Fibrotic Lung Disease Flashcards
What is sarcoidosis?
abnormal collections of inflammatory cells such as epithelioid cells, macrophages and T cells forming clumps in the lungs, skin and lymph nodes
In which ethnic demographic is sarcoidosis most common?
Afro-Caribbean
Is sarcoidosis more common in males or females?
Females
Give 3 constitutional clinical features of sarcoidosis:
- Fever
- Weight loss
- Fatigue
Give 3 renal clinical features of sarcoidosis:
1) granulomatous tubulointerstitial nephritis
2) hypercalcaemia
3) stone formation
Give 3 respiratory clinical features of sarcoidosis:
1) cough
2) dyspnoea
3) wheeze
Give 2 ocular clinical features of sarcoidosis:
1) anterior uveitis
2) keratoconjunctivitis sicca
What is keratoconjunctivitis sicca?
Dry eye syndrome
What is uveitis?
Inflammation of the uveal tract (iris, ciliary body, choroid)
Give 3 immunopathological features of sarcoidosis:
1) negative Mantoux test
2) overall lymphopenia
3) infiltration of the alveolar walls and interstitial spaces
Give two reticuloendothelial features of sarcoidosis:
1) splenomegaly
2) lymphadenopathy
Give two cutaneous features of sarcoidosis:
1) erythema nodosum
2) lupus pernio
What is erythema nodosum?
Tender red nodules caused by inflammation of fat cells usually found on the shins
What is lupus pernio?
raised discoloration of nose/ear/cheek/chin which resembles frostbite
Give six neurological features of sarcoidosis:
1) cognitive dysfunction
2) headache
3) cranial nerve palsies
4) mononeuritis multiplex
5) peripheral neuropathy
6) seizures
What is mononeuritis multiplex?
nerve damage in 2 or more nerves in separate parts of the body
Give 4 cardiac features of sarcoidosis:
1) arrhythmias
2) heart block
3) cardiomyopathy
4) sudden death
Describe the CXR findings of stage 1 pulmonary sarcoidosis:
bilateral hilar lymphadenopathy
Describe the CXR findings of stage 2 pulmonary sarcoidosis:
pulmonary infiltrates with bilateral hilar lymphadenopathy
Describe the CXR findings of stage 3 pulmonary sarcoidosis:
pulmonary infiltrates without bilateral hilar lymphadenopathy
Describe the CXR findings of stage 4 pulmonary sarcoidosis:
Fibrosis
True or false: the chest will always sound clear in sarcoidosis regardless of the extent of infiltration
true
What enzyme is raised in sarcoidosis?
angiotensin converting enzyme
Does interstitial/ fibrotic lung disease cause an obstructive or restrictive lung defect?
Restrictive
Give 6 investigations used to diagnose and manage sarcoidosis:
1) CXR (for staging)
2) HRCT (for assessment of parenchymal involvement)
3) U+Es for hypercalcaemia
4) serum ACE levels
5) lung function tests (will show restrictive defects)
6) bronchoscopy and transbronchial biopsy
Why is hypercalcaemia associated with sarcoidosis?
activated macrophages in the lung and lymph nodes are able to hydroxylate vitamin D directly independent of parathyroid levels leading to an increased intestinal absorption of dietary calcium
How is severe sarcoidosis managed? (2)
1) prednisolone
2) in some cases, immunosuppressants such as methotrexate, azathioprine and hydroxychloroquine
What are idiopathic interstitial pneumonias?
interstitial lung diseases with no known cause
What is the most common form of idiopathic interstitial pneumonias?
idiopathic pulmonary fibrosis
True or false: idiopathic pulmonary fibrosis is fatal
True
Give 3 HRCT findings associated with idiopathic pulmonary fibrosis:
1) progressive scarring seen as honey combing (mainly in the subpleural areas)
2) reticulation (linear opacities)
3) bronchiectasis
Describe the pathophysiology of idiopathic pulmonary fibrosis:
repetitive injury to the alveolar epithelium caused by an unknown environmental stimuli leads to the activation of repair pathways which become uncontrolled, leading the overproduction of fibroblasts and increased deposition of increased extracellular matrix in the interstitial
Give 4 clinical features associated with idiopathic pulmonary fibrosis:
1) cough
2) progressive dyspnoea
3) finger clubbing
4) pulmonary hypertension
Give 4 investigations used to diagnose and manage idiopathic pulmonary fibrosis:
1) CXR
2) HRCT
3) ANA and Rheumatoid Factor blood tests (to rule out autoimmune rheumatic disease)
4) Respiratory function test
What is the mean survival time for a patient with idiopathic pulmonary fibrosis?
