ILD Flashcards
Do people with ILD have an obstructed or restricted flow?
restricted
What are the three broad types of restricitve lung disease?
-extrapulmonary
-neuromuscular
-lung parenchyma
Name some causes of extrapulmonary lung conditions
-obesity
-scoliosis
-ascites
What is acities?
extra fluid in the abdomen
Name some causes of neuromuscular lung conditions
-myasthenia gravis
-motot neurone disease
-diaphragmatic paralysis
Name some causes of lung parenchyma lung conditions
-ILD
Name some ILD’s ( non idiopathic)
-drug induced lung disease
-sarcoidosis
-connective tissue disorders
-occupational lung diseases
-hypersensitivity pneumonitis
-most common type is IPF
What happens to the lungs with ILD’s?
-fibrosis (scarring) of the pulmonary interstitium and the bronchovascular and septal tissue
-may be inflammation
-alveolar airspaces and distal airways may be affected
-cause is often idiopathic
What are the four main catergories of ILD?
1-idiopathic interstitial pneumonitis ( IIP)
2-ILD due to specific causes
3-granulomatous ILD
4-rare causes
Describe the pathologic findings in IPF
-patchy, dense fibrosis +/- honeycombing
-very little inflammation
-areas of interspersed normal lung
-mixed picture of normal lung and affected areas
Describe the radiographic findings in IPF
-peripheral, lace-like areas of fine reticular opacity
-concentrated posteriorly in the bases of the lungs
-ground glass opacification (cloudy) of the lower lobe
-honeycoming and/or traction bronchiectasis
What is the treatment for IPF?
-no response to steroids or othr immunosuppressive drugs
-smoking cessation does not change course of disease
-course of disease is marked by periods of rapid clinical deterioration
What is the prognosis for IPF?
-5 year survival rate 10-50%
-median survival after diagnosis 2.5-3.5 years
-higher prevalence and mortality rates in men, who typically present age 50-70
What is the diagnosis for IPF?
-if clinical and radiographic findings are consistent and suggest IPF, a diagnosis can be made without a biopsy
-pathologic evidence is only necessary in cases where the diagnosis is uncertain
-all other forms of IIP require pathologic confirmation for diagnosis
Whats the difference between IPF v Non-usual interstitial pneumonitis ( Non-UIP)
-IPF has poor prognosis
-little effective therapy for IPF
-patients with Non- Idiopathic interstitial pneumonitis may respond to therapy
-smoking cessation may help may help Non- UIP nut NOT IPF
What is a video- assisted thoracoscopy ( VATS) ?
-keyhole surgery used to obtain lung biopsy samples
-performed under General anaesthetic
-small inscisions in the chest made close to the ribs. A thoracoscope os omseted to view the lungs
Why are VATS used?
-taking biopsies from the lung or the lining of the chest, removing lymph nodes
-removing lumps of growths from the lungs
-to treat various lung condtions such as pleural effusion
What drugs are known to cause lung damage?
-cancer chemotherapy ( bleomycin)
-drugs for heart conditions ( amiodarone)
-anti-inflam drugs ( methotrexates )
-antibiotice ( nitrofurantoin)
-paraquat
-therapeutic radiation
-recreational drugs
What is amiodarone used for?
treating cardiac arrythmias
Describe drug induced diffuse parenchymal lung disease
-patients should have RFTs prior to treatment with drugs known to damage the lungs
-if lung damage is suspected then the drug should be stopped asap
-steroids may help reverse the damage, but the damage may be irreversible
What causes hypersensitivity pneumonitis?
-also knwon as extrinsic allergic alveolitis
-caused by immune mediated inflammatory response to inhaled antigens such as mould, dust or chemicals to which the patient becomes sensitized
Name some diseases that are a result of hypersensitivity pneumonitis
-bird fanciers lung- bird droppings or antigens on bird feathers
-farmers lung - mould in hay
What is connective tissue disease/ collagen vascular diseases?
-range of autoimmune conditions attacking collagen in connective tissue
diseases associated with ILD:
-rheumatoid arthiritis, sjogrens syndrome, scleroderma
What is rheumatoid arthritis?
-autoimmune condition affecting joints causing inflam and pain
-inflam process in RA can involve other sites such as the lungs, heart, blood vessels and eyes
-lungs are common site after joints
-RA treatment may cuase drug-induced ILD
What is Sjogren’s syndrome?
-chronic autoimmune condition
-destroys glands that produce saliva and tears
-generalised dryness
What is scleroderma/ systemic sclerosis?
-autoimmune condition causing skin hardening
-in severe conditions can affect internal organs such as the lungs
-causes ILD and also affects blood vessels leading to pulmonary hypertenstion
What is occupational lung disease?
-inhalation of mineral dusts such as asbestos, silica or coal, causing direct cytotoxic effect on lung parenchyma
Describe granulomatous ILD
-granuloma is a clump of tissue macrophages ( histocytes)
-granulomas form as an inflam response to an immune reaction
-sarcoidosis is the most common type
Describe sarcoidosis
-multisystem inflam disorder of unknown cause
-deposition of granulomas in the lungs
-Hilar lymphadenopathy- lymph nodes in the centre of the chest become inflammed
-mild form-few systems
-may cause restricive and/or obstructive defect
What are some rare causes of ILD?
-infiltrative diseases- amyloidosis
-post inflam disease- ARDS
-post infective disease- HIV
-post bone marrow transplant
-rare lung diseases- Langerhan’s cell histiocytosis
List some typical presentations of IPF
-breathlessness
-persistant/ non-productive cough
-bilateral inspiratory crackles
-decreased excersise tolerance with desaturation on excersise
-fatigue
-weight loss
-fever
-joint pain
-clubbing
How do we diagnose ILD?
-full patient history and physical examination
-respiratory function test
-x-ray or CT scan
-VATS biopsy
-blood tests such as - rheumatoid factor,check for immune disease such as antibodies to avian precipitans, check for infection
Whats the effect of ILD on lung function?
-lungs have decreased compliance due to inflam infiltrates or increased fibrous tissue
-thickening of alveoli makes it more difficult for gas exchange to occur
-patient’s lung volume is reduced as unable to expand the lungs
-increased work to breathing
-patients will take smaller and more rapid breaths than healthy subjects
What are some treatment options of ILD?
-pulmomary rehabilitation
-long term oxygen therapy
-lung transplant- 50% survival at 5 years
-pallative care
-drug treatment
Name some drugs used to treat ILD
-antitussive medication
-pain relief
-steroids
-N-acetylcysteine
-antifibrotic agents
-immunosuppressive agents- azothiprine and methotrexate
What is N-acetylcysteine?
-a precursor to glutathione which is an antioxidant
-mucolytic
-thought to restore oxidant-antioxidant balance
-given in combination with steroids and azathioprine
-antifibrotic properties
When are immunosuppresive agents considered?
-patients who do not respond to steroids or have contraindications to high-dose corticosteroid therapy
-Azathioprine is less toxic than methotrexate
-can cause ILD
Describe antifibrotic agents
-pirfenidone and nintedanib
-suitable for patients with mold to moderat disease, FVC berween 50 and 80% predicted, now also patients with FVC> 80%
side effects- tiredness,weight loss, sensitivity to sunlight, dizziness, nauseas and vomiting
-specialist centres
