Diffuse Parenchymal Lung Disease

Question 1

Interstitial lung disease is often times, called __________.

Answer 1

Diffuse parenchymal lung disease (DPLD)

Question 2

Diffuse parenchymal lung disease (DPLD) all have involvement of _________ on histopathology.

Answer 2

Distal lung parenchyma

Question 3

How can we classify Diffuse Parenchymal Lung Diseases on histopathology?

Answer 3

• 1. Inflammation and fibrosis
• 2. Granulomatous changes

Question 4

What histopathologic patterns have interstitial involvement?

Answer 4

• 1. UIP (usual interstitial pneumonitis)
• 2. NSIP (Nonspecific interstitial pneumonitis)
• 3. BOOP (bronchiolitis obliterans organizing pneumonia)/ COP (cryptogenic organizing pneumonia)

Question 5

What histological pattern is seen with UIP?

Answer 5

Heterogenous involement of lung with different stages of progression of fibrosis.

Question 6

Usual interstitial pneumonitis is associated with several diseases or exposures including what?

Answer 6

1. pneumoconioses
2. radiation injury
3. end-stage hypersensitivity pneumonitis
4. advanced sarcoid

Question 7

If no underlying process is ID’d, UIP is instead diagnosed as what?

Answer 7

Idiopathic pulmonary fibrosis

Question 8

• What pattern is seen in non-specific intersitial pneumonitis (NSIP) on histo?
• CT?
• Assx with what diseases?

Answer 8

• Uniform involement of lung parenchyma, with cellular infiltartion or fibrosis.
• Ground glass infiltrates
• Autoimmune CT disorders

Question 9

What pattern is seen in BOOP/COP on histo?

CT?

Assx with what diseases?

Answer 9

• Small airway bronchiolitis with granulomas + organizing pneumonia

Question 10

_________ granulomas are typical for sarcoidosis.

________ granulomas are typical for hypersensitivity pneumonitis.

Answer 10

• well-formed non-caseating
• loosely formed granulomas

Question 11

How are DLPD’s diagnosed?

Answer 11

Thorough hx, PE, lab and imaging studies, open lung biopsy.

Question 12

What symptoms raise the possibility of DPLD?

Answer 12

1. Progressive dyspnea, over months
2. Reduced excercise tolerance
3. Persistant dry cough

Question 13

• Idiopathic pulmonary fibrosis most often occurs in patients ______.
• Interstitial lung disease assx with … occurs in patients ________
  
  • CT disease
  • Sarcoidosis
  • Lymphangioleimyomatosis
  • LCH

Answer 13

• Over 50 YO.
• 20-40 YO

Question 14

What DPLDs occurs most in females?

Answer 14

Lymphangioleimyomatosis

Question 15

What DPLDs are most associated with smoking?

Answer 15

• 1. RB-ILD
• 2. Desquamative intersitial pneumonia
• 3. Langerhan cell histiocytosis

Question 16

If patient has exposure history to birds, hay, mold or other organic material, what DPLDs should we consider?

Answer 16

Hypersensitivity pneumonitis

Question 17

If patient has occupational exposure, what DPLDs should we consider?

Answer 17

1. Asbestosis

2. Silosis

Question 18

It patient has acute onset, what DPLDs should we consider?

Answer 18

• 1. Acute Interstitial Pneumonia
• 2. Acute eosinophilic pneumonia
• 3. COP
• 4. Hypersensitivity pneumonitis
• 5. Drug-induced interstiial lung disease
• 6. Diffuse alveolar hemorrhage syndrome

Question 19

What findings are indicative of sarcoidosis or CT diseases?

Answer 19

• 1. Erythema nodosum
• 2. Uveitis/conjunctivitis
• 3. Arthritis

Question 20

What findings are indicative of sarcoidosis and Sjrogens syndrome?

Answer 20

Enlargement of lacrimal/salivary gland

Question 21

Lymphadenonapthy/hepatosplenomegaly is indicative of what DPLD?

Answer 21

Sarcoidosis

Question 22

Muscle weakness is associated with what DPLD?

Answer 22

• Polymtositis
• Dermatomyositis

Question 23

Clubbing is indicative of what DPLD?

Answer 23

Idiopathic pulmonary fibrosis.

