Types of Interstitial Lung Diseases Flashcards
(34 cards)
What is the primary pulmonary manifestation associated with CTDs?
Interstitial Lung Disease (ILD)
ILD can also include pleural diseases, airway complications, pulmonary vascular disease, opportunistic infections, and drug toxicity.
Define Interstitial Lung Disease (ILD).
Group of respiratory diseases that cause progressive fibrosis (scarring) of the interstitium and space around alveoli
ILD can be classified by its underlying cause.
How many main types of ILD classifications are there?
3 main types
The types include exposure-related ILDs, systemic disease ILDs, and drug-induced ILDs.
What are the three main types of ILD classifications?
- Exposure-related ILDs
- Systemic disease ILDs
- Drug-induced ILDs
What are exposure-related ILDs caused by?
Occupational exposure, environmental exposure, intentional exposure, iatrogenic exposures
These exposures lead to various forms of lung disease.
What is the most common cause of systemic disease ILDs?
Connective diseases (CDs), sarcoidosis, and vasculitis
Connective diseases often lead to ILD.
What characterizes ILD associated with rheumatoid arthritis (ILD-RA)?
- More common in males than females
- Presents as idiopathic pulmonary fibrosis or nonspecific interstitial pneumonia
- High titres of rheumatoid factor increase risk
- No relationship between joint involvement and ILD progression
- RA treatments can cause pulmonary toxicity
What is the most common form of ILD in Sjogren’s syndrome?
Nonspecific interstitial pneumonia (NSIP)
It can also present as idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), or lymphocytic interstitial pneumonia (LIP).
What are typical respiratory symptoms in ILD-Sjogren’s syndrome?
Cough and dyspnea
These symptoms are commonly associated with the disease.
What are the clinical features of ILD in systemic sclerosis?
- More common in diffuse cutaneous systemic sclerosis than limited cutaneous systemic sclerosis
- Dyspnea and cough that progress slowly
- Most commonly presents as nonspecific interstitial pneumonia
True or False: There is a relationship between skin involvement in systemic sclerosis and the progression of ILD.
False
No relationship exists between skin involvement and ILD progression.
What can repeated flare ups of acute lupus pneumonitis cause in ILD-SLE?
Interstitial fibrosis
This indicates the chronic impact of lupus pneumonitis on lung health.
What percentage of cases in ILD-Dermatomyositis see ILD precede skin or muscle involvement?
33%
This highlights the varied presentation of ILD in dermatomyositis.
What are common presentations of ILD-Dermatomyositis?
- Acute interstitial pneumonia (AIP)
- Idiopathic pulmonary fibrosis (IPF)
- Cryptogenic organizing pneumonia (COP)
- Abnormal radiological lung findings without respiratory symptoms
What are the main respiratory symptoms of ILD?
Dyspnea
Dyspnea can also be influenced by cardiac involvement in conditions like dermatomyositis.
What systemic drug can cause interstitial lung disease (ILD)?
Methotrexate: Pulmonary toxicity side effect can present as methotrexate interstitial pneumonitis.
Typically subacute onset of cough, dyspnea and fever. Incidence increases as MTX dose increases.
What is another systemic drug that can cause pneumonitis?
Leflunomide: Leflunomide alone or in conjunction with methotrexate can cause pneumonitis.
What is the risk associated with anti-TNF biologics?
Anti-TNF biologics: Increase risk of ILD and can cause fatal exacerbations in patients with pre-existing ILD.
What is Idiopathic Pulmonary Fibrosis (IPF)?
Chronic, patchy fibrosing interstitial pneumonitis of unknown cause that is limited to lungs.
Typical age onset over 50 yrs old and is more common in males than females.
What are the clinical features of IPF?
Slowly-worsening dyspnea that is worse on exertion and non-productive cough. Typically no extrapulmonary involvement.
What are the radiological findings for IPF?
Lower lung zone predominant, subpleural reticular opacities with areas of honeycombing.
What is Nonspecific Interstitial Pneumonia (NSIP)?
Chronic diffuse, uniform infiltration of interstitium with varying amounts of chronic inflammation and fibrosis.
Typical age onset 40-50 yrs old, and is equally common in males and females.
What are the clinical features of NSIP?
Progressive dyspnea, cough and fatigue.
What are the radiological findings for NSIP?
Diffuse bilateral reticular and ground-glass opacities and no honeycombing.
