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45 Cards in this Set

  • Front
  • Back
Interstitial lung disease:

... present on auscultation, digital ..., and relapsing infiltrates with fever may be present in some interstitial diseases.

On X-ray, these disease states will produce a ... pattern.
Crackles
clubbing
reticular nodular
Tests that help diagnose the disease include:

-Chest x-ray
-pulmonary function tests – will show a ... pattern
-lung biopsy to rule out specific causes of interstitial disease (nowadays, high resolution CT scanning)
-RBC count
-hemoglobin – Hb will be ...
-CBC – increase in eosinophils in certain ones
-ACE levels – increased ACE in ...
-high index of suspicion
restrictive
increased
sarcoidosis
... is a disease characterized by diffuse interstitial fibrosis of unknown etiology and frequently occurs between the ages of 50-70.

Pt will often have a preceding ... prior. Persistent pneumonia. A pt who presents to you several times w/ persistent pneumonia --> have a high index of suspicion of ....
Idiopathic interstitial fibrosis (IIF)
URI
interstitial fibrosis
Clinical picture: (IIF)

Patients present with ... cough and shortness of breath and sometimes with fever, arthralgias and ... phenomenon.

... (40-70%) tachypnea and persistent coarse basal crackles are encountered on physical exam. Often they are using ... muscles of inspiration for tidal breathing.
dry
Raynaud’s
Clubbing
accessory
Clinical picture: IIF

CXR: Normal to reticulonodular to ... (usually in late stage). HRCT may show ground glass appearance or honeycomb cysts. In ... interstitial pneumonitis alveolar features can be recognized.

PFT show a ... and diffusion defects. Patients are hypoxic and desaturate significantly with ....

In 25-45%, serum cryoglobulins, RF and ANA are present.

BAL (bronchiolar lavage): Predominance of .... (although some can be eosinophils)
honeycombing
Desquamative
restrictive
exercise
neutrophils
Pathology: IIF

Early IPF: Desquamation predominates where alveolar macrophages fill alveolar spaces (...).

Late IPF: Inflammation of alveolar walls continues and fibroblast proliferation with collagen formation occurs (...).
desquamative interstitial pneumonitis or DIP

usual interstitial pneumonitis of UIP
Diagnosis: IIF

By ... (using hx and bx), it will be necessary to rule out other known causes for fibrosis like Silicosis or drugs. A long list of conditions can give rise to interstitial fibrosis.

Transbronchial lung ... can identify interstitial fibrosis. Usually need ... lung biopsy to confirm.

HRCT

CXR honeycombing
exclusion
biopsy
open
Tx: IIF

-Therapy is useful in ... stages when there is significant inflammatory process.
-Once the fibrosis is ... or if the chest X-ray has been stable for years or if there is ..., therapy is of no benefit.
-Corticosteroids ± immunosuppressants may help (cyclophosphamide or azathioprine); anecdotal reports describing colchicine or methotrexate or cyclosporine.
-... is often prescribed for these patients, probably not influencing pulmonary hypertension.
-In some cases Plasmapheresis and Lung Transplant are considerations.
-Transplantation: 50% two year survival with single lung.
acute
chronic
honeycombing
Oxygen
Prognosis: IIF

In the acute progressive form “... Syndrome” (later stages of pulmonary fibrosis in which both sides of the lungs are involved) the prognosis is ....

In the ... form patients can live long with disability.

Median survival 4-6 years after diagnosis

87% mortality
Hamman-Rich
poor
chronic
What is this?

-MC in African American females
-non-caseating granuloma
-can present with hypercalcemia and/or painful rash
sarcoidosis
Sarcoidosis:

-a disease of unknown etiology.
-a multisystem granulomatous disorder
-often affects young adults
-presents most frequently w/ bilateral lymphadenopathy, pulmonary infiltration, and skin or eye lesions
-diagnosed by the presence of characteristic clinical and radiographic data w/ histologic evidence of widespread ... epithelioid cell granulomas from biopsy material in more than one organ
-majority of cases present during the ... to the ... decade of life
noncaseating
second
fourth
Bilateral lymphadenopathy --> think of ... and ....

Must prove it’s not ... by biopsying full lymph node
sarcoid and lymphoma
lymphoma
Sarcoid is one of the major causes of hyper...
calcemia
Most patients with sarcoidosis have a characteristic appearance on chest radiography.

