SESSION TITLE: ILD Student/Resident Case Report Posters
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of amorphous, periodic acid-Schiff (PAS)-positive lipoproteinaceous material in the distal airspaces, with preserved lung architecture and no lung inflammation. Three forms of PAP are recognized: congenital, secondary, and acquired form. Patients with PAP, however, are at increased risk of opportunistic infections which usually follow the diagnosis of PAP. Here we present a case of PAP diagnosed after disseminated nocardiosis.
CASE PRESENTATION: The patient is 45 year old male with history of pleural nocardiosis. He was treated with moxifloxacin and bactrim for 6 months. Seven months later he presented with pulmonary and cerebellar relapse that needed craniotomy to relief high intracranial pressure, and started on amikacin, linezolid and bactrim according to culture and sensitivity results. Four months later he presented with worsening shortness of breath. CT scan showed diffuse lung infiltrates predominantly in the left mid lung zones and lung bases. These findings were not recognized in the previous imaging. Video-assisted thoracoscopy with lung biopsy were done. The biopsy revealed pulmonary alveolar proteinosis with no bacteria, no fungi, and negative cultures. Then the patient got Whole lung lavage, after which his symptoms improved significantly and he was weaned off oxygen.
DISCUSSION: The most common form of PAP is the acquired one due to malfunction of alveolar macrophages. This is result of granulocyte macrophage colony-stimulating factor (GM CSF) receptor autoantibodies, and accumulation of PAP materials which further impairs macrophages functions. These antibodies can be measured in the serum and bronchoalviolar lavage of patients and can inactivate normal macrophages. Literature review showed many opportunistic infections associated with PAP, at least 32 case of PAP were reported in patients with nocardiosis, sequentially or simultaneously , most of the times, however, the infection was preceded by PAP which raise the question: is it cause or consequence of opportunistic infections, and is PAP overlooked when infectious etiology is identified? Besides, many cases were cured by total lung lavage which also makes the autoimmune etiology questionable.
CONCLUSIONS: Patient who had nocardiosis is at risk of PAP. Further research is needed to fully understand PAP pathophysiology to help establish the treatment.
Reference #1: Uchida, K., et al., GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis. New England Journal of Medicine, 2007. 356(6): p. 567-579.
Reference #2: Punatar, A.D., et al., Opportunistic infections in patients with pulmonary alveolar proteinosis. Journal of Infection, 2012. 65(2): p. 173-179.
DISCLOSURE: The following authors have nothing to disclose: Nibal Saad, Pornchai Leelasinjaroen, Eguakhide Inegbenebor
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