The Pulmonary System: Diffuse Interstitial Disease Flashcards
Diffuse Interstitial Diseases:
a group of chronic disorders characterized by inflammation and fibrosis of the pulmonary interstitial tissue (wall of alveoli)
Diffuse interstitial diseases are collectively known as _______, _____, or ______-
restrictive lung disease, pulmonary fibrosis, or interstitial fibrosis
Idiopathic pulmonary fibrosis
syndrome characterized by a chronic process of pulmonary fibrosis or scarring due to unknown cause
idiopathic pulmonary fibrosis can be cause by recurrent______ and ____ in susceptible persons. It will result in _______ lung compliance.
inflammation and repair; decrease
Is diffuse interstitial disease hereditary?
yes
On spirometry, the hallmark of idiopathic pulmonary fibrosis is decreased ________
lung volume (Decreased FVC - aka restrictive lung disease
idiopathic pulmonary fibrosis is associated with _______ and _____–
pulmonary hypertension, and high heart failure
Idiopathic Pulmonary fibrosis starts _____, with gradually increasing dyspnea on exertion and dry cough, with hypoxemia, cyanosis, and clubbing occurring
insidiously
Symptoms of Idiopathic Pulmonary Fibrosis
shortness of breath, chronic dry, hacking coughing, fatigue and weakness, chest discomfort, loss of appetite and rapid weight loss
CRX features of idiopathic pulmonary fibrosis
reduced lung volume, reticular opacities, lower lobe and peripheral field predominance, honeycombing may be visible
Connective Tissue Diseases (autoimmune)
- 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)
associated causes of Connective Tissue diseases
(autoimmune disorders) such as rheumatoid arthritis, systemic sclerosis, and lupus erythematosus
Pneumoconiosis :
lung fibrosis in response to inhaled particles and aerosols
In Pneumoconiosis- macrophages ingest particles, die and release lysosomal contents, cytokines, causing fibrosis without ______ the inflammatory stimulus
eliminating ( particles stay behind and are re-ingested)
Particles that are ___ to ___ micrometers (extremely fine) are most likely to reach terminal alveoli and cause this disorder.
What are some causes?
1 to 5
mineral dust, organic dust, chemical fumes
Drug- induced diffuse interstitial disease
certain drugs may be associated with acute and chronic alterations in respiratory structure and function: similar prices to idiopathic pulmonary fibrosis
Granulomatous Diseases: a granulotoma is a ___
- a firm nodule resulting from chronic inflammation, often with a fibrous capsule
- contain macrophages, lymphocytes, plasma cells, and/or multinucleated giant cells
Granulomatous disease may contain _____ organisms and may have central ____
infectious, necrosis
Sarcoidosis (type of Granulomatous disease) is _____-
an immunologically-mediated stems disease of unknown cause characterized by non-caseating granulomas in many tissues and organs
- genetic predisposition
CRX representation of Sarcoidosis
bilateral hilar lymph node enlargement; may have large granulomas that cause interstitial fibrosis or edema
Hypersensitivity Pneumonitis
immunologically mediated inflammatory lung disorders caused by intense, usually prolonged exposure to inhaled animal, microbial, and some chemical antigens
Hypersensitivity Pneumonitis causes diffuse ______. Requires abnormal sensitivity or reactivity to antigen. ( only _____ are affected)
inflammation of interstitial lung, terminal bronchiole, and alveoli, can result in fibrosis
- only susceptible individuals are affected
Pneumonitis is primarily type ___ and ___ hypersensitivity reactions while asthma is type ____
3 (immune complex) and 4 (delayed hypersensitivity); asthma is type 1
Common sensitizing agents for Hypersensitivity Pneumonitis are:
organic dust with bacterial spores, fungi, animal proteins, bacterial products Ex: -Farmers lung -Bird Breeders lung -Humidifer or air conditioner lung
Pulmonary Eosinophilia:
several pulmonary disorders characterized by an infiltration of eosinophils into alveoli, resulting in intraseptal inflammation, and fibrosis
- frequently idiopathic