“In other words, patients with systemic sclerosis probably have quite pronounced microangiopathy early in their disease despite the fact that, with our normal tools, [they] lack clinical signs of that,” Dr. Walker said. “And I think that should be investigated.”
In the case of this 49-year-old patient, he noted, anticentromere antibodies were found, even though they’ve been shown to be protective against myositis—making this case “somewhat puzzling.”
Calcinosis with SSc
Another case-based presentation focused on calcinosis associated with SSc. The cases, out of the Royal Free Hospital in London, covered an array of severity and contexts for calcinosis.
There was a case of diffuse SSc with rheumatoid arthritis overlap, in which the patient had calcific periarthritis with significant functional problems. The patient was treated with adalimumab. A case of limited SSc, with recalcitrant digital calcinosis and ulceration, was treated with surgery.
A case of limited SSc was treated with minocycline and surgery, with surgery bringing the biggest benefit. Another case of limited SSc, but with debilitating calcinosis, has required large doses of opioids for the patient’s pain.
“Calcinosis is a really major morbidity for patients with systemic sclerosis”—with a diversity of ways to cause problems, said Christopher Denton, MD, professor of experimental rheumatology at Royal Free Hospital and University College of London Medical School.
Treatment is a challenge, and some of the cases show the reliance on surgery, Dr. Denton said. “Surgical management … really is perhaps the only approach to therapy that has unequivocal benefit in the right patient group,” he said. Minimally invasive approaches, using a dental drill or laser excision, have proven effective, he said.
Calcinosis can be assessed with radiography, CT scanning, ultrasound, and isotope bone scanning. But Dr. Denton stressed the importance of keeping function in mind. “We have got to look at the impact of the calcinosis, not just the extent and anatomical location,” he said. “Because it’s really the functional impact that is important and that our patients are troubled by.” This was seen in the case of the patient who had limited SSc, but whose calcinosis left her wheelchair bound.
The diversity of the calcinosis cases makes it hard to identify a unified approach. One attempt, detailed in a forthcoming article by Herrich et al (Rheumatology, in press) divides calcinosis into mild, moderate, and severe, depending on the density of the calcification and the number of sites, although Dr. Denton said the approach can be problematic because the effects can be devastating even in cases not classified as severe.