2-5 years
Give 2 drugs that can be used to help manage idiopathic pulmonary fibrosis:
1) pirfenidone
2) nintedanib
Describe how pirfenidone can help manage idiopathic pulmonary fibrosis:
it acts as an antifibrotic agent that has been shown to slow the rate of forced vital capacity (FVC) decline
Describe how nintedanib can help manage idiopathic pulmonary fibrosis:
it is an intracellular inhibitor of tyrosine kinase, preventing activation of scarring repair pathways
What is desquamative interstitial pneumonia? (5)
1) Presents with insidious onset of dyspnoea and dry cough over weeks/months (+/- clubbing of digits)
2) Smoking is VERY common
3) Often in 4-5th decade of life
4) Excellent response to Steroids and Smoking Cessation
5) It is caused by the accumulation of pigmented macrophages within alveolar spaces
What is respiratory bronchiolitis interstitial lung disease?
where the lumen of respiratory bronchioles are filled with pigmented macrophages, appearing as centrilobular nodules on imaging
What is acute interstitial pneumonia?
sudden dyspnoea and progressive respiratory failure due to diffuse alveolar damage with no known cause
What is cryptogenic organising pneumonia?
Buds of connective tissue known as Masson bodies form in alveoli and alveolar ducts causing influenza-like symptoms with cough and dyspnoea
What is lymphoid interstitial pneumonia?
where lung interstitial is infiltrated by lymphocytes, macrophages and plasma cells often seen in HIV, presenting with insidious dyspnoea, dry cough and systemic upset
What is hypersensitivity pneumonitis?
inflammation of the lung parenchyma caused by antigen exposure
Give 7 types of hypersensitivity pneumonitis:
1) Famer’s lung
2) Bird fancier’s lung
3) Malt worker’s lung
4) Humidifier fever
5) Mushroom worker’s lung
6) Cheese worker’s lung
7) Winemaker’s lung
What allergen causes Farmer’s lung?
inhaling thermophilic actinomycetes (a group of bacteria found on decaying vegetations e.g. mouldy hay)
What allergen causes Bird fancier’s lung?
proteins on feathers and faeces of birds
What allergen causes Malt worker’s lung?
causes Malt worker’s lung?
inhaling Aspergillus clavatus antigens found on germinating bale
What allergen causes Humidifier fever?
inhaling a variety of bacterium or amoeba from contaminated humidifying systems
What allergen causes Mushroom worker’s lung?
inhaling thermophilic actinomycetes found on mouldy mushroom compost
What allergen causes Cheese washer’s lung?
inhaling Penicillium casei and Aspergillus clavatus (associated with mouldy cheese)
What allergen causes Wine maker’s lung?
inhaling Botrytis, a type of necrotrophic fungus associated with mould on grapes
How does allergy lead to hypersensitivity pneumonitis?
cellular immunity and deposition of immune complexes results in chronic inflammatory infiltrates, interstitial granulomas and interstitial fibrosis
Give 6 clinical features of hypersensitivity pneumonitis:
1) fever
2) weight loss
3) malaise
4) clubbing
5) cough
6) dyspnoea
Give two auscultation findings associated with hypersensitivity pneumonitis:
1) inspiratory squeaks (due to bronchiolitis)
2) bilateral fine crackles
Give 3 investigations used to diagnose and manage hypersensitivity pneumonitis:
1) CXR (diffuse nodules and increased reticular shadowing)
2) HRCT (shows nodules and honey comb changes)
3) antibody test (to test for particular allergens
How is hypersensitivity pneumonitis treated? (2)
1) avoidance of inciting allergen
2) prednisolone
Name 6 rare interstitial lung disease:
1) Langerhans cell histiocytosis
2) Pulmonary lymphangioleiomyomatosis (smooth muscle infiltration)
3) Pulmonary alveolar proteinosis (lipoproteins accumulation in alveoli)
4) small-vessel vasculitides
5) Anti-glomerular basement membrane disease
6) diffuse alveolar haemorrhage
Give 3 examples of small-vessel vasculitides:
1) Granulomatosis with polyangiitis
2) Microscopic polyangiitis
3) Eosinophilic granulomatosis with polyangiitis
What is the triad of anti-glomerular basement membrane disease?
1) pulmonary haemorrhage
2) glomerulonephritis
3) antibodies against the basement membrane
Give 4 pulmonary manifestations of rheumatoid disease/ arthritis:
1) rheumatoid nodules
2) obliterative bronchiolitis (narrowing bronchioles until they close)
3) cricoarytenoidal arthritis (inflammation of larynx joint)
4) Caplan’s syndrome
Give 3 clinical presentations associated with cricoarytenoidal arthritis:
1) dyspnoea
2) stridor
3) hoarseness
What is Caplan’s syndrome?
Rheumatoid arthritis with silicosis
What is the most common allergen associated with pulmonary infiltration with eosinophilia?
Aspergillus fumigatus
Give 5 manifestations of Aspergillus fumigatus in the lung:
1) fibrosis
2) mucous plugs
3) lung collapse
4) aspergillomas (fungus ball often formed in old TB capsule)
5) invasive aspergillosis (lung is overwhelmed with fungus)
What is the name given to the condition where irradiation of the lung during radiotherapy?
radiation pneumonitis
