Thanks, Tom Bolig, for presenting on idiopathic inflammatory myopathy-associated ILD, focusing on anti-MDA5 ILD.
My takeaways:
- while a dermatomyositis, there are mild to no muscle symptoms; there are hallmark cutaneous manifestations though
- is important to test for specific antibodies and involve Rheumatology early
- lower overall survival and higher risk for developing a rapidly-progressive ILD
- may be triggered by anti-TNFalpha therapy; some mimicking of psoriatic arthritis
- more specific cutaneous phenotypes are skin ulcerations (OR 18) and palmar papules
- no guideline-recommended treatment but common to see combination immunosuppression such as glucocorticoid with calcineurin inhibitors (tacrolimus)
- there may be benefit to triple therapy up front compared with stepwise
- salve therapy with plasma exchange is promising, as are use of rituximab and tofacitnib
Thanks, Tom!