What is the primary condition associated with anti-topoisomerase antibodies?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Anti-topoisomerase antibodies, particularly anti-Scl-70 antibodies, are primarily associated with diffuse systemic scleroderma (also known as systemic sclerosis). This autoimmune condition is characterized by the overproduction of collagen leading to fibrosis in the skin and internal organs. The presence of anti-topoisomerase antibodies indicates a severe form of the disease that often involves rapid progression and widespread skin involvement, alongside potential complications affecting internal organs such as the lungs, kidneys, and gastrointestinal tract.

In contrast, while drug-induced lupus erythematosus can lead to various antibody formations, including anti-nuclear antibodies, it is not strongly linked to anti-topoisomerase antibodies. Primary biliary cirrhosis is associated with anti-mitochondrial antibodies, and various forms of vasculitis can involve other specific autoantibodies, such as anti-neutrophil cytoplasmic antibodies (ANCA), but not typically anti-topoisomerase. Therefore, diffuse systemic scleroderma is the condition most accurately identified in relation to the presence of anti-topoisomerase antibodies.

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