Sjogren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis.
We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A 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 IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.
Tsai
Tsung-Chang
TC
Division of Nephrology, Department of Medicine, Tian-Sheng Memorial Hospital, Tong-Kang, Ping-Tong, Taiwan.
Chen
Chen-Yin
CY
Lin
Wei-Tung
WT
Lee
Wei-Jen
WJ
Chen
Hung-Chun
HC
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001707:Biopsy, Needle; D005260:Female; D005500:Follow-Up Studies; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007677:Kidney Function Tests; D006435:Renal Dialysis / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D016896:Treatment Outcome; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
D000305:Adrenal Cortex Hormones