1. Enlargement of the ... and ... lymph nodes is the most consistent finding (80-90%).
2. Symmetric enlargement of both ... is found in 90% of those who present with adenopathy. Unilateral ... enlargement does occur but is distinctly unusual. ... lymph nodes are also enlarged.
3. Parenchymal abnormalities are present about 60% of the time with a ... pattern being most common.
4. In 10-15% of cases, the chest-x-ray is normal.
hilar and mediastinal
hila
hilar
Mediastinal
reticulonodular
Staging: Sarcoidosis

Group 1: Bilateral hilar adenopathy without evidence of ... disease
Group 2: Bilateral hilar adenopathy with ... disease
Group 3: nonfibrotic parenchymal (staring fibrosis, but no ... yet)
Group 4: Fibrosis and distortion of the lung (...)

These categories have been useful for prognosis but have a low correlation with symptoms and pulmonary function
parenchymal
parenchymal
honeycombing
honeycombing
The characteristic features on CT/HRCT (for sarcoid) are small, irregular nodules (1-5 mm) that are found along the ... bundles and in the subpleural region

High resolution CT is able to demonstrate the presence of nodules even when the chest x-ray is normal.
bronchovascular
Prognosis:

Approximately 20% of patients with sarcoidosis will progress to ... and be fatal. ... is most useful in this advanced form of sarcoidosis because of its ability to demonstrate important complications such as bullae, infection, bronchiectasis, vascular obstruction, and mycetoma (fungus) formation.

~50% won’t have any progression above stage 1
pulmonary fibrosis
CT
Sarcoidosis:

LABS:
-... CD4/CD8 ratio
-... antibiotics to different virus
- (+) R.F
- (+) ANA
- ***very often ...***
- ***... ACE levels (80% will be ...)

TREATMENT:
-1. Steroids (long term)
-2. Tx ... w/ diuretics, making sure to fill them w/ fluid beforehand
increased
increased
hypercalcemia
increased
high
hypercalcemia
look at slide 19-20
ok
What am I?

-rare in adults but more common in children.
-cause unknown
-up to ¾ of patients have a serum protein abnormality, most commonly a polyclonal gammopathy, and especially in children, hypogammaglobulinemia
-Sjögren’s syndrome is associated with 1/4 of reported cases
Lymphocytic interstitial pneumonitis (LIP)
Signs and symptoms of LIP:

... and ... - slowly progressive over months or, in some cases, years - are the most common presenting symptoms.

Others include weight loss, fever, arthralgias, and pleuritic chest pain.

Chest examination may reveal crackles
Cough and Dyspnea
LIP:

The diagnosis is established by demonstration of an interstitial infiltrate (of ... and plasma cells), the information of germinal centers, and multinucleated ... cells with noncaseating granulomas by biopsy.
lymphocytes
giant
Treatment: LIP

... therapy alone or in combination with other drugs has been used to treat symptomatic patients with LIP, although its efficacy has not been established.
Corticosteroid
What do you think of when you hear these?

-Loeffler pneumonia
-Acute Eosinophilic pneumonia
-Chronic eosinophilic pneumonia
-Asthma
-Allergic Bronchopulmonary Aspergillosis
-Churg-Strauss Syndrome
-Parasitic (strongyloids, E. histolytic)
-Drugs (ASA, sulfa, MTX, phenytoin, etc)
-Cancer (by chemotactic)
Eosinophilic pumonary diseases (PIE syndrome)
PIE syndrome:

... pneumonia
-a.k.a.: simple pulmonary eosinophilia
-***... peripheral infiltrates***
-very little respiratory s/s
-check for ... in all these lung diseases - strongyloides, ascaris, toxocara
Loeffler
migratory
parasite
Acute Eosinophilic pneumonia
-uncommon, but very malignant
-fever
-Tx with ...
-may end up on vent
-bilateral effusion often
steroids
Chronic Eosinophilic pneumonia
-50% have ...
-> in ... 2:1
-median age is ...
-fever
-resp. distress
-high blood eosinophilia > 40%
-... ESR & IgE
-... image (on CXR) of CHF
*e.g. peripheral infiltrates
asthma
females
50
­increased
negative
If we have a patient w/ chronic asthma that does not respond to treatment, think of ...
-treat w/ high dose steroids for weeks.
allergic bronchopulmonary aspergillosis
What am I?

-Young female of child bearing age presents w/ pneumothorax.
-milky substance comes out once a chest tube was put in
Lymphangioleiomyomatosis (LAM)
LAM:

-Females
-chylous pleural effusions
-interstitial infiltrate at ... (d/t smooth muscle proliferation)
-post-obstructive emphysema
-+/- spontaneous pneumothorax (DDX: ... granuloma)
-similar appearance of tuberous sclerosis

Tx: ... therapy, oophorectomy
bases
eosinophilic
hormonal
look at slide 29-30
ok
What am I?

