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Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Pulmonary Tuberculosis III

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Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.
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Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Systemic lupus erythematosus with multiple lung cysts.

Ryo Maeda1, Noritaka Isowa, Hiroshi Miura

  • 1Division of Thoracic Surgery, Matsue Red Cross Hospital, Matsue, Shimane, Japan. ryomaedamatsue@yahoo.co.jp

Interactive Cardiovascular and Thoracic Surgery
|March 14, 2009
PubMed
Summary

This case study highlights a rare instance of recurrent pneumothorax in a woman with systemic lupus erythematosus (SLE) and multiple lung cysts. It underscores the unusual pulmonary complications that can arise from SLE.

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Medical Case Reports

Background:

  • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems.
  • Pulmonary manifestations are common in SLE, including pleuritis, pneumonitis, and pulmonary hypertension.
  • Cystic lung disease is an exceptionally rare complication of SLE.

Observation:

  • A 53-year-old non-smoking woman with a history of SLE since age 29 presented with recurrent spontaneous pneumothorax.
  • Chest computed tomography (CT) revealed multiple bilateral lung cysts.
  • The patient experienced sequential episodes of pneumothorax on the same side over two months.

Findings:

  • The case demonstrates a rare association between SLE, multiple lung cysts, and recurrent pneumothorax.
  • This presentation is unusual, as significant cystic lung disease is not a typical feature of SLE-related pulmonary involvement.

Implications:

  • This case expands the spectrum of known pulmonary complications associated with SLE.
  • It suggests the need for heightened awareness and investigation of cystic lung disease in SLE patients presenting with respiratory symptoms.
  • Further research may elucidate the specific mechanisms linking SLE to cystic lung development.