ProSP-B as a potential biomarker in diffuse parenchymal lung disease (DPLD)

Pneumologie ◽  
2012 ◽  
Vol 66 (06) ◽  
Author(s):  
N Kahn ◽  
A Rossler ◽  
K Hornemann ◽  
T Muley ◽  
A Warth ◽  
...  
Keyword(s):  
Lung Disease ◽  
Potential Biomarker ◽  
Diffuse Parenchymal Lung Disease ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

scholarly journals Categorizing diffuse parenchymal lung disease in children

2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Matthias Griese ◽  
Armin Irnstetter ◽  
Meike Hengst ◽  
Helen Burmester ◽  
Felicitas Nagel ◽  
...  
Keyword(s):  
Lung Disease ◽  
Diffuse Parenchymal Lung Disease ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

scholarly journals Genetic testing in diffuse parenchymal lung disease

2012 ◽  
Vol 7 (1) ◽  
pp. 79 ◽  
Author(s):  
Paolo Spagnolo ◽  
Fabrizio Luppi ◽  
Stefania Cerri ◽  
Luca Richeldi
Keyword(s):  
Genetic Testing ◽  
Lung Disease ◽  
Diffuse Parenchymal Lung Disease ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

scholarly journals Update in Diffuse Parenchymal Lung Disease 2011

2012 ◽  
Vol 186 (1) ◽  
pp. 24-29 ◽  
Author(s):  
Tracy R. Luckhardt ◽  
Joachim Müller-Quernheim ◽  
Victor J. Thannickal
Keyword(s):  
Lung Disease ◽  
Diffuse Parenchymal Lung Disease ◽  
Parenchymal Lung Disease ◽  
Parenchymal Lung

scholarly journals Analysis of pulmonary features and treatment approaches in the COPA syndrome

2018 ◽  
Vol 4 (2) ◽  
pp. 00017-2018 ◽  
Author(s):  
Jessica L. Tsui ◽  
Oscar A. Estrada ◽  
Zimu Deng ◽  
Kristin M. Wang ◽  
Christopher S. Law ◽  
...  
Keyword(s):  
Family History ◽  
Lung Disease ◽  
Alveolar Haemorrhage ◽  
Diffuse Alveolar Haemorrhage ◽  
Diffuse Parenchymal Lung Disease ◽  
History Of Disease ◽  
History Of ◽  
Parenchymal Lung Disease ◽  
Copa Syndrome ◽  
Parenchymal Lung

The COPA syndrome is a monogenic, autoimmune lung and joint disorder first identified in 2015. This study sought to define the main pulmonary features of the COPA syndrome in an international cohort of patients, analyse patient responses to treatment and highlight when genetic testing should be considered.We established a cohort of subjects (N=14) with COPA syndrome seen at multiple centres including the University of California, San Francisco, CA, USA. All subjects had one of the previously established mutations in the COPA gene, and had clinically apparent lung disease and arthritis. We analysed cohort characteristics using descriptive statistics.All subjects manifested symptoms before the age of 12 years, had a family history of disease, and developed diffuse parenchymal lung disease and arthritis. 50% had diffuse alveolar haemorrhage. The most common pulmonary findings included cysts on chest computed tomography and evidence of follicular bronchiolitis on lung biopsy. All subjects were positive for anti-neutrophil cytoplasmic antibody, anti-nuclear antibody or both and 71% of subjects had rheumatoid factor positivity. All subjects received immunosuppressive therapy.COPA syndrome is an autoimmune disorder defined by diffuse parenchymal lung disease and arthritis. We analysed an international cohort of subjects with genetically confirmed COPA syndrome and found that common pulmonary features included cysts, follicular bronchiolitis and diffuse alveolar haemorrhage. Common extrapulmonary features included early age of onset, family history of disease, autoantibody positivity and arthritis. Longitudinal data demonstrated improvement on chest radiology but an overall decline in pulmonary function despite chronic treatment.

Sign in / Sign up

Export Citation Format

Share Document