Question 24

Hx of someone with suspected DPLD should be focused on what?

Answer 24

Looking for underlying causes

Question 25

What should docs look for on **PE** of **DPLDs**?

Answer 25

* 1. **Oxygenation status** (desaturation with exertion, which is an early sign) * 2. **Crackles** on inspiration * 3. **Clubbing** * 4. **Autoimmune diseases** * 5. **Right sided heart failure,** a consequence of DPLD.

Question 26

What is used as a diagnostic evaluation of **DPLD** to tell us about **distribution and extent** of disease.

Answer 26

**HRCT (High-resolution CT)**

Question 27

What can lead to a **diagnosis** without the need for **biopsy**?

Answer 27

**HRCT**

Question 28

What will people with **DPLD** show on **PFT**?

Answer 28

* **Decreased lung volumes** (total lung capacity, residual volume, functional residual capacity) * **Decreased diffusing capacity**

Question 29

Is a **lung biopsy** always needed to diagnosis **DPLD**? If so, how?

Answer 29

**No**, not always. Best performed via an **open procedure** using a **thorascopic approach.**

Question 30

What 2 diseases produce a **granuloma response?** ## Footnote *-All others will produce inflammation and fibrosis-*

Answer 30

**Sarcoidosis** and **hypersensitivity pneumonitis** * 1. Drug-induced parenchymal lung disease * 2. Smoking-related DPLD * 3. Connective Tissue-Associated DPLD * 4. Pneumoconiosis * 5. Idiopathic Pulmonary Fibrosis * 6. Acute

Question 31

What drugs cause **drug-induced parenchymal lung disease?**

Answer 31

* **1. Amiodorone** * **2. Methotrexate** * **3. Nitrofurantoin**

Question 32

**DPLD** is associated with which CT disorders?

Answer 32

* **1. RA** * **2. Progressive systemic sclerosis (scleroderma)** * **3. Polymyositis/dermatomyositis** * **4. Sjogen syndrome** * **5. Behcet disease**

Question 33

When **DPLDs** is associated with **asbestos**, **silicon** or **beryllium**, they are called \_\_\_\_\_\_.

Answer 33

**Pneumoconiosis**

Question 34

Imaging of **pneumoconiosis** will show what?

Answer 34

* **Basilar and subpleural bilateral, linear intersitial markings** * **Calcified pleural plaques**

Question 35

**Idiopathic pulmonary fibrosis** is important to diagnose, why?

Answer 35

**Poor prognosis**

Question 36

Histopathology of Idiopathic Pulmonary fibrosis shows what?

Answer 36

**UIP**

Question 37

**Acute intersitial pneumonia** is also called \_\_\_\_\_\_\_\_\_\_. Imaging: Diagnosis: Histopathology:

Answer 37

* **Hamman-Rich syndrome** * Bilateral alveolar disease with ground-glass change * Open lung biopsy * DAD (diffuse alveolar damage)

Question 38

When is **organizing pneumonia** termed **BOOP** or **COP**? Tx?

Answer 38

* Proximate cause is IDd =\> BOOP * No cause found =\> COP * **Systemic glucocorticoids**

Question 39

**_Hypersensitivity Pneumonitis_** * Occurs when? * HRCT shows * Histo shows: * Tx:

Answer 39

* Repeated immunologic reaction to inhalation of antigens * Ground glass opacities with centrilobular nodues * Noncaseating granulomas * Avoid causes and steroids for chronic sx.

Question 40

**_Sarcoidosis_** * Is? * Age? * PFT shows * Imaging: * Histo shows: * Tx:

Answer 40

* Formation of granulomas all over body, including lung that affects mostly blacks * Bimodal: Peaks over 18 YO and ppl 50-60 * Restriction, reduced diffusing capacity, obstruction of airways * Bilateral hilar adenopathy and/or interstitial infilteates or patchy alveolar infiltrates * Bronchocentric noncaseating granulomas * Systmic glucocorticoids

Question 41

Many patients with severe/chronic DPLD develop what?

Answer 41

**Pulmonary HTN**

Question 42

Treatments for people with **DPLD**?

Answer 42

* 1. Stop smoking * 2. Supp O2 * 3. Symptomatic tx for breathing and treatment of infections * 4. Vasodilating agents to reduce right side vascular resistance