousin, aunt and grandmother. At referral, blood pressure was 167/77 mmHg and she had tenderness to palpation in the epigastric region and right upper quadrant with no rebound or guarding. Besides the erythematous skin rash, pitting edema was noted in the lower extremities with an associated 25 lb. weight gain. Laboratory data showed BUN of 25 mg/dl, creatinine of 1.6 mg/dl, and albumin of 1.8 g/dl. Urinalysis revealed proteinuria of 300 and RBC > 182. Computerized tomography of the abdomen/pelvis showed bilateral pleural effusions, small amount of ascites, and mild retroperitoneal pelvic and inguinal adenopathy. Creatinine subsequently increased to 3.2 mg/dl. Spot urine protein to creatinine ratio was 7.5 g/g. Serologic data showed hypocomplementemia with C3 of 38 mg/dl (Ref 83-193 mg/dl), C4 of 4.5 mg/dl (Ref 15-57 mg/dl) and C1q of 1.8 mg/dl (Ref 11.8-24.4 mg/ dl). Anti-C1q antibodies were positive. ANA, lupus anticoagulant, ANCA, anti-Smith, RNP, rheumatoid factor and cryoglobulins were negative. A kidney biopsy was performed, but given the high index of suspicion for Lupus Nephritis, she was treated empirically with pulse steroid doses of Solumedrol 1 g i.v.daily for 3 days followed by Prednisone 1 mg/kg daily (80 mg). The kidney biopsy showed a “full house pattern” on immunofluorescence with diffuse endocapillary and extracapillary proliferative glomerulonephritis consistent with crescentic lupus nephritis class IV. Cellular crescents were found in 11 of 14 glomeruli along with interstitial inflammation with diffuse and severe injury, minimal (5%) tubular atrophy and interstitial fibrosis and focal arterial edema. Activity index according to NIH criteria was 19 (Scale 0–24) with a chronicity index of 1 (scale 0–12) (Figs. 1 and 2). Due to financial constraints she was started on Mycophenolate Mofetil 3 g daily, steroids were tapered and she was discharged with instructions for close outpatient follow-up. Two weeks later her creatinine had improved to 2.0 mg/dL. At 6-month follow-up, she did not have recurrence of rashes but had episodic arthralgias. Serum creatinine was 1.2 but urine sediment remained active, complement levels were still very low.