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Protein Alveolar Proteinosis Secondary to MAI and Recurrence Due to Nocardiosis FREE TO VIEW

Kevin Haas, MD; Norma Ramey, MD; Michael Markos, MD; Sarah Usmani, MD; Howard Jaffe, MD; Min Joo, MD
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University of Illinois at Chicago, Chicago, IL

Chest. 2013;144(4_MeetingAbstracts):179A. doi:10.1378/chest.1703802
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SESSION TITLE: Infectious Disease Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Protein alveolar proteinosis (PAP) is a well-known rare pulmonary disease (0.37 per 100,00 patients) characterized by alveolar accumulation of surfactant3. We describe an unusual case of PAP occurring twice within the same year associated with different infections. The initial diagnosis was based on video-assisted thoracic surgery biopsy with associated mycobacterium-avium intracellulare (MAI) infection. The patient presented again five months later with a new cavitary lesion and bronchoalveolar lavage (BAL) revealed nocardosis. We also describe successful treatment with whole lung lavage in the supine position and negative pressure applied for the withdrawal of lavage fluid.

CASE PRESENTATION: A 54 year old man with a past medical history of crack cocaine abuse, diabetes mellitus, and tobacco usage presented with complaints of worsening dyspnea on exertion and was found to have radiographic imaging consistent with PAP (Figure 1). He underwent video-assisted thoracic surgery biopsy showing pathologic findings consistent with PAP and mycobacterium-avium intracellulare in the tissue culture. The patient underwent whole lung lavage and was started on MAI treatment. Five months later, patient was admitted with worsening dyspnea. A new right upper lobe cavity (figure 2) was noted compared to the CT scan in April. The patient was taken to the operating room for whole lung lavage. The patient was maintained in the supine position due to oxygen saturations into the mid 80s in the lateral position. Between 1-1.5L of warmed normal saline was instilled at a time, then suction was applied to the endotracheal tube and volume was removed after a percussion vest was applied. After the first few instillations of fluid, the procedure was complicated by hypoxia into the mid to low 80s. Culture of the right upper lobe bronchoalveolar lavage revealed nocardia asteroids.

DISCUSSION: For patients with recurrent PAP, whether by radiography or lavage fluid analysis, a high clinical suspicion should be kept to re-assess for new infections and clearance of prior ones.

CONCLUSIONS: Whole lung lavage can be done safely and effectively in the supine position with negative pressure used during the removal of lavage fluid. Hypoxia should be anticipated and expected early in the procedure with improvement during subsequent lavage cycles.

Reference #1: Browne S, Holland S. Anticytokine autoantibodies in infectious diseases: pathogenesis and mechanisms. Lancet Infectious Disease. 2010.10:875-885.

Reference #2: Punatar A, Kusne S, Blair J. et al. Opportunistic infections in patients with pulmonary alveolar proteinosis. Journal of Infection. 2012.65;173-179.

Reference #3: Trapnell B, Whitsett J, Nakata K. Pulmonary Alveolar Proteinosis. NEJM. 2003.349;2527-39.

DISCLOSURE: The following authors have nothing to disclose: Kevin Haas, Norma Ramey, Michael Markos, Sarah Usmani, Howard Jaffe, Min Joo

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