Case 486 -- A 10-year-old male with decreased complement

Contributed by Ibrahim Batal, MD and Bruce Rabin, MD, PhD
Published on line in November 2006

The patient is a 10 year old male who was active and feeling well until July 2006 when he presented to the hospital with a two week history of low grade fever and fatigue, this was accompanied by a facial rash with malar distribution, a livedo reticularis rash over all his extremities, and a painless oral ulceration. No joint pain or swelling was appreciated, and no family history of autoimmune disease was reported.

Lab results from an outside hospital showed +3 blood in the urine. BUN and Cr were within the normal limit. Hematology work up revealed normocytic anemia and lymphopenia, and thyroid function tests showed only slightly elevated TSH at 5.9 U/ml.

Immunology lab results at UPMC revealed:

The patient was given high doses of steroids (60 mg/day) with good response regarding both anemia and hematuria. The plan was to follow up this patient every 2 months with CBC, urinalysis, ANA, and complement levels with a goal of tapering the steroid doses down to approximately 0.25 mg/kg/day if the improvement continued.


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