Primer 24 - Restrictive Lung Disease Flashcards

1
Q

What is interstitial lung disease?

A

Noninfectious, inflammation and pathological changes of the alveolar wall.

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2
Q

What is eosinophilic granuloma?

A

A subset of histiocytosis X, there are Langherhan-like cells that have tennis-racquet organelles called Birbeck granules. Clinicallt, unifocal lytic lesions are found in bones such as the skull, ribs and femur. Because of this, bone pain and pathologic fractures are common.

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3
Q

What is a risk factor for eosinophilic granuloma?

A

Smoking.

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4
Q

What is Goodpasture syndrome?

A

An autoimmune disease that can cause pulmonary hemorrhage and acute or rapidly progressive glomerulonephritis. It is characterized by Anti-basement membrane antibodies.

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5
Q

What is idiopathy pulmonary fibrosis?

A

Fibrosis of the lungs that does not have a known cause. It is causes interstitial lung disease and is quite fatal.

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6
Q

What is Sarcoidosis?

A

Immune mediated, widespread noncasiating granulomas and elevated serum ACE levels. Causes interstitial fibrosis with bilateral lymphadenopathy.

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7
Q

What are the clinical symptoms of Sarcoidosis?

A

[GRUELING-AV]
Granulomas, Rheumatoid arthritis, Uveitis (eye), erthema nodosum (tibial), Lymphadenopathy (hilar, bilateral), Idiopathic, Not TB, Gamma globulinemia, Increase in ACE and Increase in vitamin D.

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8
Q

What is the major treatment for sarcoidosis?

A

Steroids.

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9
Q

What is hypersensitivity pneumonitis?

A

Patients exposed to organic dust leads to inflammation of the alveoli; this involves farmer’s lungs and pigeon breeder lungs.

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10
Q

What is the general mechanism of damaged caused by pneumoconioses?

A

Macrophages causing local inflammation (release fibrogenic factors) as they absorb the inorganic particles in the lung.

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11
Q

What is anthracosis?

A

A pneumoconioses caused by the inhalation of carbon dust; the alveolar macrophages ingest the dust causing a black deposits on the lungs. Usually asymptomatic.

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12
Q

What is coal-workers pneumoconiosis?

A

A level up from anthracosis, it is due to carbon but now there are bronchiolar macules and signs of fibrosis that can lead to cor pulmonary. There is no increase in lung cancer.

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13
Q

What section of the lungs does asbestosis affect?

A

Lower lobes.

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14
Q

What are ferruginous bodies?

A

A histopathological finding in interstitial lung disease suggestive of significant asbestos exposure. They appear as small brown nodules in the septum of the alveolus that resemble dumbbells. They are iron-rich material derived from proteins such as ferritin and hemosiderin are believed to be formed by macrophages that have phagocytosed and attempted to digest the fibers.

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15
Q

Which types of cancer is asbestosis associated with?

A

Increased risk of bronchogenic carcinoma and mesothelioma.

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16
Q

Which types of jobs is silicosis associated with?

A

Sandblasting, mines and foundries.

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17
Q

What is silicosis?

A

A form of pneumoconiosis, it is when their is inhalation of silica and the macrophages in the lungs try to digest it. Can cause calcification of the hilar lymphnodes “egg shells” on X-ray.

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18
Q

Which section of the lungs does silicosis affect?

A

The upper lobes of the lungs.

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19
Q

What is silicosis associated with?

A

Increased risk of contracting TB.

20
Q

What is Berylliosis?

A

Exposure to beryllium and its many forms, it causes noncasiating granulomas. It increases risk of lung cancer.

21
Q

What are two pathologies that causes noncaseating granulomas?

A

Sarcoidosis and Berylliosis.

22
Q

RFF: Langerhan cells.

A

Eosinophilic granuloma.

23
Q

RFF: Anti-basement membrane Ab.

A

Goodpasture syndrome.

24
Q

RFF: Increased ACE levels.

A

Sarcoidosis.

25
Q

RFF: Organic Dust (Farmers).

A

Hypersensitivity pneumonitis.

26
Q

RFF: Black deposits and asymptomatic.

A

Anthracosis.

27
Q

RFF: Black deposits, symptomatic.

A

Coal worker’s lung.

28
Q

RFF: Ferruginous bodies.

A

Asbestosis.

29
Q

RFF: Sand blasting.

A

Silicosis.

30
Q

What is ARDS and what are the causes?

A

Acute Respiratory Distress Syndrome. It can be trauma, sepsis, shock, toxic gas inhalation, gastric aspiration, uremia, acute pancreatitis, high [O2], heroin OD. It causes inflammatory cells/mediators and oxygen free radicals that does damage to endothelial or alveolar epithelial (type 1) cells.

31
Q

What other pathologies can ARDS lead to?

A

Diffuse alveolar Damage (DAD), and Hyaline Membrane Disease (HMD).

32
Q

What is Neonatal Respiratory Distress Syndrome?

A

Respiratory distress caused by surfactant deficiency (Dipalmitoyl Phosphatidylcholine).

33
Q

What are some risk factors that can cause Neonatal respiratory distress syndrome?

A

Prematurity of the baby, maternal diabetes, and cesarian section.

34
Q

What is Sleep apnea?

A

When a person stops breathing for at least 10 seconds during sleep.

35
Q

What is the treatment for central sleep apnea?

A

Caffeine.

36
Q

What is the difference between sleep apnea and obstructive sleep apnea?

A

In sleep apnea, there is no drive to breathe when sleeping while obstructive sleep apnea, there is an obstruction, usually the soft palate falling back.

37
Q

What is the treatment for obstructive sleep apnea?

A

Weight loss and Continuous positive airway pressure (CPAP).

38
Q

What stimulant can patients with obstructive sleep apnea benefit from?

A

Modafinil.

39
Q

A preterm infant has difficulty breathing. An X-ray reveals diffuse air space and interstitial opacities, with air bronchograms. What is the diagnosis, and what could have prevented this condition..

A

Neonatal respiratory distress syndrome. Prevented with maternal steroids 24-48 hours prior to delivery.

40
Q

H&E of lung biopsy from a plumber shows elongated structures with clubbed ends in tissue. What is the diagnosis, and what is he at increased risk for?

A

Asbestosis and he is in an increased risk of mesthelioma and bronchogenic carcinoma.

41
Q

What do patients with silicosis need to be worried about?

A

Increased susceptibility to TB.

42
Q

RFF: bilateral hilar adenopathy and uveitis.

A

Sarcoidosis.

43
Q

RFF: Vasculitis and glomerulonephritis.

A

Goodpasture syndrome and Wegener’s granulomatosis.

44
Q

RFF: Honecomb lung on x-ray.

A

Interstitial fibrosis.

45
Q

RFF: “Tennis-racket” shaped cytoplasmic organelles.

A

Birbeck granules (eosinophilic granuloma).

46
Q

What is the classic facial feature with Wegener’s granulomatosis?

A

Saddle nose: when the bridge of the nose collapses due to the destruction of nasal cartilage.

47
Q

What are some extrapulmonary factors that can cause restrictive lung disease?

A

Morbid obesity, Guillain-Barre, myasthenia gravis, khyphosis.