parenchymal lung disease Flashcards
another name for parenchymal lung disease
interstitial lung disease
what are they characterised by
- inflammation
- fibrosis
where does it affect initially
interstitium of lung
what does it present with
- exertional dyspnoea
- maybe cough
granulomatous diseases
- sarcoidosis
- hyperesensitivity pneumonitis
- infections e.g. TB
what is most common cause of ILD
idiopathic pulmonary fibrosis
what is the cause of IPF
unknown
when is IPF onset
sixties
who is more likely to get IPF men or women
men
what is histological finding in IPF
interstitial pneumonia
what is seen in lung pathology of IPF
- marked fibrosis
- honeycombing
what happens to elasticity of lung in IPF
there is a loss
where can TB affect
anywhere
symptoms of IPF
- progressive dyspnoea
- cough
- mucus (sometimes)
- finger clubbing
- bi-basal end inspiratory crackles
spirometry results in IPF
restrictive
what is done to diagnose IPF
CT
what does CT show in IPF
- basal distribution
- subpleural reticulation
- traction bronchiectasis
- honeycombing
how does honeycombing show in CT
cystic airspaces with irregularly thickened walls
when is bronchoalveolar lavage done for IPF
in infective or malignant cause suspected
differential diagnosis for IPF
- interstitial pulmonary oedema
- infection
- lymphangitis carcinomatosa
what is survival rate for IPIF
2-5 years
treatment of IPF
- pirfenidone
- nintedanib
what is pirfenidone
an antifibrotic agent
SE: rash
what is nintedanib
inhibitor of tyrosine kinases
what causes hypersensitivity pneumonitis
allergic reaction affecting the small airways and alveoli
what are the causes of hypersensitivity pneumonitis
- farmers
- bird fanciers
- maltworkers
- humidifier
- mushroom worker
- cheese washer
- winemaker
what is most common cause of hypersensitivity pneumonitis
farmer lung
histological findings of hypersensitivity pneumonitis
- chronic inflammatory infiltrate
- poorly defined interstitial granulomas
- interstitial fibrosis and honeycomb
symptoms of hypersensitivity pneumonitis
- malaise
- dyspnoea
- cough
- weight loss
- inspiratory squeaks
- fine crackles
when do symptoms present for hypersensitivity pneumonitis
4-6 hours after exposure
what does CT show in hypersensitivity pneumonitis
ground glass appearance
what is spirometry in hypersensitivity pneumonitis
restrictive
what can bronchoalveolar lavage good in hypersensitivity pneumonitis
to differentiate between sarcoidosis
treatment of hypersensitivity pneumonitis
- avoidance of exposure
- prednisolone
does fibrosis resolve
no
what causes humidifier fever
- humidifiers
- air conditioning units
what is langerhans cell histiocytosis characterised by
proliferaiton of langerhans cells
what is langerhans cell histiocytosis associated with
smoking
what does CT show in langerhans cell histiocytosis
- interstitial thickening
- nodules
- cysts
- honeycombing
treatment for langerhans cell histiocytosis
- smoking cessation
- corticosteroids
- chemotherapy agents
- lung transplant
what is Pulmonary lymphangioleiomyomatosis
rare disorder of premenopausal women
wha does Pulmonary lymphangioleiomyomatosis cause
hamartomatous smooth muscle infiltration of lungs
treatment of Pulmonary lymphangioleiomyomatosis
- lung transplantation
what is a granuloma
mass or nodule composed of chronically inflamed tissue formed by the response of the mononuclear phagocyte
what is granuloma characterised by
presence of epithelioid multinucleate giant cells
why does granuloma formation occur
to confine a pathogen and limit the extent of surrounding inflammation
where are granulomas seen
- TB
- fungal infections
- sarcoidosis
- hypersensitivity pneumonitis
what is sarcoidosis
multisystem granulomatous disorder
who does sarcoidosis affect
young adults
wha does sarcoidosis present with
- bilateral hilar lymphadenopathy
- pulmonary infiltration
- skin or eye lesions
what other infection can mimic sarcoidosis
beryllium posoning
how is sarcoidosis detected
CXR
who is most likely to contract sarcoidosis
Afro-Caribbean
what is Mantoux test results in sarcoidosis
negative
where can sarcoidosis affect
any organ
symptoms of sarcoidosis
- eye, skin or lymph nodes involved
- fatigue
- bilateral hilar lymphadenopathy
- erythema nodosum
- arthralgia
- fever
- wheeze
- SOB
- cough
- hepatomegaly
HRCT in sarcoidosis
non-caseating granulomas
what is bilateral hilar lymphadenopathy associated with
- dull ache in chest
- malaise
- mild fever
what is most common cause of erythema nodosum
sarcoidosis
what is initial test for sarcoidosis
chest x-ray
what does transbronchial biopsy show in sarcoidosis
- non-caseating granulomas
first line treatment for sarcoidosis
prednisolone
4-6 weeks
what is 2nd line treatment for sarcoidosis
immunosuppressant
e.g. methotrexate, azathioprine
(can be added to prednisolone)
what is end stage treatment for sarcoidosis
lung transplantation
who does Granulomatosis with polyangiitis affect
older adults
what is c-ANCA result in Granulomatosis with polyangiitis
positive with elevated PR3 antibodies
symptoms of Granulomatosis with polyangiitis
- bloody nasal discharge
- crusting and destruction
- sinusitis
- cough
- dyspnoea
- pleuritic chest pain
how should diagnosis be confirmed in Granulomatosis with polyangiitis
biopsy
another name for Anti-glomerular basement membrane disease
Goodpasture syndrome
what is goodpasture syndrome characterised by
- pulmonary haemorrhage
- glomerulonephritis
- presence of circulating antibodies
treatment of Goodpasture syndrome
plasmapheresis
prevents further antibody production
what is caplan syndrome due to
occupational dust inhalation
who gets caplan syndrome
coal workers pneumoconiosis
haven’t done pulmonary infiltration with eosinophilia
haven’t done pulmonary infiltration with eosinophilia
