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ANCA-associated vasculitis in systemic sclerosis report of 3 cases

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Abstract

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.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, ON, Canada

    Mohammed A. Omair, Sindhu R. Johnson, Zareen Ahmad & Peter Lee

  2. Department of Pathology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, ON, Canada

    Nasreen Mohamed

  3. Rebecca MacDonald Centre for Arthritis and Autoimmune Disease, Scleroderma Clinic, Mount Sinai Hospital, University of Toronto, 60 Murray Street, Box 9, Toronto, ON, M5T 3L9, Canada

    Peter Lee

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  1. Mohammed A. Omair
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  2. Nasreen Mohamed
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  3. Sindhu R. Johnson
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  4. Zareen Ahmad
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  5. Peter Lee
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Correspondence to Peter Lee.

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Source of support

Dr. Sindhu Johnson has been awarded a Canadian Institutes of Health Research Clinician Scientist Award and is supported by the Norton-Evans Fund for Scleroderma Research.

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Omair, M.A., Mohamed, N., Johnson, S.R. et al. ANCA-associated vasculitis in systemic sclerosis report of 3 cases. Rheumatol Int 33, 139–143 (2013). https://doi.org/10.1007/s00296-011-2359-z

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  • DOI: https://doi.org/10.1007/s00296-011-2359-z

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