Resp. Pathology - 5 - SRS Flashcards
What are the seven broad categories of infiltrative/restrictive diseases?
- Idiopathic pulmonary fibrosis
- Cryptogenic Organizing Pneumonia
- Environmental Interstitial Lung Diseases
- Sarcoidosis
- Surfactant Disorders
- Pulmonary Eosinophilia
What are the six causes of environmental interstitial lung disease he highlighted?
- Coal
- Silica
- Asbestos
- Drugs
- Hypersensitivity Pneumonitis
- Tobacco
What pattern do infiltrative diseases have on x-ray?
Reticulonodular or ground glass
What are the four common clinical signs for restrictive lung diseases?
- dyspnea
- tachypnea
- cyanosis
- end-inspiratory crackles
what is the end stage of these fibrosing disorders?
Honeycomb lung
What are the major categories of chronic interstitial lung disease?
- Fibrosing
- Granulomatous
- Smoking related
- Other…
What are the fibrosing diseases?
3 bolded
4 non-bolded
- Usual interstitial pneumonia (idiopathic pulmonary fibrosis)*
- Nonspecific interstitial pneumonia*
- Cryptogenic organizing pneumonia*
- Connective tissue disease-associated
- Pneumoconioses
- Drug reactions
- Radiation pneumonitis
What are the etiologies for:
- Usual interstitial pneumonia (idiopathic pulmonary fibrosis)*
- Nonspecific interstitial pneumonia*
- Cryptogenic organizing pneumonia*
Unknown
What are the granulomatous interstitial lung diseases?
1 bolded
1 bolded AND italicized
1 plain font
(3 total)
- Sarcoidosis*
- Hypersensitivity pneumonitis
- Eosinophilic (eosinophilic pneumonias)*
What are the two smoking-related interstitial lung diseases?
- Desquamative interstitial pneumonia
- Respiratory bronchiolitis-associated interstitial lung disease
What are the three interstitial lung diseases that fall into the “other” category?
- Pulmonary Langerhans cell histiocytosis
- Pulmonary alveolar proteinosis *
- Lymphoid interstitial pneumonia *
What is another term for the idiopathic pulmonary fibrosis?
Usual interstitial pneumonia
What pattern is shown on this x-ray?
Reticular pattern (or knot? Nut? pattern… couldn’t understand what he said)
The biopsy attached shows early phase usual interstitial pneumonia, with mononuclear infiltrates and fibrotic thickening of the interstitium.
What cells are being slaughtered here and what cells are taking their place?
What is causing the pathology in this condition?
- • Type I pneumocyte death, type II hyperplasia
- Pathology occurs as a result of:
- TH2 lymphocytes,
- cytokines,
- macrophages,
- fibroblast/myofibroblast proliferation (TGF-β1 driven),
- collagen deposition
What gene mutations predispose one to development of idiopathic pulmonary fibrosis?
TERT and TERC
There is some evidence the activated fibroblasts in idiopathic pulmonary fibrosis exhibit signaling abnormalities in a particular pathway that leads to synthesis and deposition of collagen leading to interstitial fibrosis and eventual respiratory failure. What pathway is this?
PI3K/AKT pathway
When epithelium is injured it releases TGF-B which not only stimulates fibroblasts and myofibroblasts to deposit collagen, it also inhibits what inhibitor of collagen production?
Caveolin in fibroblasts
Idiopathic pulmonary fibrosis is an insidious, upredictable disease that typically strikes in middle age. (>50). The patient may complain of dyspnea, dry cough, hypoxemia with cyanosis and have digital clubbing.
What is the median survival?
3 years
The repeated cycles of alveolitis in idiopathic pulmonary fibrosis lead to healing/scarring and patchy interstitial fibrosis.
What part of the lung is typically involved?
What is the treatment for this condition?
Predominantly the subpleural/interlobar and lower lobe
Lung transplant. (does not respond well to anti-inflammatory agents)
This image shows fibrosis of the subpleural region which is typical of the changes seen in?
Usual interstitial pneumonia
This image of usual interstitial pneumonia show fibrolastic foci with fibers running parrallel to the surface and bluish myxoid extracellular matrix.
What is shown on the left side of the image?
Honeycombing - associated with the end stages of this disease
What is shown here?
Honeycomb lung
What will the lesions in idiopathic pulmonary fibrosis look like as far as age and progression?
Variable age and progression
Patient presents with dyspnea and cough of several months duration, is middle age and a non-smoker. You biopsy the lung at the lingula and get lucky. The lesions you find are all at the same stage of progresion with lymphocytes and some plasma cells. There is mild patchy or diffuse interstitial fibrosis present. What is the disease this patient likely has?
Do you expect to see honeycombing or fibroblastic foci?
Nonspecific interstitial pneumonia
Do not see fibroblastic foci or honeycombing. If you do, then it may have to be reclassified d/t progression.
You see a patient with cryptogenic organizing pneumonia for a check up. Being the sweet doc that you are, you this disease has what two specific characteristics?
The patient expresses concerns about their lung turning into some kind of cereal lung. What do you tell them?
- All connective tissue is of the same age
- No interstitial fibrosis
Since no fibrosis, does not progress to honeycomb lung. Reassure the patient this will not happen.
You obtain the following biopsy from a patient with cough and dyspnea. What is indicated by the green and blue arrows respectively?
What disease are these findings indicative of?
What is the treatment?
Cryptogenic Organizing Pneumonia (COP)= Bronchiolitis Obliterans Organizing Pneumonia (BOOP)
Green arrow = Masson Bodies
Blue Arrow = Obliterated bronchiole
Treat with oral steroids for 6 or more months
Cryptogenic Organizing Pneumonia (COP) is of unknown etiology by definition but organizing pneumonias can be a reaction to what two other events?
- Infection
- Inflammatory process
What are the examples of collagen vascular disorder related autoimmune interstitial lung diseases?
6
- •Rheumatoid arthritis (30-40% abnormal PFT Caplan syndrome - combination of rheumatoid arthritis and pneumoconiosis
- •Scleroderma (systemic sclerosis)
- •Systemic lupus erythematosus
- •Sjӧgren syndrome
- •Polymyositis/dermatomyositis
- •Mixed connective tissue disorder
Your patient presents with evidence of lung disease, you biopsy the lung and find the two particulalry significant samples attached to this card. What are the findings you see here and what do they point to this patient having?
Image at left: Follicular bronchiolitis - parabronchiolar lymphoid follicles seen here indicate a likely autoimmune process.
Image at right: a rheumatoid nodule - granuloma form surrounding necrotic tissue.
These two things combine to strongly suggest Rheumatoid Arthritis
Environmentally related lung diseases are inhalation related but can have a genetic predisposition to developing disease. What particles are dangerous?
- 1-5 micron size are most dangerous
- size, shape and buoyancy play a role - an example of shape is asbestos, shaped like a thin spear.
- soluble/cytotoxics
- concomitant irritants
What is pneumoconiosis?
originally limited to mineral dust inhalation but now all inclusive of pretty much anything you breath at work
What are the mineral dusts that cause pneumoconiosis?
(4 bold and likely on exams, three extra)
- Coal dust
- Silica
- Asbestos
- beryllium
- iron oxide
- barium sulfate
- tin oxide
What diseases does coal dust cause? 4
- Anthracosis
- Macules
- Progressive massive fibrosis
- Caplan syndrome
What diseases does silica inhalation lead to? 2
Silicosis
Caplan syndrome