What are the sign/sx's of chronic interstitial pulmonary diseases?
- Dyspnea and Tachypnea
- End-respiratory crackles
- Cyanosis (late)
- NO wheezing
What is characteristically seen on CXR of someone w/ chronic interstitial pulmonary diseases?
- Bilateral lesions that take form of small nodules, irregular lines, or
- Ground-glass shadows
Grossly, what is the end-stage lung seen in chronic interstitial pulmonary diseases referred to as?
Which 2 chronic interstitial lung diseases are categorized as granulomatous?
- Hypersensitivity pneumonitis
What are some of the enviornmental and occupational factors which may be associated with Idiopathic Pulmonary Fibrosis?
- Cigarette smoking
- Expsoure to metal fumes and wood dust
- Occupations such as: farming, hair-dressing, and stone-polishing
- GERD has also been implicated
Loss-of-function mutations in which 2 genes has been implicated in Idiopathic Pulmonary Fibrosis?
*Encode components of telomerase
Some cases of Idiopathic Pulmonary Fibrosis are associated with a genetic variant leading to ↑ secretion of which mucin?
Idiopathic pulmonary fibrosis is a disease most common in which age group?
The histologic pattern of fibrosis seen in Idiopathic Pulmonary Fibrosis is referred to as what; can be diagnosed based on its characteristic appearance via what imaging modality?
- Usual interstitial pneumonia (UIP)
- On CT
Microscopically, what is the hallmark of Idiopathic Pulmonary Fibrosis; there is coexistence of what?
- Patchy interstitial fibrosis***, which varies in intensity and age
- Coexistence of early (fibroblastic foci) and late (collagenous) lesions
In Idiopathic Pulmonary Fibrosis the formation of cystic spaces lined by hyperplastic type II pneumocytes or bronchiolar epithelium is known as what?
The earliest lesions in Idiopathic Pulmonary Fibrosis contain exuberant what?
Fibroblastic proliferation (fibroblastic foci)
Diagnosis of Idiopathic Pulmonary Fibrosis requires what?
Classic findings on high-resolution CT or pulmonary biopsy
What are the initial sx's of Idiopathic Pulmonary Fibrosis and the later sx's with progression?
- Early ---> ↑ dyspnea on exertion and dry cough
- Later --> hypoxemia, cyanosis and clubbing
What is the prognosis and only definitive therapy for Idiopathic Pulmonary Fibrosis?
- Median survival = 3 years after Dx
- Lung transplantation
Why is it important to recognize pt's with Nonspecific Interstitial Pneumonia?
Have much better prognosis than those w/ UIP
What is the morphology of both the cellular and fibrosing patterns of Nonspecific Interstitial Pneumonia?
- Cellular = mild to moderate chronic interstitial inflammation; in UNIFORM or patchy distribution
- Fibrosing = diffuse or patchy fibrotic lesions of roughly the SAME age
What is a major morphological difference between the lesions of Nonspecific Interstitial Pneumonia and Idiopathic Pulmonary Fibrosis?
In NSIP the lesions are at roughly SAME stage of development
What 4 morphologial findings are absent in Nonspecific Interstitial Pneumonia?
- NO fibroblastic foci
- NO honeycombing
- NO hyaline membranes
- NO granulomas
Which population is most likely to be affected by Nonspecific Interstitial Pneumonia?
Female NON-smokers in their 6th decade of life
What are the key features of Nonspecific Interstitial Pneumonia seen on high-resolution CT?
Bilateral, SYMMETRIC, predominantly lower lobe reticular opacities
How do patients with Cryprogenic Organizing Pneumonia present and what is seen radiographically?
- Cough and dyspnea
- Patchy SUBpleural or peribronchial areas of airspace consolidation
What is the hallmark histology seen with Cryprogenic Organizing Pneumonia?
- Polypoid plugs of loose organizing CT (Masson bodies) within alveolar ducts, alveoli, and often bronchioles
- "Balls of Fibroblasts"
Prognosis and Tx of Cryprogenic Organizing Pneumonia?
- Some recover spontaneously
- Most need oral steroids x 6 months + for complete recovery
Cryprogenic Organizing Pneumonia is most often caused by what?
As a response to infection or inflammatory injury to the lungs
Which 3 autoimmune/CT disease can manifest as interstital lung disease?
- Rheumatoid arthritis
- Systmic sclerosis (scleroderma)
Inhalation of particles, pneumoconiosis, stimulate what inside the lung which lead to progression of disease?
Resident INNATE immune cells
Inhalation of coal dust may be a part of what 4 diseases?
- Coal macules/nodules
- Progressive massive fibrosis --> "Black lung"
- Caplan syndrome
What occurs to the inhaled carbon pigment from coal, leading to Anthracosis?
Engulfed by alveolar or interstitial macrophages, which then accumulate in the CT along lymphatics in lungs or in hilus
Where are lesions (macules and nodules) of simple coal workers' pneumoconiosis most often seen in the lungs?
Upper lobes and upper zones of lower lobes