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Clinical Medicine II > Reactive/Interstitial Pulmonary Disorders > Flashcards

Reactive/Interstitial Pulmonary Disorders Flashcards

1
Q

With restrictive diseases, what do you expect to see on a PFT?

A

Normal or increased FEV1/FVC

Decreased lung volumes (TLC, RV)

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2
Q

Dyspnea and/or insidious non-productive cough and constitutional symptoms

PE reveals fine bibasilar inspiratory crackles, clubbing of fingers

A

Idiopathic pulmonary fibrosis

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3
Q

What would you expect to see on a CT/CXR with a patient who has idiopathic pulmonary fibrosis?

A

Diffuse reticular opacities (honeycombing)

Ground glass opacities

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4
Q

What would you expect to see on a lung biopsy of a person who has idiopathic pulmonary fibrosis?

A

Honeycombing (Large cystic airspaces from cystic fibrotic alveolitis)

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5
Q

Chronic progressive interstitial scarring (fibrosis) from persistent inflammation which leads to loss of pulmonary function with restrictive component of unknown cause. Most common in men 40-50 y.o.

A

Idiopathic pulmonary fibrosis (Fibrosing interstitial pneumonia)

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6
Q

What would you expect to see in a PFT of a patient with idiopathic pulmonary fibrosis?

A

PFT consistent with restrictive lung disease

Decreased DLCO

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7
Q

How do you manage a patient with idiopathic pulmonary fibrosis?

A

Smoking cessation and oxygen therapy

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8
Q

What is the only known cure for idiopathic pulmonary fibrosis?

A

Lung transplant

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9
Q

What is the most common diagnosis among patients with interstitial lung disease?

A

Idiopathic pulmonary fibrosis

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10
Q

Chronic fibrotic lung disease secondary to inhalation of mineral dust. The mineral dust is ingested by alveolar macrophages which triggers inflammation and the release of chemical mediators. This causes parenchymal fibrosis which leads to restrictive lung disease and decreased lung compliance.

A

Pneumoconiosis

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11
Q

What are the most common pneumoconioses?

A

Asbestosis
Coal workers pneumoconiosis (Black lung dz)
Berylliosis
Silicosis

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12
Q

Results from silica dust inhalation (mining, quarry work with granite/slate/quarts, pottery sandblasting

A

Silicosis

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13
Q

Type of pneumoconiosis often asymptomatic with or without dyspnea on exertion and a nonproductive cough

A

Silicosis

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14
Q

CXR reveals multiple small, round nodular opacities primarily in upper lobes. + eggshell calcifications of hilar and mediastinal nodes

A

Silicosis

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15
Q

What is the treatment for silicosis?

A

Supportive: Steroids, O2, rehab

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16
Q

Caused by inhalation of coal, carbon mine dust.

A

Coal workers Pneumoconiosis (black lung dz)

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17
Q

CXR shows Small upper lobe nodules with hyperinflation of lower lobes (resembles centrilobar emphysema seen with smoking)

A

CWP

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18
Q

What is Caplan syndrome?

A

Coal workers pneumoconiosis + RA

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19
Q

How do you manage CWP?

A

Supportive

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20
Q

Seen with high-technology fields, electronics, aerospace, ceramics, neckwear power, fluorescent light bulbs and tool and die manufacturing that causes noncaseating granulomas. Results in an increased risk of lung, stomach, and colon cancer

A

Berylliosis

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21
Q

Symptoms are similar to silicosis + joint pains, weight loss, and fever

A

Berylliosis

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22
Q

CXR reveals diffuse infiltrates and hilar adenopathy; increased interstitial lung markings, normal 50% of the time

A

Berylliosis

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23
Q

What is the management plan for berylliosis?

