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.