BIRMINGHAM, ENGLAND — Tumor necrosis factor blockade may offer a successful therapeutic alternative to high-dose corticosteroids in the rare, potentially life-threatening occlusive vasculitis known as Takayasu's arteritis.
This large-vessel vasculitis typically involves the aorta and its main branches, causing stenosis or even obstruction. It occurs most commonly in young women.
In a case report presented at the joint meeting of the British Society for Rheumatology and the German Society for Rheumatology by Lucy E. Coates, M.D., of the Royal National Hospital for Rheumatic Diseases in Bath, England, a 17-year-old female patient developed severe left-sided facial pain that radiated into her neck and left arm.
Initially, the symptoms were thought to be musculoskeletal in origin, and she was treated with physiotherapy, analgesics, and tricyclic antidepressants.
Subsequent investigations, however, revealed the presence of markedly elevated inflammatory markers, including a plasma viscosity of 2.41 millipascal seconds (normal range 1.50–1.70 mPa·s).
She also had microcytic anemia, and levels of immunoglobulins were raised, with IgG at 17.4 g/L, IgA at 4.2 g/L, and IgM at 3.8 g/L. Various imaging studies were done, including MRI of the brain and cervical spine and CT of the abdomen and pelvis, without result.
Two years later, a lump was discovered in her neck. On examination, she was found to have bilateral carotid bruits. Magnetic resonance angiography (MRA) results showed thickening in the wall of the right brachiocephalic artery, narrowing the vessel. The subclavian artery was narrowed at its origin and underfilled distal to its origin. This was suggestive of a proximal stenosis such as is seen in Takayasu's arteritis, Dr. Coates said.
The patient was started on prednisone and azathioprine, but 1 year later her symptoms had worsened, as had the imaging findings on repeat MRA. The decision was made to institute more aggressive therapy because of concerns that the blood vessels supplying her brain were being affected, Dr. Coates said in a poster session.
Infliximab treatment was begun, with infusions of 5 mg/kg at weeks 0, 2, 6, and 10 and then every 6 weeks.
She also continued to take azathioprine (100 mg/day) and prednisone (20 mg/day). Symptomatic improvement was immediate, and another MRA the following year showed significant improvement in the caliber and appearance of the aortic arch vessels.
“Anti-TNF therapy appears to offer a promising alternative in Takayasu's arteritis, particularly if other forms of immunosuppression fail to control progression,” Dr. Coates said in her poster presentation.
Her findings support those seen in another recent series in which 14 of 15 patients with the condition responded and 10 experienced sustained remission and were able to discontinue glucocorticoid therapy (Arthritis Rheum. 2004;50:2296–304).