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Hidden Giant: Medium Vessel Vasculitis as a Cause for Unresolving Fever

Hidden Giant: Medium Vessel Vasculitis as a Cause for Unresolving Fever

A 71-year-old man with a history of Parkinson’s disease experienced a sudden decline in function, with multiple falls over 2 weeks. He had previously been able to ambulate independently but had become chair-bound over this period.

Forty-eight hours into admission he was noted to be spiking fevers of 38°C-39°C daily, without chills or rigors. There were no localizing symptoms for an infective source, although he was intermittently confused and delirious. There had not been any recent travel, and he had no sick contacts.

Assessment

Physical examination revealed a pill-rolling tremor and hypomimia, consistent with Parkinson’s disease. Results from cardiopulmonary and abdominal examinations were unremarkable. There was no clinical evidence of meningism. There was no clinical evidence of autoimmune disease.

Laboratory studies revealed leukocytosis of 17.63 × 109/L (predominantly neutrophils, with no eosinophilia), normochromic normocytic anemia (hemoglobin 9.1 g/dL), and thrombocytosis of 409 × 109/L. His C-reactive protein and erythrocyte sedimentation rate were significantly elevated at 241 mg/L and >145 mm/h, respectively.

Blood and urine cultures did not yield any bacterial growth. Human immunodeficiency virus screening and a blood film for malaria parasites were both negative. Cerebrospinal fluid was acellular, with normal protein and glucose levels. Bacterial culture yielded no growth, and a polymerase chain reaction panel looking for the common pathogens responsible for meningo-encephalitis (including Escherichia coliHaemophilus influenzae, herpes simplex virus type 1, herpes simplex virus type 2, Listeria monocytogenesNeisseria meningitidis, human herpesvirus 6, Streptococcus agalacticae, varicella-zoster virus, cytomegalovirus, and cryptococcus) was also negative.

Magnetic resonance imaging of the brain did not reveal any masses, restricted diffusion, or leptomeningeal enhancement. Computed tomography of the thorax, abdomen, and pelvis and magnetic resonance imaging of the spine did not reveal any occult malignancy or infective collections.

The antinuclear antibody titer was strongly positive (at a dilution of 1:640, speckled pattern). Complement and double-stranded deoxyribonucleic acid levels were normal. Anti-myeloperoxidase and anti-proteinase 3 antibodies were not detected.

After 2 weeks of inpatient investigation, there continued to be no clear explanation for his symptoms. He continued to spike temperatures daily despite treatment with broad-spectrum antibiotics, which were stopped subsequently.

On further examination, prominent pulsations were noted over both temporal arteries. There was no report of jaw claudication or of any visual loss. This, together with the high erythrocyte sedimentation rate and positive antinuclear antibody titer, raised the suspicion for an underlying vasculitis. He was referred for a temporal artery biopsy, which revealed a chronic medium-sized vasculitis.

Microscopic sections showed a medium-sized artery with intramural chronic inflammatory infiltrate consisting of lymphocytes, plasma cells, histiocytes, and occasional eosinophils (Figure). No definite giant cells were identified. There was associated thickening of the intima and asymmetrical fibrosis of the vessel wall, with patchy destruction of the elastic laminae.

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-Rachel Choe, MBBS, Benjamin Hooi, MBBS, MMED (Int Med), MRCP (UK), Hui Min Tan, MBBS, Garvi Pandya, MBBS, MD, D.N.B., MRCP (UK)

This article originally appeared in the May issue  of The American Journal of Medicine.

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