Sirolimus-induced secondary pulmonary alveolar proteinosis
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome that is characterized by the accumulation of excess surfactant in the alveolar space, leading to impaired gas exchange. Sirolimus-induced PAP is an extremely rare entity that has only been described in the literature in a small number...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-01-01
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| Series: | Respiratory Medicine Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213007121002288 |
| _version_ | 1798034423050928128 |
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| author | Stephanie Wang, MD Elinor Lee, MD, PhD Ryan Lau, MD Tisha Wang, MD |
| author_facet | Stephanie Wang, MD Elinor Lee, MD, PhD Ryan Lau, MD Tisha Wang, MD |
| author_sort | Stephanie Wang, MD |
| collection | DOAJ |
| description | Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome that is characterized by the accumulation of excess surfactant in the alveolar space, leading to impaired gas exchange. Sirolimus-induced PAP is an extremely rare entity that has only been described in the literature in a small number of case reports. We present a case of a 39-year-old female with acute lymphocytic leukemia who underwent stem cell transplant, complicated by graft-versus-host-disease (GVHD) involving the skin for which she was treated with steroids, photopheresis, sirolimus, and ruxolitinib. She was admitted to the intensive care unit (ICU) for acute on chronic hypoxic respiratory failure requiring intermittent mechanical ventilation. Computed tomography (CT) of the chest showed thickened inter- and intralobular septa with ground glass opacities and consolidation with a limited geographic pattern. Bronchoalveolar lavage fluid was stained with Periodic acid-Schiff (PAS), which was positive for extracellular proteinaceous material. Autoimmune studies including antibody levels for primary autoimmune pulmonary alveolar proteinosis (PAP) were negative. The patient was diagnosed with sirolimus-induced secondary PAP, and sirolimus was discontinued. A year later, she no longer required supplemental oxygen, and repeat CT imaging showed only faint residual disease. This is the only documented case of sirolimus-induced PAP in a stem cell transplant recipient and the first case reported in which the patient developed severe hypoxic respiratory failure requiring mechanical ventilation. In the right clinical context, PAP can be diagnosed with characteristic high resolution computed tomography (HRCT) findings, serum GM-CSF antibody levels, and bronchoscopy with bronchoalveolar lavage. |
| first_indexed | 2024-04-11T20:44:10Z |
| format | Article |
| id | doaj.art-169b3fca8a6f4c8098f0d0b227aa4faf |
| institution | Directory Open Access Journal |
| issn | 2213-0071 |
| language | English |
| last_indexed | 2024-04-11T20:44:10Z |
| publishDate | 2022-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Respiratory Medicine Case Reports |
| spelling | doaj.art-169b3fca8a6f4c8098f0d0b227aa4faf2022-12-22T04:04:06ZengElsevierRespiratory Medicine Case Reports2213-00712022-01-0135101566Sirolimus-induced secondary pulmonary alveolar proteinosisStephanie Wang, MD0Elinor Lee, MD, PhD1Ryan Lau, MD2Tisha Wang, MD3Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, USADepartment of Medicine, David Geffen School of Medicine, University of California, Los Angeles, USA; Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, USA; Corresponding author. Division of Pulmonary, Critical Care Medicine University of California, Los Angeles 650 Charles E. Young Drive South, 43-229 CHS, Los Angeles, CA, 90095-1690, USA.Department of Pathology, University of California, Los Angeles, USADepartment of Medicine, David Geffen School of Medicine, University of California, Los Angeles, USA; Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, USAPulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome that is characterized by the accumulation of excess surfactant in the alveolar space, leading to impaired gas exchange. Sirolimus-induced PAP is an extremely rare entity that has only been described in the literature in a small number of case reports. We present a case of a 39-year-old female with acute lymphocytic leukemia who underwent stem cell transplant, complicated by graft-versus-host-disease (GVHD) involving the skin for which she was treated with steroids, photopheresis, sirolimus, and ruxolitinib. She was admitted to the intensive care unit (ICU) for acute on chronic hypoxic respiratory failure requiring intermittent mechanical ventilation. Computed tomography (CT) of the chest showed thickened inter- and intralobular septa with ground glass opacities and consolidation with a limited geographic pattern. Bronchoalveolar lavage fluid was stained with Periodic acid-Schiff (PAS), which was positive for extracellular proteinaceous material. Autoimmune studies including antibody levels for primary autoimmune pulmonary alveolar proteinosis (PAP) were negative. The patient was diagnosed with sirolimus-induced secondary PAP, and sirolimus was discontinued. A year later, she no longer required supplemental oxygen, and repeat CT imaging showed only faint residual disease. This is the only documented case of sirolimus-induced PAP in a stem cell transplant recipient and the first case reported in which the patient developed severe hypoxic respiratory failure requiring mechanical ventilation. In the right clinical context, PAP can be diagnosed with characteristic high resolution computed tomography (HRCT) findings, serum GM-CSF antibody levels, and bronchoscopy with bronchoalveolar lavage.http://www.sciencedirect.com/science/article/pii/S2213007121002288 |
| spellingShingle | Stephanie Wang, MD Elinor Lee, MD, PhD Ryan Lau, MD Tisha Wang, MD Sirolimus-induced secondary pulmonary alveolar proteinosis Respiratory Medicine Case Reports |
| title | Sirolimus-induced secondary pulmonary alveolar proteinosis |
| title_full | Sirolimus-induced secondary pulmonary alveolar proteinosis |
| title_fullStr | Sirolimus-induced secondary pulmonary alveolar proteinosis |
| title_full_unstemmed | Sirolimus-induced secondary pulmonary alveolar proteinosis |
| title_short | Sirolimus-induced secondary pulmonary alveolar proteinosis |
| title_sort | sirolimus induced secondary pulmonary alveolar proteinosis |
| url | http://www.sciencedirect.com/science/article/pii/S2213007121002288 |
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