Occupational and Environmental Lung Diseases |

A “Crazy Cluster” in Connecticut FREE TO VIEW

Christopher Erb, MD; Mohammad Ali Jafri, MD; Gaetane Michaud, MD
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Yale University, New Haven, CT

Chest. 2015;148(4_MeetingAbstracts):759A. doi:10.1378/chest.2281191
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SESSION TITLE: Occupational and Environmental Lung Diseases Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a rare condition associated with the accumulation of surfactant in alveoli leading to progressive dyspnea. Reported incidence is one:one million. Known associations are hematopoietic malignancy, autoimmune disease, infection, and environmental exposures such as tobacco smoke and silica dust. An association with autoantibodies against GM-CSF receptors is described and serologic testing has become routine. If detected, therapy aimed at replacing GM-CSF serum levels is indicated. Other treatment options include Rituximab, plasmapheresis, and whole lung lavage, and multi-modality therapy is being explored.

CASE PRESENTATION: We present a series of six cases of PAP with an average age of 39.8 years and equal gender distribution. Three patients smoked at least one pack per day, but two were non-smokers. Most used alcohol “regularly” to “excessively”. They worked in manual labor jobs with exposures to dusts and chemicals. None had known exposures to the “classic” PAP risk factors of silica and metals such as Indium and Aluminum. In all cases, chest imaging revealed extensive airspace opacities with interlobular thickening, described as the “crazy paving” pattern. Every patient underwent at least one bilateral lung lavage procedure. In all cases, testing for autoantibodies against GM-CSF was positive, suggesting an autoimmune etiology, and supplementation therapy was offered.

DISCUSSION: Our patients with autoimmune PAP clustered in an industrial area of Connecticut, raising our suspicion for an epidemiologic association. These were all “blue-collar” workers who may have been exposed to dust or chemicals in their job. In addition, all were alcohol drinkers; in some cases, binge drinkers. An association between alcohol use and PAP has not been reported, but we wonder whether these patients’ heavy use of alcohol may have contributed to the development of PAP, and believe this warrants further evaluation.

CONCLUSIONS: Pulmonary Alveolar Proteinosis is an uncommon clinical entity thought to be sporadic and is not known to “cluster” in any discernable geographic pattern. Our series of cases presented over a two-year period from one region of Connecticut. Whether these patients’ work exposures or their behavioral characteristics increased their risk for PAP is unclear, but raises our curiosity about what appears to be a “crazy cluster” of PAP in Connecticut.

Reference #1: Michaud G, Reddy C, Ernst A. Whole-lung lavage for pulmonary alveolar proteinosis. Chest 2009;136(6):1678-81.

DISCLOSURE: The following authors have nothing to disclose: Christopher Erb, Mohammad Ali Jafri, Gaetane Michaud

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