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.