The Pulmonary System: Diffuse Interstitial Disease

Question 1

Diffuse Interstitial Diseases:

Answer 1

a group of chronic disorders characterized by inflammation and fibrosis of the pulmonary interstitial tissue (wall of alveoli)

Question 2

Diffuse interstitial diseases are collectively known as _______, _____, or ______-

Answer 2

restrictive lung disease, pulmonary fibrosis, or interstitial fibrosis

Question 3

Idiopathic pulmonary fibrosis

Answer 3

syndrome characterized by a chronic process of pulmonary fibrosis or scarring due to unknown cause

Question 4

idiopathic pulmonary fibrosis can be cause by recurrent______ and ____ in susceptible persons. It will result in _______ lung compliance.

Answer 4

inflammation and repair; decrease

Question 5

Is diffuse interstitial disease hereditary?

Answer 5

yes

Question 6

On spirometry, the hallmark of idiopathic pulmonary fibrosis is decreased ________

Answer 6

lung volume (Decreased FVC - aka restrictive lung disease

Question 7

idiopathic pulmonary fibrosis is associated with _______ and _____–

Answer 7

pulmonary hypertension, and high heart failure

Question 8

Idiopathic Pulmonary fibrosis starts _____, with gradually increasing dyspnea on exertion and dry cough, with hypoxemia, cyanosis, and clubbing occurring

Answer 8

insidiously

Question 9

Symptoms of Idiopathic Pulmonary Fibrosis

Answer 9

shortness of breath, chronic dry, hacking coughing, fatigue and weakness, chest discomfort, loss of appetite and rapid weight loss

Question 10

CRX features of idiopathic pulmonary fibrosis

Answer 10

reduced lung volume, reticular opacities, lower lobe and peripheral field predominance, honeycombing may be visible

Question 11

Connective Tissue Diseases (autoimmune)

Answer 11

• associated with pulmonary hypertension, right heart failure; can involve the lung in pulmonary interstitial fibrosis; similar presentation idiopathic pulmonary fibrosis, but the cause is known (autoimmune disease)

Question 12

associated causes of Connective Tissue diseases

Answer 12

(autoimmune disorders) such as rheumatoid arthritis, systemic sclerosis, and lupus erythematosus

Question 13

Pneumoconiosis :

Answer 13

lung fibrosis in response to inhaled particles and aerosols

Question 14

In Pneumoconiosis- macrophages ingest particles, die and release lysosomal contents, cytokines, causing fibrosis without ______ the inflammatory stimulus

Answer 14

eliminating ( particles stay behind and are re-ingested)

Question 15

Particles that are ___ to ___ micrometers (extremely fine) are most likely to reach terminal alveoli and cause this disorder.

What are some causes?

Answer 15

1 to 5

mineral dust, organic dust, chemical fumes

Question 16

Drug- induced diffuse interstitial disease

Answer 16

certain drugs may be associated with acute and chronic alterations in respiratory structure and function: similar prices to idiopathic pulmonary fibrosis

Question 17

Granulomatous Diseases: a granulotoma is a ___

Answer 17

• a firm nodule resulting from chronic inflammation, often with a fibrous capsule
• contain macrophages, lymphocytes, plasma cells, and/or multinucleated giant cells

Question 18

Granulomatous disease may contain _____ organisms and may have central ____

Answer 18

infectious, necrosis

Question 19

Sarcoidosis (type of Granulomatous disease) is _____-

Answer 19

an immunologically-mediated stems disease of unknown cause characterized by non-caseating granulomas in many tissues and organs
- genetic predisposition

Question 20

CRX representation of Sarcoidosis

Answer 20

bilateral hilar lymph node enlargement; may have large granulomas that cause interstitial fibrosis or edema

Question 21

Hypersensitivity Pneumonitis

Answer 21

immunologically mediated inflammatory lung disorders caused by intense, usually prolonged exposure to inhaled animal, microbial, and some chemical antigens

Question 22

Hypersensitivity Pneumonitis causes diffuse ______. Requires abnormal sensitivity or reactivity to antigen. ( only _____ are affected)

Answer 22

inflammation of interstitial lung, terminal bronchiole, and alveoli, can result in fibrosis
- only susceptible individuals are affected

Question 23

Pneumonitis is primarily type ___ and ___ hypersensitivity reactions while asthma is type ____

Answer 23

3 (immune complex) and 4 (delayed hypersensitivity); asthma is type 1

Question 24

Common sensitizing agents for Hypersensitivity Pneumonitis are:

Answer 24

organic dust with bacterial spores, fungi, animal proteins, bacterial products 
Ex:
-Farmers lung
-Bird Breeders lung
-Humidifer or air conditioner lung

Question 25

Pulmonary Eosinophilia:

Answer 25

several pulmonary disorders characterized by an infiltration of eosinophils into alveoli, resulting in intraseptal inflammation, and fibrosis
- frequently idiopathic