-affects men and women equally, usually beginning in their 40s or 50s.
-in ¾ of pts, symptoms last <2 mo; few have symptoms for >6 mo before dx.
-a flu-like illness, characterized by cough, fever, malaise, fatigue and weight loss (heralds the onset of the disease in 2/5 of pts)
-pts will have a viral syndrome from 2 wks to 2 months prior. After they recover from the viral syndrome, they will have progressive SOB.
-CXR will show a nodular type pattern
Idiopathic Bronchiolitis Obliterans w/ Organizing pneumonia (BOOP)
aka
Cryptogenic organizing pneumonitis
Labs: BOOP

-... (increase in WBC) without an increase in ... occurs in about 1/2 of patients.
-The initial ESR is frequently ....
-Pulmonary function tests usually show a ... defect.
-Chest x-ray show ... alveolar opacities with normal lung volumes.
-A ... distribution of the opacities, similar to that thought to be virtually pathognomonic for chronic eosinophilic pneumonia, may also be seen.
-Recurrent and ... pulmonary opacities are common.
-HRCT scans of the lung show patchy airspace consolidation, ground-glass opacities, small nodular opacities, and bronchial wall thickening and dilation.
Leukocytosis
eosinophils
elevated
restrictive
bilateral
peripheral
migratory
If we see Langerhan’s granules, automatically think of ...
primary pulmonary histiocytosis X
Primary pulmonary histiocytosis X (also known as pulmonary eosinophilic granuloma) is a disease of unknown cause that is characterized by patchy infiltration of the lung with ... and eosinophilic granulomas.

The ... in this disease are a key diagnostic feature.

They contain ... granules (X bodies).
histiocytes
histiocytes
Langerhans’
Clinical presentation: Primary pulmonary histiocytosis X

-commonly presents in the third or fourth decade.
-Most patients are ... (90%)
-Cough (50%)
-Exertional dyspnea (35%)
-Pneumothorax (10-20%)
-Between 25 and 30% are asymptomatic and are diagnosed because of abnormalities found in chest x-rays.
cigarette smokers
Imaging: Primary pulmonary histiocytosis X

Most Common pattern is one of irregular nodules that predominate in the ... and ... lung zones.

Cysts are rarely identified.

Can progress to severe honeycombing and bulla formation
mid and upper
What are the treatments for Primary pulmonary histiocytosis X?
steroids
spontaneous resolution
look at slide 37-39
ok
...:

-Substance closely related in its chemical and physiologic properties to surfactant.
-Believed to be normal degradation product accumulating as a result of impaired clearance.
-No signs of inflammation
-Usually normal interstitium

Protein collection in the alveolar space areas. Tx is removal of protein. Remove protein by literally beating it out of them. Put pt on anesthesia and do lavages. Beat on pts back, suck it all out, and beat em again. Beat them until lavage clears.
Protein will be so thick in the first lavage that it will look like muddy water.
Pulmonary Alveolar Phospholipoproteinosis
Clinical features: Pulmonary alveolar phospholipoproteinosis

-... onset of shortness of breath with non-production cough.
-Cyanosis and rales in lungs can be recognized.
-Chest x-ray shows diffuse bilateral airspace disease. (“...” alveolar infiltrated)
-PFT’s reveal restrictive defect with profound hypoxia
-Infection with ... (fungating type mass, common in sinuses) and Mycobacterium occurs with increasing frequency.
-... often required for diagnosis.
Insidious
Bat-wings
Nocardia
Lung biopsy
Therapy: Pulmonary alveolar phospholipoproteinosis

Whole lung ... under general anesthesia is the only option for therapy. Dramatic improvement in symptoms follows.

Relapses occur and repeated ... may be necessary.

Some develop interstitial fibrosis and cor-pulmonale. ... is a last resort option to patients with fibrosis.
lavage
lavage
Lung transplant
What am I?

-should be suspected whenever asthma is refractory and is associated with fever, cough and black or grey sputum.
-Chest x-ray often reveals evanescent pulmonary infiltrates.
-Marked eosinophilia and elevated IgE are noted.
Allergic bronchopulmonary aspergillosis
Aspergillus colonizes in the bronchi in asthmatics because of the thick secretions. A precipitation ... is produced. A type III immunological reaction follows. ... is activated. An inflammatory response occurs with damage to the bronchial wall and the bronchi get plugged with secretion. Segmental infiltrates and ... follows.
antibody
Complement
atelectasis
Diagnosis: Allergic bronchopulmonary aspergillosis

Aspergillus precipitating ... is detected in serum.

IgE levels and eosinophiles are ....

Central ... can be demonstrated by CT scan.

Tx:
-ABPA responds to systemic ....
-Dose and duration of steroids can be adjusted by following serum ... levels.
antibody
elevated
bronchiectasis
steroids
IgE