December 2021 Pulmonary Case of the Month: Interstitial Lung Disease with Red Knuckles

No abstract available. Article truncated after 150 words. History of Present Illness A 56-year-old man was referred for a second opinion on recent onset of diffuse parenchymal lung disease. He had started noting mild dyspnea with yard work approximately in March 2021. His symptoms progressed over the next month with increasing shortness of breath and some fever. He presented to outside emergency department on April 17, 2021 and chest CT showing patchy ground-glass opacities with some areas of irregular consolidation (Figure 1). He was subsequently seen by an outside pulmonologist and started empirically on prednisone (50 mg/day). An outside lung biopsy had been performed which showed nonspecific interstitial pneumonitis. There was some improvement in his symptoms and his prednisone dose was reduced to 20 mg/day; however, his symptoms subsequently worsened with saturations noted to drop to 85% with any ambulation. He also had swelling of his left face and a biopsy of the parotid gland with the findings …

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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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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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