Week 19
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Quiz 19 Context:
You are continuing to evaluate a 60-year-old female with
chronic exercise related dyspnea and chronic dry cough, in the
setting of a history of previous smoking. There is no history
of environmental exposure, infectious exposure, cardiac disease,
connective tissue disease, exposure to medications or drugs with
adverse effects. Physical examination revealed dry rales at the
bases of the lung fields as well as clubbing.
A CT of the
chest is obtained, it shows subpleural fibrosis with honeycombing
and traction bronchiectasis.
The patient
subsequently opts to undergo diagnostic biopsy. The results of
the biopsy are shown above.
Sorry, that is not the most likely answer. Try Again.
c. Excellent!
These images reveal pathology consistent with UIP, which is the pathologic diagnosis of idiopathic pulmonary fibrosis. UIP appears as a patchy and temporally variegated (both early and late components) fibrosis with little inflammation. An area of proliferating fibroblasts located at the leading edge of new fibrosis, known as the fibroblastic focus (& visible on the higher magnification slide), is the hallmark of the disease.
You have correctly identified this week's Image of the Week. Now test your knowledge of other aspects of idiopathic interstitial lung disease. Do you know…
…The proposed importance of cytokine dysregulation (involving abnormal elevations of type 2 cytokines and reduction of type 1 cytokines) thought to be a causative factor in the development of idiopathic pulmonary fibrosis? What other pulmonary diseases involve these cytokines but do not result in the pattern of injury seen with IPF?
…the diagnostic accuracy of the clinical evaluation combined with high-resolution CT compared with that of pathology obtained on surgical lung biopsy, as reported in a prospective study of IPF?
…the American Thoracic Society recommendations for first-line drug agents to be used in combination with a steroid for the initial treatment of IPF?
The answers to these questions, and a concise review of the pathogenesis, diagnosis, and treatment of idiopathic interstitial lung disease, can be found here within The Cleveland Clinic Disease Management Project.
These pathology specimens are most consistent with which of the following?
- Patchy and temporally variegated fibrosis, with fibroblastic focus.
Dx: usual interstitial pneumonitis (UIP)
- Uniform appearance of interstitial
inflammation in all areas of the biopsy. Dx: nonspecific interstitial
pneumonitis (NSIP)
- Organizing pneumonia within
the alveolar ducts & bronchioles. Dx: bronchiolitis obliterans-organizing
pneumonia (BOOP)
- Focal bronchiolar pigmented
macrophage accumulation. Dx: respiratory bronchiolitis associated
interstitial lung disease (RB-ILD)
- Diffuse
interstitial infiltration of T lymphocytes grouped into germinal
centers. Dx: lymphocytic interstitial pneumonitis (LIP)
