With regard to muscle biopsy results in IBM, both presenters explained that typical findings may include endomysial inflammation, deposits of amyloid-beta protein, rimmed vacuoles and a loss of muscle fibers. In expanding on this challenging case, Dr. Lundberg stated that it is sometimes helpful to repeat a muscle biopsy, especially if the site of the repeat biopsy can be guided by MRI of the muscles, with areas of muscle edema being key sites to sample and areas with atrophic muscles being the locations to avoid.
A Case with Many Complications
The third case discussed in this session was that of a 55-year-old woman with polyarthralgias, rash, dyspnea on exertion, fever and weight loss. She was found to have anti-MDA-5 antibody-positive dermatomyositis and a chest CT showed ILD. The ensuing treatments used in her care included glucocorticoids, rituximab, cyclophosphamide, tacrolimus and nintedanib.
Unfortunately, she developed hypoxic respiratory failure and required intravenous immunoglobulin and plasma exchange, followed by treatment with mycophenolate mofetil and tacrolimus. After this prolonged course of aggressive treatment, she developed ophthalmologic complications that were ultimately found to be due to cytomegalovirus retinitis.
In discussing this patient’s case, Dr. Wang stated that no clear evidence-based strategy exists for such complicated cases. He explained that many patients develop infections and some even die from such infections after aggressive immunosuppression. In his own practice, he rarely uses pulse steroid therapy and more typically uses 1 mg of prednisone per kg body weight per day.
Dr. Lundberg further stated that for patients like this consideration of extracorporeal membrane oxygenation or lung transplantation may need to be discussed when lung disease is severe. She also explained that no clear studies exist on the effects of intravenous immunoglobulin in severe ILD in patients with myositis, and the role of plasmapheresis in treating such patients is also unclear.
On this note, Dr. Wang did point to a recent trial evaluating the efficacy of plasma exchange in patients with anti-MDA-5 rapidly progressive ILD. In the study he referenced, patients with anti-MDA-5 rapidly progressive ILD have a poor rate of one-year transplant-free survival, and the use of plasma exchange was not associated with a better outcome. However, this treatment was used mainly in patients with more severe disease.1
Bridging Gaps in Global Access to Rheumatology Care
In the next portion of the summit, Mohammed Tikly PhD, FRCP, MMed, MBBCH, MACR, professor emeritus, Division of Rheumatology, University of the Witwatersrand, Johannesburg, South Africa, spoke to the challenges of accessing rheumatologic care in countries like his own.
