Figure 1a:
Figure 1b:
Figure 2a:
Figure 2b:
Timeline of Clinical Course
| Time point | Clinical events / Findings | Investigations | Treatment | Outcome |
|---|---|---|---|---|
| Day 0 (ICU admission) | Progressive dyspnea, fever, hypoxemia (SpO2 75% RA) | Labs: CRP 32 mg/L, ESR 24 mm/h, severe lymphopenia (0.4×103/µL); Chest CT: rapidly progressive bilateral fibrotic ILD | Oxygen therapy; empirical levofloxacin + ceftriaxone; MMF discontinued; IVIG (0.4 g/kg/day) initiated | Respiratory distress persisted |
| Day 1 | Persistent fever and worsening inflammation | Rising inflammatory markers | Continued IVIG + antibiotics | Clinical deterioration |
| Day 2 | Increasing tachypnea and oxygen requirement | CRP 170 mg/L, ESR 94 mm/h; respiratory PCR negative; CMV PCR: low-level viremia (357 IU/mL) | Antibiotics escalated to piperacillin–tazobactam | Progressive hypoxemia |
| Day 3 | Acute respiratory failure | Severe hypoxemia; BAL deferred due to PaO2 < 60 mmHg, SpO2 < 90%, hypotension and tachycardia | Endotracheal intubation and mechanical ventilation | Temporary stabilization |
| Day 4–5 | Hemodynamic instability and multiorgan dysfunction | Hypotension, tachycardia, acute kidney injury (eGFR < 15 mL/min) | Maximal supportive care and immunomodulatory therapy | Death due to refractory respiratory failure secondary to anti-MDA5–associated RP-ILD |