Effective treatment of Jo-1-associated polymyositis with T-cell-depleted autologous peripheral blood stem cell transplantation

A patient with Jo-1 antibody-associated polymyositis (Jo-1 PM) had a Karnof sky score of 40% and severe muscle, liver and lung damage that was refracto ry to standard therapy. The female patient received an autologous T-cell-de pleted haematopoietic stem cell transplant (HSCT) after myeloablative condi tioning. The transplant procedure was complicated by severe adult respirato ry distress syndrome (ARDS) and adenovirus-associated haemorrhagic cystitis as well as cytomegalovirus (CMV) reactivation. The patient's creatinine ph osphokinase (CPK) and alanine transaminase (ALT) values were normal on day 21. The patient's strength has improved remarkably and her dyspnoea is subj ectively improved. At 15 months after the transplant, the patient was well with a Karnofsky score of 80% and had been off any therapy, including stero ids, for 14 months.