2 Case Report
A 36-year-old previously healthy female visited a local hospital
presenting with cough and fever. She had undergone
induction of labor for a stillborn
fetus 2 months earlier and subsequently
noticed
erythema on her face, accompanied by
cough, but no phlegm. Allergic disease was considered, and antiallergic
treatment was given, but her symptoms did not improve. Her condition was
then managed with oral prednisolone
for 10 days, and the facial
erythema
and cough disappeared. The patient’s clinical course is shown in Figure
1.
On admission, the patient showed no cutaneous and muscular
manifestations. Computed tomography (CT) of the chest showed bilateral
ground glass opacities (Figure 2A). Anti-MDA5 antibody was not measured
because there was no consideration of CAMD and ILD. After receiving
antibiotic therapy for 12 days,
the patient’s status did not improve
and worsened in later stages; the clinical manifestations were shortness
of breath and dyspnea. CT of the chest showed bilateral pulmonary patchy
infiltrates, and interstitial pneumonia was considered (Figure 2B).
Shortly thereafter, the patient presented with acute hypoxemic
respiratory failure (PaO2/FiO2: 68 mmHg), and intubation and mechanical
ventilation were subsequently performed 3 days after admission. Using
next-generation sequencing (NGS) of the bronchoalveolar lavage fluid
(BLF) sample and cultured isolates from the patients, Pseudomonas
aeruginosa, Stenotrophomonas maltophilia andPneumocystis jirovecii were
found. Sulfamethoxazole (SMZ) was added for the treatment of
pneumocystis pneumonia (PCP) caused by Pneumocystis jirovecii .
The patient’ s respiratory status continued to deteriorate, and
mediastinal emphysema and subcutaneous emphysema developed 4 days after
invasive ventilation.
VV-ECMO via the right internal jugular and right femoral vein
cannulation was initiated on
ventilator day 4, and she was then referred to the ECMO center.
Laboratory investigations revealed that serum anti-Ro-52 was positive
via ELISA, and serum ferritin (SF)
was significantly higher without elevated serum muscle enzymes. NGS of
BLF also showed Pneumocystis jirovecii and Acinetobacter
baumannii ; thus, antibiotic therapy and SMZ were continued. Considering
the COVID-19 epidemic in China, the patient received a nucleic acid test
for COVID-19, but the result was negative.
CT of the chest showed bilateral
pulmonary extensive infiltrates and lobular interstitium thickness, and
pulmonary fibrosis was considered
(Figure 2C). CT of the head was normal (Figure 3A), and the patient was
conscious after withdrawal of the sedative. Although lung protective
ventilation, recruitment maneuver and prone position ventilation were
implemented, she did not tolerate attempts to wean from ECMO within
28 days of ECMO. She required
continuous sedation and analgesia because of patient-ventilator
asynchrony.
Therefore, a decision was made to place the patient on the lung
transplant waitlist, and she was subsequently transferred to the
transplantation center for lung transplant evaluation.
Anti-MDA5 antibody was tested by
ELISA, and the result was
positive.
Based on these findings, the patient was diagnosed with CADM and ILD. At
31 days of ECMO, the patient underwent a successful sequential double
lung transplant and received tacrolimus as an immunosuppressive regimen
after the transplant. Her explant pathology
showed extensive consolidation of
lung tissue and pulmonary interstitial fibrosis (Figure 4). The
patient’s respiratory status gradually improved, and CT of the chest
showed bilateral pulmonary scattered
infiltration (Figure 2D), which was improved compared with previous
imageological diagnosis. ECMO was weaned successfully 3 days after
transplant, and the patient’s oxygenation status did not deteriorate
with ventilator support. The patient’s state of consciousness
deteriorated, and she presented with coma. Head CT showed bilateral
parieto-occipital low-density lesions, which were considered to be due
to PRES (Figure 3B). Since the condition was considered to be related to
immunosuppressive agents, tacrolimus was suspended for 1 day, and
the dosage was gradually reduced to
0.5 mg/day, after which the patient’s consciousness returned.
Unfortunately, the patient developed a disturbance of consciousness once
more after hemodynamic instability, which may be related to implant
infection; consciousness did not return after active treatment. After 14
days of lung transplant, the patient declined further treatment for
financial reasons and was discharged.