Vasculitis in primary Sjögren's syndrome. Histologic classification and clinical presentation

Abstract

Nine out of 70 patients with primary Sjögren's syndrome had vasculitis of small and/or medium-sized vessels. The small vessel vasculitis was of the hypersensitivity type: leukocytoclastic and lymphocytic. The medium vessel vasculitis was acute necrotizing and simulated polyarteritis nodosa histologically but lacked the characteristic aneurysmal formation. A healing stage of vasculitis, presenting as endarteritis obliterans, was seen in patients with Sjögren's syndrome and a long-standing history of vasculitis. The hypersensitivity vasculitis was localized mainly in the skin and provided mild clinical symptoms. The polyarteritis nodosa-like vasculitis was localized predominantly in internal organs and was associated with life-threatening symptoms. Endarteritis obliterans was mainly seen in acral sites of the extremities and presented with recurrent symptoms of obstruction. One patient died of vasculitis. The remaining patients are alive. The presence of anemia, cryoglobulinemia, and hypocomplementemia suggested an immune-complex origin of the vasculitic process.