Two German teams report on their successful CAR-T cell treatment of refractory anti-Jo1-associated antisynthetase syndrome in two patients.
A first case reported by a team from Erlangen
- After an initial transient worsening (myalgia, CPK 13,600 U/L, fever for 3 days), the 41-year-old man’s condition quickly improved.
- Three months after the injection of CAR-T cells:
- lesions had disappeared on thigh muscle MRI;
- Oxygen weaning was complete and alveolar lesions had completely regressed on lung CT.
- At six months:
- his muscle strength was normal (MMT of 149/150),
- his muscular endurance allowed him to walk more than 5 km.
- Following the administration of CAR-T cells, the patient’s immunoglobulin (IgG) level, already low before treatment, slowly decreased, leading to the introduction of intravenous IgG replacement therapy once a month.
A second case reported by a team from Tübingen
- This 41-year-old man with anti-Jo1 antisythetase syndrome also benefited from treatment with anti-CD19 CAR-T cells, combined with 2g/day of mycophenolate mofetil from the 35th day after infusion.
- Clinical improvement was rapid after the infusion.
- Eight months later, his muscle strength (MMT8) and respiratory function were virtually normalised, his CPK level, CD8+ T subpopulations and inflammatory cytokine secretion (IFNγ, IL-1, IL-6, and IL-13) were normalised and his anti-Jo1 level was divided by four.
