Systemic Sclerosis: More Than Skin Deep

Updates from the ACR Convergence 2023 Review Course, part 6

SAN DIEGO—The pre-conference Review Course at ACR Convergence 2023, held Saturday, Nov. 11, and moderated by Noelle Rolle, MBBS, assistant professor in the Division of Rheumatology, associate program director of the Rheumatology Fellowship at the Medical College of Georgia, Augusta University, and Julia Schwartzmann-Morris, MD, associate professor, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, N.Y., tackled numerous important topics in rheumatology. Here, we report on the presentation by Francesco Boin, MD, professor of medicine, director, Division of Rheumatology, Cedars-Sinai, Los Angeles.

Dr. Boin

Dr. Boin provided an overview of how and when to screen for organ involvement in patients with systemic sclerosis (SSc). Dr. Boin noted that the evaluation of the patient with SSc always begins with the skin and with classifying the patient as having limited or diffuse cutaneous disease. Regardless of the degree of skin involvement, it’s important to realize that any patient with SSc can have multi-organ manifestations. Indeed, the risk of organ complications is higher early in the disease course than later.¹

Autoantibodies in SSc

An understanding autoantibodies in SSc can help clinicians predict manifestations and prognosis in patients:²

Anti-centromere antibodies:

• Are associated with limited cutaneous disease;

• Are more often seen in patients who are white;

• Confer a higher risk of vascular disease, such as pulmonary hypertension, compared with patients with scleroderma without this autoantibody;

• Are associated with thyroid disease and primary biliary cholangitis; and

• Are protective against interstitial lunge disease (ILD).

Anti-Scl-70 antibodies (also known as topoisomerase) are more often seen in patients who are Black and can be associated with an increased risk of ILD, advanced contractions and myocarditis.

Anti-RNA polymerase III antibodies can increase the risk of rapid skin progression, renal involvement—specifically scleroderma renal crisis—gastric antral vascular ectasias (GAVE) and cancer.

Anti-U1RNP antibodies are more common in patients who are Black, increase the risk of ILD and are often seen with overlap of connective tissue disease manifestations.

Antibodies to Anti-PM/Scl antibodies can be seen in young patients and can be associated with myositis and calcinosis, but these patients do not have an elevated risk of malignancy.

Finally, anti-U3RNP antibodies can be associated with the risk of pulmonary hypertension, severe gastrointestinal disease, ILD and pericarditis.

SSc with ILD

ILD is more commonly seen in patients with diffuse than limited cutaneous SSc. Most decline in lung function occurs in the first two to four years of disease onset. Early on, ILD may be silent, and when shortness of breath is present, it is important to rule out other causes, such as anemia, deconditioning, muscle weakness or aspiration due to chronic cough from gastroesophageal reflux disease.