Refractory Anti-HMGCR Necrotizing Myopathy With Progressive Hypercapnic Respiratory Failure Responsive to a JAK Inhibitor. (PubMed, Case Rep Rheumatol) - Oct 21, 2026 - "Herein, we report the case of a 73-year-old woman with long-standing anti-HMGCR antibody-positive IMNM, refractory to glucocorticoids, methotrexate, azathioprine, intravenous immunoglobulin (IVIg), rituximab, and plasma exchange (PLEX), who presented with progressive hypercapnic respiratory failure...The addition of tofacitinib, a Janus kinase (JAK) inhibitor, coincided with marked clinical improvement, discontinuation of MV, and successful transition to room air...The clinical benefit of JAK inhibition has been reported in dermatomyositis, overlap myositis, and anti-MDA5-positive disease, particularly in refractory cases with pulmonary or systemic involvement-but has not been described in the setting of refractory IMNM. In our patient, who had exhausted multiple conventional therapies and PLEX, the rapid clinical improvement after JAK inhibition strongly suggests a therapeutic effect and highlights the need for future studies of JAK inhibitors in IMNM."
Journal • Dermatomyositis • Immunology • Myositis • Respiratory Diseases • IFIH1
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A Nicolas; C Oddis; D P Ascherman; L Christopher-Stine; N Huang; T B George; V M Liarski
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