Yong-Long Xiao1, Kai-Feng Xu2, Yan Li3, Yan Li3, Hui Li3, Bin Shi4, Ke-Feng Zhou5, Zheng-Yang Zhou5, Hou-Rong Cai3. 1. Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China Department of Respiratory Medicine, Suqian People's Hospital of Nanjing Drum Tower Hospital Group, Suqian, Jiangsu, China. 2. Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, China. 3. Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China. 4. Department of Respiratory Medicine, Suqian People's Hospital of Nanjing Drum Tower Hospital Group, Suqian, Jiangsu, China. 5. Department of Radiology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, China.
Abstract
OBJECTIVES: Although the serum granulocyte-macrophage colony stimulating factor autoantibody (GMAb) levels have been recognised as a diagnostic marker in primary pulmonary alveolar proteinosis (PAP), their role in PAP with occupational inhalational exposure (PAPo) remains unclear. METHODS: Forty-five consecutive patients with PAP were enrolled. Each patient with PAP was assessed for baseline clinical characteristics, chest high-resolution CT (HRCT), serum GMAb and occupational exposure. Fifty healthy controls were included to define normal ranges for GMAb levels. Ninety-seven hospital controls with other respiratory diseases were included to establish prevalence of a history of occupational inhalation exposure. RESULTS: According to the serum GMAb cut-off value of 2.39 μg/mL, 84.4% of the recruited patients with PAP had positive serum GMAb with a median level of 28.7 μg/mL, defined as autoimmune PAP, and the remaining 15.6% had negative serum GMAb with a median level of 0.16 μg/mL, defined as non-autoimmune PAP. Also, 34.2% of patients with autoimmune PAP had a history of occupational inhalational exposure, which was not significantly higher than that of hospital controls (34.2% vs 19.6%, p=0.072). Four patients with PAPo showed negative GMAb. Their arterial oxygen tension, pulmonary function parameters and chest HRCT features were significantly different when compared with patients with autoimmune PAP (p<0.05). These four non-autoimmune occupational lung disease cases culminated in 3 deaths and a lung transplant. CONCLUSIONS: A number of patients with PAP who may have occupational inhalational exposure and negative serum GMAb represent a high possibility of silicoproteinosis and very poor survival. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
OBJECTIVES: Although the serum granulocyte-macrophage colony stimulating factor autoantibody (GMAb) levels have been recognised as a diagnostic marker in primary pulmonary alveolar proteinosis (PAP), their role in PAP with occupational inhalational exposure (PAPo) remains unclear. METHODS: Forty-five consecutive patients with PAP were enrolled. Each patient with PAP was assessed for baseline clinical characteristics, chest high-resolution CT (HRCT), serum GMAb and occupational exposure. Fifty healthy controls were included to define normal ranges for GMAb levels. Ninety-seven hospital controls with other respiratory diseases were included to establish prevalence of a history of occupational inhalation exposure. RESULTS: According to the serum GMAb cut-off value of 2.39 μg/mL, 84.4% of the recruited patients with PAP had positive serum GMAb with a median level of 28.7 μg/mL, defined as autoimmune PAP, and the remaining 15.6% had negative serum GMAb with a median level of 0.16 μg/mL, defined as non-autoimmune PAP. Also, 34.2% of patients with autoimmune PAP had a history of occupational inhalational exposure, which was not significantly higher than that of hospital controls (34.2% vs 19.6%, p=0.072). Four patients with PAPo showed negative GMAb. Their arterial oxygen tension, pulmonary function parameters and chest HRCT features were significantly different when compared with patients with autoimmune PAP (p<0.05). These four non-autoimmune occupational lung disease cases culminated in 3 deaths and a lung transplant. CONCLUSIONS: A number of patients with PAP who may have occupational inhalational exposure and negative serum GMAb represent a high possibility of silicoproteinosis and very poor survival. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
Authors: Paul D Blanc; Isabella Annesi-Maesano; John R Balmes; Kristin J Cummings; David Fishwick; David Miedinger; Nicola Murgia; Rajen N Naidoo; Carl J Reynolds; Torben Sigsgaard; Kjell Torén; Denis Vinnikov; Carrie A Redlich Journal: Am J Respir Crit Care Med Date: 2019-06-01 Impact factor: 21.405