1 Introduction
Clinically amyopathic dermatomyositis (CADM) is defined as the presence of typical cutaneous manifestations of dermatomyositis (DM) along with absent or minimal muscle weakness in DM[1]. Population-based data suggest that CADM occurs in approximately 20% of all adult DM cases[2], and the incidence of CADM is estimated to be 2.08 per 1 million persons[3]. Patients with CADM have an increased risk of interstitial lung disease (ILD), and especially severe cases are complicated by life-threatening rapid progressive ILD (RP-ILD)[4], emphasizing the necessity and significance of early recognition and management of the disease. ILD more frequently occurs in patients with CADM and positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody, and these patients are more likely to progress to RP-ILD and refractory acute respiratory failure and often have a poorer prognosis than those with negative anti-MDA5 antibody[5].
For RP-ILD patients with critical hypoxemia or respiratory acidosis despite conventional therapies, particularly that accompanied by other respiratory complications, venovenous extracorporeal membrane oxygenation (VV-ECMO) may be a perfect choice. The treatment is sometimes a bridge to recovery, but limited options exist for CADM patients with refractory hypoxemia who fail to wean from ECMO[6]. However, with no lung transplant consideration, using ECMO in such cases has been felt to be “bridge to nowhere”[7] due to the limited treatment response and overall options. The immunosuppressants used after transplants inevitably introduce complications; posterior reversible encephalopathy syndrome (PRES) is a common complication that manifests as various neurological symptoms[8]. Tacrolimus, an important immunosuppressive drug for organ transplantation patients, is a major risk factor for PRES[9].
Here, we present the case of a female with CADM complicated by severe RP-ILD managed with VV-ECMO. Due to refractory respiratory failure, bilateral lung transplantation was eventually performed, but the patient ultimately developed posterior reversible encephalopathy syndrome.