Relationship of Biomarkers with Clinical and Radiographic Characteristics in Autoimmune Pulmonary Alveolar Proteinosis_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

ZhangXinjing ^1,2 ,  XiaoYonglong ^1,2 , CaiHourong ¹ , LiYan ³ , XuKaifeng ³ , GuoWanhua ⁴ , ShiBin ²

1. Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, Jiangsu, 210008, China;
2. ;
3. ;
4. ;

Corresponding author：

XiaoYonglong, Email: yonglong11a@163.com

Keywords：

Pulmonary alveolar proteinosis; Granulocyte macrophage colony stimulating factor; Autoantibody

DOI：

10.7507/1671-6205.2014095

Video：

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Objective To describe the clinical,radiographic,and laboratory features of autoimmune pulmonary alveolar proteinosis (PAP) from a single center. Methods Consecutive autoimmune PAP cases diagnosed in the Nanjing Drum Tower Hospital between January 2006 and December 2012 were recruited in the study. The clinical,radiographic and laboratory data of the PAP patients were analyzed to explore the clinical significance of serum GM-CSF autoantibody (GMAb) and serum cytokeratin (CYFRA21-1). Results The median serum GMAb level of the 26 cases was 28.64 μg/mL (interquartile range,19.2-75.4 μg/mL),which were diagnosed as autoimmune PAP based on the serum GMAb levels of these patients all above the cut-off value of 2.39 μg/mL while the serum GMAb levels of 30 normal controls were 0.10(0.05-0.15)μg/mL and all below the cut-off value. 34.6% of all recruited 26 autoimmune PAP patients had identified occupational inhalational exposure. There was no significant correlation in the serum GMAb in autoimmune PAP patients with disease severity scores (DSS),lung function parameters,chest high resolution computed tomography (HRCT) scores,or PaO₂ (P>0.05). There was significant correlation of DSS of autoimmune PAP patients with PaO₂,FVC%pred,T_LCO%pred,opacity extent score of chest HRCT,and opacity severity score of chest HRCT (P<0.05). The median serum level of CYFRA21-1 of the autoimmune PAP patients was 9.9(4.3-19.5)ng/mL,which was significant higher than that of the normal control group (P<0.05). However there was no significant correlation in the serum CYFRA21-1 in the autoimmune PAP patients with DSS,lung function parameters,and chest HRCT scores. 92.3% of the chest HRCT of 26 autoimmune PAP patients had crazy paving sign,while 100% of them had geographic sparing sign. Conclusion Serum GMAb and CYFRA21-1 may be important biomarkers for diagnosis of autoimmune PAP. The PAP with occupational inhalational exposure constitutes a high proportion of autoimmune PAP patients.

Citation： ZhangXinjing, XiaoYonglong, CaiHourong, LiYan, XuKaifeng, GuoWanhua, ShiBin. Relationship of Biomarkers with Clinical and Radiographic Characteristics in Autoimmune Pulmonary Alveolar Proteinosis. Chinese Journal of Respiratory and Critical Care Medicine, 2014, 13(4): 389-393. doi: 10.7507/1671-6205.2014095 Copy

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7.	Uchida K,Nakata K,Carey B,et al.Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis.J Immunol Methods,2014,402:57-70.
8.	Holbert JM,Costello P,Li W,et al.CT features of pulmonary alveolar proteinosis.AJR Am J Roentgenol,2001,176:1287-1294.
9.	Seymour JF,Presneill JJ.Pulmonary alveolar proteinosis.progress in the first 44 years.Am J Respir Crit Care Med,2002,166:215-235.
10.	Arai T,Inoue Y,Sugimoto C,et al.CYFRA 21-1 as a disease severity marker for autoimmune pulmonary alveolar proteinosis.Respirology,2014,19:246-252.
11.	Bonella F,Bauer PC,Griese M,et al.Pulmonary alveolar proteinosis:New insights from a single-center cohort of 70 patients.Respir Med,2011,105:1908-1916.
12.	Cummings KJ,Donat WE,Ettensohn DB,et al.Pulmonary alveolar proteinosis in workers at an indium processing facility.Am J Respir Crit Care Med,2010,181:458-464.

1. Inoue Y,Trapnell BC,Tazawa R,et al.Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan.Am J Respir Crit Care Med,2008,177:752-762.
2. Trapnell BC,Whitsett JA,Nakata K.Pulmonary alveolar proteinosis.N Engl J Med,2003,349:2527-2539.
3. Kitamura T,Uchida K,Tanaka N,et al.Serological diagnosis of idiopathic pulmonary alveolar proteinosis.Am J Respir Crit Care Med,2000,162:658-662.
4. Lin FC,Chang GD,Chern MS,et al.Clinical significance of anti-GM-CSF antibodies in idiopathic pulmonary alveolar proteinosis,Thorax,2006,61:528-534.
5. Ishii H,Tazawa R,Kaneko C,et al.Clinical features of secondary pulmonary alveolar proteinosis:pre-mortem cases in Japan.Eur Respir J,2011,37:465-468.
6. Lee KN,Levin DL,Webb WR,et al.Pulmonary alveolar proteinosis:high-resolution CT,chest radiographic,and functional correlations.Chest,1997,111:989-995.
7. Uchida K,Nakata K,Carey B,et al.Standardized serum GM-CSF autoantibody testing for the routine clinical diagnosis of autoimmune pulmonary alveolar proteinosis.J Immunol Methods,2014,402:57-70.
8. Holbert JM,Costello P,Li W,et al.CT features of pulmonary alveolar proteinosis.AJR Am J Roentgenol,2001,176:1287-1294.
9. Seymour JF,Presneill JJ.Pulmonary alveolar proteinosis.progress in the first 44 years.Am J Respir Crit Care Med,2002,166:215-235.
10. Arai T,Inoue Y,Sugimoto C,et al.CYFRA 21-1 as a disease severity marker for autoimmune pulmonary alveolar proteinosis.Respirology,2014,19:246-252.
11. Bonella F,Bauer PC,Griese M,et al.Pulmonary alveolar proteinosis:New insights from a single-center cohort of 70 patients.Respir Med,2011,105:1908-1916.
12. Cummings KJ,Donat WE,Ettensohn DB,et al.Pulmonary alveolar proteinosis in workers at an indium processing facility.Am J Respir Crit Care Med,2010,181:458-464.

Chinese Journal of Respiratory and Critical Care Medicine

Relationship of Biomarkers with Clinical and Radiographic Characteristics in Autoimmune Pulmonary Alveolar Proteinosis

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