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Polymyalgia Rheumatica with Bilateral Subclavian Artery Stenosis

      To the Editor:
      The overlap between polymyalgia rheumatica and giant cell arteritis has prompted physicians' attention since the early 1970s.
      • Thompson J.R.
      • Simmons C.R.
      • Smith L.L.
      Polymyalgia arteritica with bilateral subclavian artery occlusive disease.
      • Hamrin B.
      Polymyalgia arteritica.
      Polymyalgia rheumatica occurs in approximately 50% of patients with giant cell arteritis, and approximately 15% of patients with polymyalgia rheumatica develop giant cell arteritis.
      • Brooks R.C.
      • McGee S.R.
      Diagnostic dilemmas in polymyalgia rheumatica.
      Some experts consider polymyalgia rheumatica and giant cell arteritis to be different manifestations of the same pathologic process.
      • Roche N.E.
      • Fulbright J.W.
      • Wagner A.D.
      • et al.
      Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis.
      Although the association of occlusive vascular disease is well known in giant cell arteritis, it is not considered to be a typical manifestation of polymyalgia rheumatica.
      A 66-year-old woman presented to our rheumatology practice in May 2007 with proximal muscle pain and stiffness of 1 year's duration. The patient also reported acute onset of tingling with bluish discoloration in the nailbeds and fingertips of her left hand, exacerbated with overhead activity. She denied any headaches, vision changes, scalp tenderness, or jaw claudication. Physical examination revealed warm hands and good capillary refill in all digits despite a diminished right radial artery pulse and nonpalpable left radial artery pulse. She had good pulsation without tenderness over the temporal arteries. Laboratory test results showed a normal complete blood count, elevated erythrocyte sedimentation rate of 47 mm/H, C-reactive protein of 32.4, negative rheumatoid factor and antinuclear antibody, and normal creatine phosphokinase.
      Polymyalgia rheumatica was diagnosed, and the patient was started on prednisone 10 mg twice per day for 1 week, followed by 10 mg daily. There was significant improvement in her proximal arthralgias and myalgias, but left arm claudication persisted. Doppler ultrasound of the upper extremities revealed bilateral moderate (50%-74%) stenosis of the subclavian, axillary, and brachial arteries. Magnetic resonance angiography of the aortic arch with gadolinium disclosed bilateral severe subclavian artery stenosis, distal to the origin of the vertebral arteries, with the axillary and brachial arteries not well demonstrated because of the severe proximal stenosis of the subclavian arteries (Figure).
      Figure thumbnail gr1
      FigureBilateral severe subclavian artery stenoses. These are approximately 2.5 cm beyond the origin of the subclavian artery from the brachiocephalic artery on the right and 4.5 cm from the origin of the subclavian artery of the aortic arch on the left.
      Although the patient had no cranial symptoms, giant cell arteritis was suspected to be the cause of occlusive vasculopathy. Treatment with prednisone 60 mg daily showed dramatic improvement in left arm symptoms. In August 2007, the patient underwent angiography followed by angioplasty and stenting of the left subclavian artery. Postoperatively, the patient was administered aspirin because of clopidogrel bisulfate (Plavix; Bristol-Myers Squibb, New York, NY) and aspirin with extended-release dipyridamole (Aggrenox; Boehringer-Ingelheim, Ingelheim, Germany) intolerance. The upper-extremity arterial Duplex ultrasound revealed a patent left subclavian stent. The patient was doing well, maintained with an average dose of 5 mg of prednisone per day. In February 2008, she presented with return of left arm claudication, discoloration, and absent left radial artery pulse. Noninvasive vascular testing demonstrated severe narrowing of the subclavian artery distal to the left subclavian stent. We concluded that the patient had vasculitis, most likely secondary to giant cell arteritis, evidenced by recurrent occlusive disease in the upper extremities. The decision was made to control the disease with steroid treatment and no further surgical interventions unless absolutely necessary.
      This case underlines the fact that physicians should be vigilant when assessing patients with polymyalgia rheumatica. Polymyalgia rheumatica and giant cell arteritis share pathogenic principles and are not mutually exclusive but overlapping.
      • Hamrin B.
      Polymyalgia arteritica.
      The challenge is to recognize atypical cases presenting with occlusive vasculopathy but lacking cranial symptoms suggestive of giant cell arteritis. Patients with occlusive arterial disease caused by giant cell arteritis typically respond well to a high dose of steroids. Surgery should be restricted to cases with severe persistent ischemia after the inflammatory syndrome has receded.
      • Ninet J.P.
      • Bacet P.
      • Dumontet C.M.
      • et al.
      Subclavian and axillary involvement in temporal arteritis and polymyalgia rheumatica.

      References

        • Thompson J.R.
        • Simmons C.R.
        • Smith L.L.
        Polymyalgia arteritica with bilateral subclavian artery occlusive disease.
        Radiology. 1971; 101: 595-596
        • Hamrin B.
        Polymyalgia arteritica.
        Acta Med Scand Suppl. 1972; 533: 1-131
        • Brooks R.C.
        • McGee S.R.
        Diagnostic dilemmas in polymyalgia rheumatica.
        Arch Intern Med. 1997; 157: 162-168
        • Roche N.E.
        • Fulbright J.W.
        • Wagner A.D.
        • et al.
        Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis.
        Arthritis Rheum. 1993; 36: 1286
        • Ninet J.P.
        • Bacet P.
        • Dumontet C.M.
        • et al.
        Subclavian and axillary involvement in temporal arteritis and polymyalgia rheumatica.
        Am J Med. 1990; 88: 13-19