Clinical History:
- A 40-year-old woman has had a cough that is productive of purulent sputum. On occasion, she notes spots of blood in the sputum. She has been hospitalized for pneumonia twice in the past year. She does not have dyspnea, but she has recently developed wheezing episodes. Laboratory findings include an elevated WBC count with neutrophilia and left shift. Sputum culture grew 3+ Serratia marcescens and 2+ Pseudomonas aeruginosa. A chest radiograph reveals abnormalities involving the right lower lobe.
- What is the most striking architectural change in the lung? Describe it and the related changes?
There is bronchiectasis, with the bronchi markedly dilated. There has been destruction of the bronchial walls, and there is an intense chronic inflammatory infiltrate around them. Note that although these are "large" bronchi, there is no cartilage present. The epithelial lining is focally eroded, with underlying granulation tissue. There is peribronchial scarring and parenchymal collapse.
- What are the possible etiologies of this condition?
Multiple possible etiologies, including congenital bronchiectasis, post obstructive, secondary to necrotizing pneumonia (Staphylococcal or TB), immunodeficiency disorders, or the immotile cilia and Kartagener's syndromes.
Clinical History: (images 4.5 - 4.8):
- A 24-year-old man had the findings at autopsy shown below. At autopsy his heart weighed 450 grams and had a dilated right heart with right ventricular wall measuring 0.7 cm thick (normal < 0.5 cm).
- What do you think is the etiology for his pulmonary findings? What changes do you see in the pulmonary vessels, image 4.8? How does this relate to autopsy findings?
The extensive nature of the bronchiectasis here, along with the young age of the patient, is consistent with cystic fibrosis. This is probably the most common etiology overall for bronchiectasis, but in such cases both lungs are extensively involved. The pulmonary arteries show changes of pulmonary hypertension, as shown by image 4.8 which highlights a plexiform arteriopathy typical for pulmonary hypertension. There is thickening of arterial and arteriolar walls, and narrowing of lumina. This is related to the relative cardiac hypertrophy, and thickening of the right ventricular wall (normal is up to 0.5 cm thick), indicating a degree of cor pulmonale.
- Do you think he was currently suffering from acute respiratory symptoms when he died? Why or why not?
Probably, as there are areas of consolidation and acute pneumonia. Depending on the severity of the generalized process throughout the lungs he may or may not have been in severe distress. Many cystic fibrosis patients have chronic infections with Pseudomonas.
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