A female (55y) patient had the diagnosis of malignancy associated dermatomyositis 2014. After the tumor was removed (+ additional chemotherapy) the dermatomyositis resolved. We saw the patients first time 07/2017 when she had a relaps of her dermatomyositis. Tumorscreening (incl. PET-CT) was negative, so she get a treatment with prednisone and additional azathioprine (steroid sparing), but after prednisone was tapered below 20mg the dermatomyositis relapsed. The immunosuppressive treatment was changed to MMF. Few weeks late the patient developed neurological symptoms (double vision) and was addmitted to a neurological department. The colleges diagnosed a PML (progressive multifocal leucencephalopathy) and stopped the immunosuppressive treatment. The neurological symptoms persited unchanged. 2 month later the patient had still a severe immunodeficiency (very low T-helper-cells, B-cells and cytotoxic-T-cells) but no progress of her neurological symptoms, so we had strong doubts about the diagnosis of PML. But external experts about neuroradiology and neurology confirmed the diagnosis, so we are afraid of starting an immunosuppressive treatment again.

Again two month later the immune system normalized but at the same time the dermatomyositis relapsed. A therapy with i.v. immunoglobulins was started but without any efficacy. The dermatomyositis worsened. We started a treatment with cyclosporine, but once again without effect. So we decided to start an immunoadsorption with good efficacy. Tumorscreening was repeated twice over this time with negative results.

Who knows how often immunoadsorption is necessary for a stable remission of dermatomyositis?

Who has experience with immunoadsorption in dermatomyositis?

Maybe someone knows a good alternative treatment option for this patient?

With many thanks for your input.