Neurovascular mechanisms as a possible cause of remission of rheumatoid arthritis in hemiparetic limbs.

C holesterol emboli syndrome (CES) can be confused with a small vessel vasculitis but it is unusual for it to be mistaken for giant cell arteritis. We report a patient with temporal headaches, transient visual loss, and raised inflammatory indices, which led to an initial diagnosis of giant cell arteritis, but in whom CES was the eventual explanation. A 64 year old woman with treated hypertension who smoked was referred with a 1 year history of left sided temporal headaches and pain in the proximal lower limbs. The headaches were intermittent but were increasing in frequency and intensity. She also described two episodes of blurred vision of short duration in the left eye. There were no other associated neurological symptoms. She gave a history of Raynaud's phenomenon in both hands and worsening activities of daily living limited by fatigue. She was overweight with a body mass index of 27 kg/m 2 ; there were no mucocutaneous abnormalities. The temporal arteries were not palpable and a fundoscopic examination was normal. All peripheral pulses were present and there were no nailfold capillary changes. She had no tender or swollen joints; there was functional range of movement in axial and peripheral joints. Laboratory investigations showed haemoglobin of 127 g/l, a white cell count of 10.7610 9 /l with a normal differential, and 625610 9 /l platelets. The erythrocyte sedimentation rate (ESR) was 60 mm/1st h and C reactive protein (CRP) 290 mg/l. She had a serum urea and creatinine of 5.5 mmol/l and 96 mmol/l, respectively, with normal urine microscopy. The liver enzymes were not raised. Rheumatoid and antinuclear factor were absent. Based on her symptoms and raised inflammatory indices, a provisional diagnosis of temporal arteritis was made and treatment was started with prednisolone 50 mg daily. A left temporal artery biopsy showed atherosclerotic change without evidence of granulomatous inflammation. This negative result was attributed to sampling error. She continued to have multiple episodes of transient monocular visual loss which were highly suggestive of amaurosis fugax. She had also developed more troublesome acrocyanosis, livedo reticularis, and had a blood pressure of 190/83. The neurological examination was normal and there were no audible carotid bruits. Urine analysis remained negative, with normal renal function and inflammatory indices, but the total cholesterol was raised at 7.4 mmol/l. Her ECG did not suggest a rhythm abnormality. Formal fundoscopic examination showed a cholesterol crystal lodged in a vessel …