Thanks, Dr. Philippa van Dantzig for such a fascinating case at the Global Meet the Experts (Myositis)!!
#ACR - the experts weigh in: "for these cases, evaluation by a muscle pathologist is really important ... what types of inflammatory cells? muscle fibers? invasion by inflammatory T-cells for instance?"
DDx is really important for this type of pt - DDx should include IBM, Sarcoid, AAV, PM Immuno-mediated necrosis myopathy, Isolated myositis, Paraneoplastic. Dr. GuoChun Wang reemphasizes careful interpretation of the muscle biopsies.
*Isolated vasculitis (not isolated myositis)
Is there any utility in repeating a muscle biopsy?
According to the experts:
- May be helpful, esp if MRI-guided and biopsy is incongruent to clinical features
- Avoid necrotic areas, try to get edematous areas on MRI
Likelihood of paraneoplastic?
According to the experts:
- Necrotizing myositis and rare inflammatory infiltrate would be one sign on biopsy
- Serologies (e.g. TIFF1-gamma, NXP2) can be revealing too
Usefulness of EMG in this setting?
According to experts:
- Not specific for diagnosis so immune tests and biopsies are more important
- May be an indictor of a myopathy (to evaluate motor neurone disease, neuropathies, etc.)
Distal muscle weakness in myositis?
According to experts:
- Sarcoid has multiple forms (acute, chronic progressive, IBM-like) and so that may be potentially consistent
- IBM classically involves finger flexors (and may be much more assymetric than others)
- CK levels may help too
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Dr. Ramya Puligari presents the third case of myositis at #ACR22 Global Meet the Experts for Myositis
What to do next?
According to experts:
- No data out there for stopping rapidly progressive ILD in MDA5-Myositis
- Infectious risk must be balanced with enhanced immunomodulation. Combine with IVIg?
- Prednisone (1mg/kg)?
Also,
- Interprofessional collaboration with pulmonologists, radiologists, and others (get ready for ECMO, lung transplantation)
Interesting questions being posed for the Global Meet the Experts Myositis session #ACR22
Regarding Q1 (re: MDA5), according to experts:
- The presence of MDA5 should raise risk of ILD (get HR-CT)
- Careful reevaluation of muscle strength and endurance (repetition tests)
- Keep under tight observation
(The Myositis Functional Index - standardized repetitions for upper and lower extremities)