https://doi.org/10.1351/goldbook.13711
Fibrosing disease of unclear etiology that affects multiple organ systems.
Notes:
- The skin ("scleroderma") and blood vessels (arteries, small vessels) are most commonly affected, but involvement of the lungs and gastrointestinal tract (esophagus) may also be observed.
- Anticentromere antibodies (ACA) as well as autoantibodies against DNA topoisomerase I (scl-70) and various nucleolar antigens are diagnostic and prognostic markers and are often detectable years before disease manifestation. They are also detectable in quartz dust-exposed individuals.