A

Steroids, O2, methotrexate if corticosteroids fail

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24
Q

Due to cotton exposure in patients employed in the textile industries; aka “Monday fever” or “Brown lung disease”

A

Byssinosis

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25
Sxs: Dyspnea, wheezing, cough, chest tightness. Symptoms tend to get worse at the beginning of the work week, then improve later in the week or on the weekend.
Byssinosis
26
Seen 15-20 years after long term exposure to asbestos (destruction/renovation of old buildings, insulation, ship building, pipe fitters) Symptoms similar to those of silicosis
Asbestosis
27
What does asbestosis put patients at an increased risk for?
Malignant mesothelioma of pleura Bronchogenic carcinoma Tuberculosis
28
CXR reveals pleural plaques* (pleural thickening), interstitial fibrosis (honeycomb lung); primarily seen in *lower lobes* "Shaggy heart sign"
Asbestosis
29
Asbestosis is commonly seen in the _______ lung lobes whereas CWP and silicosis is primarily seen in the ______ lung lobes
Lower: Asbestosis Upper: CWP/silicosis
30
Biopsy revelas linear asbestos bodies in lung tissue (brown rods due to iron/protein deposits)
Asbestosis
31
What is the management plan for patients with asbestosis?
Steroids, O2 with or without lung transplant
32
What reduces synergistic cancer risk in those with asbestosis, and is VERY important in their treatment?
Smoking cessation!
33
Besides smoking cessation, what is very important to tell patients with any form of pneumoconiosis?
To be vaccinated with pneumococcal and influenza vaccines
34
What is a possible complication of CWP?
Progressive massive fibrosis
35
What is a complication of berylliosis?
Requires chronic steroids - puts at risk for disorders that are triggered by heavy steroid use
36
Chronic multisystemic, inflammatory, granulomatous disorder of unknown etiology. All organs are affected. Due to a disordered *immune* regulation in genetically predisposed individuals exposed to certain environmental antigens.
Sarcoidosis
37
Which organ is the most commonly affected by sarcoidosis?
Lungs (90%)
38
What is the second most commonly affected organ in patients with sarcoidosis?
Skin
39
Mass of immune cells formed by macrophages, epithelioid cells (activated macrophages) and multinucleated giant cells surrounded by a rim of lymphocytes (t4), mast cells, and fibroblasts
Granuloma
40
Why are granulomas a problem within the body?
They take up space disrupting the normal structure and/or function of the tissues they form in
41
Granulomas either resolve, or undergo ________
fibrosis
42
How many patients with sarcoidosis are asymptomatic?
50%
43
What are the 7 different clinical manifestations that can occur with sarcoidosis?
1. Pulmonary (90%) 2. Lymphadenopathy 3. Skin (25%) 4. Visual (20%) 5. Myocardial 6. Rheumatologic 7. Neurologic (5%)
44
Sxs: Dry (nonproductive) cough, dyspnea, chest pain These are all clinical manifestations of what type of sarcoidosis?
Pulmonary symptoms of sarcoidosis
45
- Painless intrathoracic lymphadenopathy - Hilar nodes with or without paratracheal involvement These are all clinical manifestations of what type of sarcoidosis?
Lymphadenopathy symptoms of sarcoidosis
46
Bilateral tender red nodules on anterior legs that can resolve spontaneously within 2-4 weeks; increased incidence in northern europeans
Erythema nodosum
47
Violaceous, raised discoloration of so, ear, cheek and chin (resembles frost bite)
Lupus pernio pathognomonic
48
What is the most common dermatologic manifestation of sarcoidosis?
Maculopapular rash
49
What are the dermatologic clinical manifestations of sarcoidosis?
- Erythema nudism - Lupus pernio pathognomonic - Maculopapular rash - Subcutaneous nodules/waxy nodules - Parotid enlargement
50
Blurred vision, ocular discomfort, photophobia, ciliary flush, and floaters are all symptoms of what disorder? This is a clinical manifestation of what disease?
Uveitis Clinical manifestation of sarcoidosis
51
Why should you always do an eye exam if you think your patient has sarcoidosis?
