A Case of Idiopathic Pneumonia Syndrome in a Patient With Recent Stem Cell Transplant

Document Type

Conference Proceeding

Publication Date

5-21-2024

Publication Title

Am J Respir Crit Care Med

Abstract

Introduction:Idiopathic pneumonia syndrome (IPS) represents a rare but severe complication observed in up to 10% of patients undergoing allogeneic stem cell transplantation (SCT), that carries a high mortality rate. IPS usually manifests within four months post-SCT, making it crucial to promptly identify and manage. Case Presentation:A 68 year-old male with a medical history of acute myeloid leukemia on chemotherapy who received allogenic SCT 26 days prior to admission presented to the emergency department with fever and dyspnea. Upon arrival, the patient exhibited hypoxia and increased work of breathing necessitating BiPAP support and subsequent intubation. CT of the chest showed diffuse bilateral consolidative and ground-glass opacities. Broad-spectrum antibiotics were initiated while awaiting results of additional workup. Bronchoscopy and bronchoalevoar lavage (BAL) did not reveal an infectious etiology. Transbronchial biopsies not completed given thrombocytopenia. Respiratory culture, fungal culture, and AFB smear and culture were unrevealing. Respiratory viral PCR, PJP PCR, fungitell, and aspergillus galactomannan were negative. No eosinophils were seen on BAL given the recent Daptomycin thus excluding acute eosinophilic pneumonia. CMV DNA quant was elevated at 1002 so the patient was transitioned to Ganciclovir and given Cytogam. Levels were 123 a week later but felt presentation was less consistent with CMV pneumonitis. Given lack of improvement within 72 hours of presentation and that infectious etiology seemed less likely, the patient was started on methylprednisolone 2 mg/kg divided twice daily for treatment of suspected IPS. Additionally, Etanercept was started twice weekly. After ten days of mechanical ventilation, the patient was extubated to heated high flow nasal cannula. Discussion:The exact etiology of IPS remains elusive, thought to be a complex interplay of factors, including lung lining damage from conditioning regimens and graftversus- host disease. IPS diagnosis is based on the exclusion of infectious and cardiac etiologies in the presence of clinical and radiographic findings suggestive of pneumonia, typically confirmed through lung biopsy. However, obtaining a lung biopsy may not always be feasible, as in the presented case, due to patient-specific factors. Treatment for IPS involves a combination of approaches, including oxygen therapy, ventilatory support if needed, systemic corticosteroids, and immunosuppressive medications such as Etanercept which block tumor necrosis factor. Early recognition and intervention are crucial for achieving favorable outcomes in these challenging cases. This also highlights the importance of treating possible infectious etiologies while awaiting workup and when unable to rule out certain infections.

Volume

209

Issue

9

First Page

A5313

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