Background Sjögren’s syndrome (SS) is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren’s syndrome patients also have disease in other organs.
Objectives To study the cases of SS associated with MPO-ANCA-positive polyangiitis.
Results We report 4 cases (were female) with Sjögren syndrome developed microscopic polyangiitis. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness and characteristic appearance of a biopsy sample from a minor salivary gland, and presence of anti-SS-A autoantibody. The patient also tested positive for myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA), with changes in the MPO-ANCA titer that paralleled changes in the symptoms. Histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was necrotizing crescentic membranous glomerulonephritis. The patients were treated with oral glucocorticoids in combination with cyclophosphamide and with local symptomatic therapy for ocular and oral dryness. Renal function stabilized after 2 months, and tests for anti-myeloperoxidase reverted to negative.
Conclusions SS is one of the most common autoimmune disorders and vasculitis is one of the most characteristic extraglandular manifestations, but wide spectrum of cutaneous involvement in SS has been little studied.
Disclosure of Interest None Declared
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