Because ocular involvement occurs in >20% of cases!
52
What are the visual clinical manifestations of sarcoidosis?
- Uveitis - Conjunctivitis (tearing, erythema) - Glaucoma, cataracts, blindness
53
What are the myocardial sxs of sarcoidosis?
Arrythmias, cardiomyopathies
54
Arthralgias, fever, malaise, weight loss, and hepatosplenomegaly are all what type of clinical manifestations related to sarcoidosis?
Rheumatologic sxs related to sarcoidosis
55
What CN Palsy is common with sarcoidosis?
Facial nerve/CN 7
56
What are the neurologic sxs that are associated with sarcoidosis?
CN palsies, diabetes insipidus, hypthalamic/pituitary lesions
57
What is diagnosis based on when diagnosing sarcoidosis?
1. Compatible clinical/radiologic findings 2. Noncaseating granulmoas 3. Exclusion of other dz
58
What is the classic non-specific histological finding with a tissue biopsy when trying to diagnose sarcoidosis?
Noncaseating granulomas (NCG)
59
What does "noncaseating" mean?
No central necrosis in the granuloma
60
In patients with sarcoidosis, a CXR is almost always ____
abnormal
61
What are the two things that can be seen on a CXR in patients with sarcoidosis?
Bilateral hilar lymphadenopathy*** Classic symptom | Or interstitial lung disease that shows opacities with or without a ground glass appearance
62
What stage is a patient with sarcoidosis in if their CXR shows BHL and they are asymptomatic or have mild pulmonary symptoms?
Stage 1
63
What stage is a patient with sarcoidosis in if their CXR shows BHL and ILD with moderate pulmonary symptoms?
Stage 2
64
What stage is a patient with sarcoidosis in if their CXR shows ILD only?
Stage 3
65
What stage is a patient with sarcoidosis in if their CXR shows Fibrosis with volume loss and restrictive disease patterns?
Stage 4
66
When will restrictive PFT results be seen in a patient with sarcoidosis?
Advanced disease
67
What is the most common PFT finding in a patient with sarcoidosis?
Decreased DLCO
68
What are PFTs commonly used for in patients with sarcoidosis?
To monitor response to treatment
69
Remind me, what are the restrictive PFT patterns again?
Normal or INCREASED FEV1/FVC Normal or DECREASED FVC DECREASED lung volumes: VC, RV, FRC, *TLC*
70
What should you expect to see on a CT scan of a patient with sarcoidosis?
Hilar/mediastinal lymphadenopathy Ground glass opacities Parenchymal infiltration
71
If you see fibrosis on a CT scan of a patient with sarcoidosis, what does that mean?
A poor prognosis
72
What are some common lab findings in patients with sarcoidosis?
``` **Increased ACE** (40-80%) Hypercalciuria Eosinophilia **Cutaneous Anergy** Increased IgG and ESR ```
73
Why would you see an increase in angiotensin converting enzyme in patients with sarcoidosis?
Granulomas secrete ACE!
74
What does a result of "cutaneous anergy" mean?
There is immune unresponsiveness
75
How many patients with sarcoidosis spontaneously resolve?
40%
76
How many patients with sarcoidosis improve with treatment?
40%
77
How many patients with sarcoidosis progress to irreversible lung injury?
20%
78
Which stages of sarcoidosis are associated with a poorer prognosis?
Interstitial lung disease and lupus pernio
79
How do you manage sarcoidosis?
1. Observation (majority of patients have spontaneous remission!) 2. Oral corticosteroids 3. Methotrexate 4. Hydroxychlorquine 5. NSAIDS for msk symptoms and erythema nordosum 6. Single lung transplant
80
What is the treatment of choice if treatment is needed in sarcoidosis?
Oral corticosteroids: Reduce granuloma formation and fibrosis
81
What are the indications for treatment with sarcoidosis? *Know this!*
Worsening sxs, deteriorating lung function, progressive radiologic decline
82
Before initiating steroid treatment with sarcoidosis, what should you first rule out?
TB | Infectious causes
83
If you are just observing a patient with sarcoidosis for treatment, how should you follow up?
Periodic evaluation with CXR and PFTs

Clinical Medicine II (10 decks)

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