title: ANCA-associated vasculitis in systemic sclerosis report of 3 cases.
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.
authors.affiliation: Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, ON, Canada.
authors.forename: Mohammed A
authors.initials: MA
authors.lastname: Omair
authors.forename: Nasreen
authors.initials: N
authors.lastname: Mohamed
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
authors.forename: Zareen
authors.initials: Z
authors.lastname: Ahmad
authors.forename: Peter
authors.initials: P
authors.lastname: Lee
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006417:Hematuria / Q000150:complications; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications; D008875:Middle Aged; D011507:Proteinuria / Q000150:complications; D012074:Remission Induction; D051437:Renal Insufficiency / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids