Bilateral lung transplantation for rapidly progressive interstitial lung
disease associated with clinically amyopathic dermatomyositis followed
by posterior reversible encephalopathy
Abstract
A 36-year-old woman was referred to a local hospital with cough and
fever. She had a history of facial erythema and cough that began 2
months ago. Her status developed into rapid progressive interstitial
lung disease (RP-ILD) with mediastinal emphysema and subcutaneous
emphysema after admission, and venovenous extracorporeal membrane
oxygenation (V-V ECMO) was initiated. After several failed attempts to
wean the patient from ECMO, a decision was made to place the patient on
the lung transplant waitlist. She underwent a double lung transplant on
ECMO day 31 and received tacrolimus as an immunosuppressive regimen. The
patient presented with positive anti-MDA5 and anti-Ro-52 antibodies and
a high ferritin level, all of which indicated the presence of clinically
amyopathic dermatomyositis (CADM). The patient was weaned from ECMO at 3
days after transplantation, but the patient’s state of consciousness
deteriorated, and head CT was considered for posterior reversible
encephalopathy syndrome (PRES). After the temporary cessation of
calcineurin inhibitors and a dosage reduction, the patient’s state of
consciousness returned to normal. Because of another disturbance of
consciousness, the patient declined further treatment and was discharged
14 days after transplantation.