Fever, Headache, and Nodular Lesions: Can You Make the Diagnosis?

Stephen Paget, MD

Disclosures

February 22, 2011

Patient Profile

I recently saw a 56-year-old Russian American man who presented for a second opinion with regard to the future treatment of giant cell arteritis.

At the age of 21, while still in Russia, the patient developed Henoch-Schönlein purpura manifested by palpable purpura on the lower extremities, arthritis, and abdominal pain. Eventually, this resolved after he was treated with a short course of prednisone. As a child, the patient was treated with Bacillus Calmette-Guerin (BCG).

One year ago, the patient developed the onset of a burning sensation in his scalp that resolved in 1 week. Six months ago the patient developed a temperature to 101, severe frontal headache, severe fatigue, eye pain, and chills with some mild blurriness of vision. He denied proximal aches or soreness. In the setting of a workup, the patient was found to have Waldenström macroglobulinemia with an elevated immunoglobulin (Ig)M level of 1500 and a bone marrow showing low-grade B-cell lymphoma with plasmacytic components with findings of a clonal IgM lambda plasma cell population. Cryoglobulins were negative, serum viscosity was normal, white count was 11,000, hematocrit 36%, hemoglobin 12.6, platelet count 316,000, sedimentation rate was 33, and C-reactive protein was elevated at 6.8. No specific therapy was recommended for the patient's macroglobulinemia.

The patient had a CT scan of the chest, abdomen, and pelvis, which showed no adenopathy or other abnormality. A bilateral temporal artery biopsy was performed; it was normal on the right, and the left showed 5-10 lymphocytes in adipose tissue adjacent to the artery. Patchy disruption of the internal elastic lamina was seen, and healed inactive giant cell arteritis could not be excluded. No amyloid deposits were seen.

The patient was started on prednisone 30 mg/day with a rapid and marked overall improvement and disappearance of fever, night sweats, eye pains, and headache. When the patient's prednisone was tapered over the next 3 months to 5 mg/day, all of the patient's symptoms recurred including headache, night sweats, fever, and a sedimentation rate of 60. The patient was placed back on prednisone 20 mg/day with control of all symptoms and full function.

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