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