EXAM #2: NON-INFECTIOUS INFLAMMATORY LUNG DISEASE Flashcards

1
Q

What is Sarcoidosis?

A
  • Mutlisystem granulomatous inflammatory disease

- Unknown etiology

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2
Q

What patient population is commonly affected by Sarcoidosis?

A

Aoki= young-middle aged adults (3rd decade)

Pathoma= African American females

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3
Q

How does the presentation of Sarcoidosis in African Americans differ from other patients?

A

African Americans= more severe and more widely distributed

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4
Q

What is the most common presenting symptom of Sarcoidosis?

A

Dyspnea with nonproductive cough

*Less common sx. include: constitutional sxx, lymphadenopathy, skin changes, dysrhythmia

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5
Q

What organs/ organ systems are commonly involved outside of the lungs?

A

1) Lymphatic system
2) Uvea= uveitis
3) Skin= cutaneous nodules, lupus perino*, or erythema nodosum
4) Salivary or lacrimal glands (Resembles Sjogren’s Syndrome)

*violet rash involving the nose and cheeks.

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6
Q

If a patient presents with a history and physical suspicious for Sarcoidosis, what should be included in the diagnostic work-up?

A

1) PFT
2) Labs
- Ca++
- ACE
- Liver enzymes
- Creatinine
3) ECG
4) Ophthalmalogic exam (uveitis)
5) TB skin test (r/o TB)

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7
Q

What type of presentation is associated with Sarcoidosis on PFT? Draw the flow-volume loop that you would expect to see compared to the normal.

A

Restrictive

  • Decreased TLC
  • Decreased VC
  • Decreased RV

*Note that this patient CAN present with obstructive disease depending on the location of the granulomas

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8
Q

Outline the radiographic staging of Sarcoidosis.

A
0= Normal
I= Hilar adenopathy 
II= Hilar adenopathy + abnormal lung parenchyma
III= No adenopathy BUT abnormal lung parenchyma 
IV= Fibrotic changes/ structural distorsion
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9
Q

Describe the appearance of Sarcoidosis on CT.

A

Granulomatous nodules

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10
Q

If history, exam, CXR, and/or CT point to Sarcoidosis, how do you confirm diagnosis?

A

Brochoscopy

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11
Q

What will happen with most patients affected by Sarcoidosis?

A

Spontaneous remission NOT requiring treatment

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12
Q

What are the indications for systemic steroids in Sarcoidosis?

A

1) Cardiac involvement
2) Ocular disease
3) Neurologic disease
4) Hypercalcemia
5) Lupus pernio
6) Symptomatic stage II
7) Stage III

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13
Q

List the most common connective tissue disorders that involve the lung.

A

1) RA
2) Scleroderma
3) SLE
4) Sjogren Syndrome
5) Dermatomyositis and polymyositis

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14
Q

What is the common age of patients with Rheumatic Lung Disease? Is there a gender predominance?

A
  • 50-60 y/o

- Yes, males

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15
Q

What are the signs/sx. of Rheumatic Lung Disease (RLD)?

A

1) Non-productive cough
2) Dyspnea on exertion
3) Clubbing
4) Inspiratory fine crackles
5) Pleural rub

Note that Rheumatic Lung Disease may PRECEED joint disease

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16
Q

How does RLD appear on imaging?

A

1) Nodules that can CAVITATE
2) Interstitial disease

*Note that TB and other infections can cavitate and must be ruled out first before starting on immunosuppressive therapy

17
Q

What are the treatments for RLD?

A

1) Methotrexate
2) Etanercept
3) Infliximab

18
Q

Pleural effusion associated with RLD has characteristic lab finding?

A

Low glucose

19
Q

What is Scleroderma? What are the two presentations of Scleroderma?

A
  • Generally, an autoimmune disorder characterized by sclerosis of the skin and visceral organs. There is:
    1) Diffuse Scleroderma
    2) CREST Syndrome
C= calcinosis 
R= raynaud 
E= esophageal dysmotility 
S= sclerodactyly
T= telangiectasias
20
Q

What is CREST Syndrome associated with?

A

Pulmonary HTN*

*This is an indication for vasodilators

21
Q

What is the presentation of diffuse Scleroderma?

A
  • Inspiratory crackles

- Restrictive lung physiology

22
Q

What is the most common presentation of SLE involving the lung?

A

Serositis i.e. inflammation of the serous tissues of the body, such as the pleura

23
Q

Aside from serositis, what else is associated with SLE involving the lung?

A

1) Pneumonitis
2) Alveolar hemorrhage
3) Diaphragmatic dysfunction
4) Increased risk for DVT/PE*

*Emphasized in lecture

24
Q

How does Sjogren’s Syndrome present when it involves the lung?

A
  • Bronchiectasis
  • Bronchiolitis

*Especially in the context of other Sjogern’s Symptoms i.e. dry eyes, dry mouth

25
Q

On imaging, how does bronchiectasis appear?

A

Airway diameter is large than blood vessel diameter

26
Q

What is polymyositis? What is dermatomyositis?

A

Polymyositis= uncommon inflammatory disease that causes muscle weakness affecting both sides of your body

Dermatomyositis= disease related to polymyositis that affects the muscles and skin

27
Q

How does polymyositis/ dermatomyositis present with lung involvement?

A

1) Aspiration pneumonia from pharyngeal muscle weakness
2) Diaphragm elevation and decreased lung volumes
3) Basilar atelectasis

28
Q

What are the different froms of pulmonary vasculitis?

A

1) Wegener’s Granulomatosis
2) Churg Strauss Syndrome
3) Goodpasture’s Syndrome
4) Diffuse Alveolar Hemorrhage

29
Q

What is Wegener’s Granulomatosis?

A

Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys

30
Q

What is the classic presentation of Wegener’s Granulomatosis?

A

Middle aged male w/

  • Sinusitis or nasopharyngeal ulceration
  • Hemoptysis
  • Bilateral nodular lung infiltrates
  • Hematuria
31
Q

What serum antibody is associated with disease activity in Wegener’s Granulomatosis? What is the utility of this antibody?

A

c-ANCA*

*Can be used to differentiate from TB–look v. similar on CXR

32
Q

What will biopsy of Wegener’s Granulomatosis reveal?

A

Large necrotizing granulomas with adjacent necrotizing vasculitis

33
Q

What is Goodpasture Syndrome?

A
  • Rare autoimmune disease
  • antibodies attack the basement membrane in lungs and kidneys
  • Causes bleeding from the lungs and kidney failure
34
Q

How can patient’s with Goodpasture Syndrome present?

A

Alveolar hemorrhage and renal failure

35
Q

How can you diagnose alveolar hemorrhage?

A

Bronchoscopy/ alveolar lavage

36
Q

What is Churg-Strauss Syndrome?

A

Necrotizing granulomatous inflammation with eosinophils involving the heart and lungs

37
Q

What is the triad of Churg-Strauss Syndrome?

A

1) Asthma
2) Hypereosinophilia
3) Necrotizing vasculitis

38
Q

What antibody correlates with disease progression in Churg-Strauss Syndrome?

A

p-ANCA