Abstract Introduction Interstitial lung disease (ILD) can result from various immune, infectious, and environmental causes, often progressing to acute respiratory failure when complicated by infections or cardiac disease. The management becomes especially challenging when a superimposed infection occurs in the setting of suspected autoimmune conditions. This case highlights the complexity of diagnosing and treating ILD with acute lung injury due to superimposed fungal pneumonia and multiorgan failure. Case Presentation A 58-year-old woman with a history of Raynaud’s phenomenon, radiographic nonspecific interstitial pneumonia (NSIP), and suspected connective tissue disease (scleroderma) presented with progressive shortness of breath, gastrointestinal symptoms, and recent travel to the Philippines. Initially treated for suspected non-ST elevation myocardial infarction (NSTEMI), her clinical course rapidly deteriorated with recurrent ventricular tachycardia (VT) and cardiogenic shock, necessitating Impella 5.5 placement. Despite aggressive therapy, including mechanical ventilation, steroids, and antiarrhythmics, the patient developed acute respiratory distress syndrome (ARDS) and septic shock secondary to aspiration pneumonia. Postmortem examination revealed acute bronchopneumonia with yeast elements (Candida tropicalis and Candida glabrata), diffuse alveolar damage consistent with acute lung injury, and subpleural fibrosis with early honeycombing indicative of chronic ILD- likely connective tissue disease-related. Hilar lymph nodes showed rare non-necrotizing granulomas, raising the possibility of an underlying granulomatous process like sarcoidosis, though this could not be definitively diagnosed due to a lack of diagnostic criteria or granulomatous inflammation in multiple organs and tissues. Cardiac findings included myocardial fibrosis, likely related to recent inflammatory myocarditis from CoxSackie virus rather than an autoimmune etiology. Discussion This case underscores the diagnostic uncertainty in patients with chronic ILD when suspected autoimmune disease overlaps with acute infectious processes. While scleroderma was clinically suspected, the autopsy revealed fungal bronchopneumonia as the primary cause of death, with chronic ILD and cardiogenic shock as contributing factors. The absence of definitive autoimmune markers and the presence of rare non-necrotizing granulomas in hilar lymph nodes raised the possibility of undiagnosed sarcoidosis, though a clear diagnosis was not rendered. Conclusion This case emphasizes the need for careful diagnostic evaluation in patients with chronic ILD, particularly when autoimmune and infectious etiologies are suspected. The rapid deterioration highlights the importance of early recognition of superimposed infections, especially in immunosuppressed patients with systemic disease. Further research is warranted to explore the relationship between chronic ILD, immunosuppression, and susceptibility to severe fungal infections. Multidisciplinary collaboration across pulmonary, rheumatology, cardiology, and infectious disease teams is critical for optimizing outcomes in such complex cases. This abstract is funded by: none
Kumar et al. (Fri,) studied this question.