DIFFUSE PARENCHYMAL LUNG DISEASE (DPLD) IN PATIENTS WITH RHEUMATOID ARTHRITIS (RA): IMPORTANCE OF DIFFERENTIAL DIAGNOSIS

Parenchymal lung disease often presents on imaging with particular patterns that allow for recognition of certain clinical entities that may form the basis for an imaging differential diagnosis. Focal pulmonary opacities and multi-focal pulmonary opacities may be due to an infectious or neoplastic etiology, amongst other possibilities. Segmental/lobar opacities are also associated with a set of differential diagnoses. Diffuse parenchymal disease, while also associated with some infections and neoplasms, can additionally be seen in the setting of pneumoconioses and several idiopathic interstitial pneumonias. Combining clinical information including laboratory results with the imaging findings on chest radiography and computed tomography (CT) allows the physician to formulate an appropriate differential diagnosis or reach one specific diagnosis. This review contains 16 figures, 4 tables and 32 references Keywords: Pulmonary Opacity, Pulmonary Infection, Eosinophilic Pneumonia, Lipoid Pneumonia, Pulmonary Tuberculosis, Organizing Pneumonia, Lung Cancer, Diffuse Lung Disease, Pneumoconiosis, Idiopathic Interstitial Pneumonia

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Transbronchial Lung Cryobiopsy and Video-Assisted Thoracoscopic Lung Biopsy in the Diagnosis of Diffuse Parenchymal Lung Disease: A Meta-analysis of Diagnostic Test Accuracy

Annals of the American Thoracic Society ◽

10.1513/annalsats.201701-086sr ◽

2017 ◽

Cited By ~ 8

Author(s):

Imran H Iftikhar ◽

Lana Alghothani ◽

Alejandro Sardi ◽

David Berkowitz ◽

Ali I Musani

Keyword(s):

Lung Disease ◽

Diagnostic Test ◽

Lung Biopsy ◽

Meta Analysis ◽

Diagnostic Test Accuracy ◽

Test Accuracy ◽

Diffuse Parenchymal Lung Disease ◽

Transbronchial Lung Cryobiopsy ◽

Parenchymal Lung Disease ◽

Parenchymal Lung

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Diffuse Parenchymal Lung Disease

Key Topics in Respiratory Medicine ◽

10.1201/b14650-17 ◽

1998 ◽

pp. 59-63

Keyword(s):

Lung Disease ◽

Diffuse Parenchymal Lung Disease ◽

Parenchymal Lung Disease ◽

Parenchymal Lung

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Analysis of pulmonary features and treatment approaches in the COPA syndrome

ERJ Open Research ◽

10.1183/23120541.00017-2018 ◽

2018 ◽

Vol 4 (2) ◽

pp. 00017-2018 ◽

Cited By ~ 32

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.

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