Multiple Therapies for MDA5
Anti-melanoma differentiation association gene 5 (MDA5) dermatomyositis is a subset of myositis-related ILD that must be caught early and treated aggressively. When rapidly progressing, ACR guidelines call for IV glucocorticoids and two additional therapies, such as rituximab, cyclophosphamide, IVIG, calcineur inhibitor or JAK inhibitors.7
“Which two you pick will be a mix of what your institution or the patient’s insurance company lets you have, what you are comfortable using and what is right for your patient,” said Dr. Wilfong. “There is literature supporting all of three.”
She cautions that aggressive immunosuppression can increase infection and worsen outcomes, so this therapy may not be appropriate for all patients.
ILD in Pregnancy
A group that needs attention are women who are pregnant or desire a pregnancy.
“When we think about pregnancy, it is critical our patients feel safe talking to us about having a child,” said Dr. Wilfong. “At the end of the day, they can get pregnant with or without us. It is safer for both mom and baby if done as a team.”
The first concern is reevaluating medications and moving away from teratogens. These should be addressed before pregnancy to ensure the disease remains stable.
Depending on the mom’s disease severity, counseling should note that as ILD severity increases, so does the risk of miscarriages and preterm births. It is imperative the mother is evaluated for pulmonary hypertension, which increases morbidity and mortality in both the mother and the fetus.8
In addition to reevaluating medication regimens, the rheumatologist should stay in close consultation with the patient’s other physicians. Also, plan to increase pulmonary monitoring because the goal for oxygen saturation in pregnancy is 95%.
She suggests her patients get a pulse oximeter for home use, which makes it possible to gather information on what happens, for instance, when a patient walks upstairs or in other real-world situations.
How to Define PPF?
There is some controversy in deciding on a definition for progressive pulmonary fibrosis (PPF). For example, the INBUILD study criteria included a ≥10% decline in forced vital capacity (FVC), a ≥5% FVC decline with worsening respiratory symptoms, a ≥5% FVC decline with worsening HRCT, or worsening of both respiratory symptoms and HRCT.9
“We need to acknowledge up front that the most important indicator of PPF is FVC decline of greater than 10%,” said Justin Oldham, MD, PhD, an associate professor at the University of Michigan. “Many studies of different processes show FVC had very strong prognostic significance compared to other markers.”
