PMC8495060_01
Imaging of most severe autoimmune lung disease on admission. (A) Recruitment CT-scan for acute respiratory distress syndrome (ARDS) at FiO2 of 100% in deep analgo-sedation, as well as muscle relaxation with cis-atracurium at a PEEP-level of 5 cmH2O and PIP at 15 cmH2O with tidal volumes at 6 ml/kg ideal body weight (IBW) in an expiratory hold after 3 min at this level; (B) CT-scan after 1 min of recruitment at a PEEP-level of 10 cm H20 and a PIP of 35 cm H2O, followed by 1 min at a PEEP-level of 20 cm H2O and a PIP of 45 cm H2O at an inspiratory hold; (C) Conventional imaging with bilateral inflammatory consolidations in lower lobes.
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F1
A recruitment CT scan for acute respiratory distress syndrome (ARDS) did not show a significant recruitment potential of the lungs (Figures 1A,B). Small airway disease was diagnosed with bilateral inflammatory consolidations in the lower lobes, which, at this time, appeared as a typical bilateral pneumonia in the conventional im-aging (Figure 1C).
Course of autoimmune disease during therapy. The 17-year old patient required intubation on the first day of hospital admission and also, a few hours later, ECMO therapy needed to be initiated on the first day of hospital admission. (A) ECMO and mechanical ventilation conditions during treatment in the ICU, including implantation and explantation of tracheal cannula (TC explant), PIP, PEEP, blood flow, oxygen flow and immunosuppressive therapy with Eculizumab, Cyclophosphamide, and Prednisolone; (B) Titers of anti-GBM and complement C3/C4 during therapy (normal ranges: S-GBM Elisa <7 U/ml; C3 0.83-1.52 g/l; C4 0.13-0.37 g/l) ECMO, extracorporeal membrane oxygenation; TC, tracheal cannula; CPM, Cyclophosphamide; Predni, Prednisolone; PIP, positive inspiratory pressure; PEEP, positive end expiratory pressure; Explant, explantation.
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F2
ECMO conditions included a blood flow of 4.5 l/min and an oxygen flow of 5 l/min. ECMO-weaning was successful at day 24 after admission to our University hospital (Figure 2A). After the last course of Cyclophosphamide (1,000 mg), the patient was subsequently discharged from our ICU unit and transferred back home to his local hospital (Figure 2B).