ANCA-associated vasculitis in systemic sclerosis report of 3 cases

作者：Omair Mohammed A; Mohamed Nasreen; Johnson Sindhu R; Ahmad Zareen; Lee Peter^*

来源：Rheumatology International, 2013, 33(1): 139-143.

DOI：10.1007/s00296-011-2359-z

• Systemic sclerosis
• Scleroderma
• Anti-neutrophil cytoplasmic antibody-associated vasculitis
• Acute nephritis

The aim of the study was to describe the occurrence of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) in systemic sclerosis (SSc) patients. SSc patients who developed biopsy-proven AAV were identified. Their clinical manifestations, autoantibodies, presentation with vasculitis, treatment and outcome were described and compared with previously reported patients with these two conditions. Of 985 patients, 3 were identified. All patients had interstitial lung disease, and all presented with acute renal failure, proteinuria and hematuria, and were P-ANCA- and anti-Scl-70-positive. One required hemodialysis. Two were hypertensive; additionally, one patient had sinusitis, and another had monoarthritis and a macular rash. All were treated with high-dose corticosteroids and responded to therapy and attained remission at 6 months. At 1 year, one patient died of pneumonia. ANCA-associated vasculitis is a rare but serious finding in SSc patients. Positive anti-Scl-70 antibody is found commonly in these patients. Different treatment modalities are effective. Serious infections can complicate therapy and lead to death.

• 出版日期2013-1