Antineutrophil cytoplasmic antibody-associated vasculitis in a patient with diffuse scleroderma

• Main Authors: B N Shiva Prasad, H A Karthik Urala
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2021-01-01
• Series: Indian Journal of Rheumatology
• Subjects: antineutrophil cytoplasmic antibody-associated vasculitis; antimyeloperoxidase antibodies; diffuse scleroderma; neuropathy
• Online Access: http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2021;volume=16;issue=2;spage=217;epage=220;aulast=Shiva

Scleroderma is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with scleroderma is a rare association. Renal and pulmonary complications occur more frequently than expected in scleroderma/ANCA-associated vasculitis overlap. The definitive diagnosis usually made based on autoantibody titers and biopsy of involved tissue. We present a case of diffuse scleroderma with ANCA-associated vasculitis presenting with neurological symptoms.