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CR - Interstitial Lung Disease (Pul Fibrosis) (iv) IPF (usual interstitial…
CR - Interstitial Lung Disease (Pul Fibrosis) (iv) IPF
sarcoidosis
restrictive
multisystemic
eyes
any part
visual impairment
skin
erythema nodosa - hot painful lumps on ant shin (also caused by IBD)
lupus pernio - painful red/purply rash on face
nodes (usually bihilar adenopathy of mediastinal nodes - hard to access for Bx)
spleen in 10%
lungs in 90-95%
BM in 40%
parotid gland (painful enlargement in 10%)
liver
heart
brain (v rare, only in v extensive cases)
kidneys
cranial Ns
musculoskeletal system
Dx of exclusion
many dDxs as many conditions cause granulomatous inflamm (TB, berylliosis, silicosis, foreign body, EAA, lymphoma, carcinoma)
always requires CPC @ MDM
aetiology
unknown
disordered immune regulation in genetically predisposed individuals (esp excessive cellular immunity - CD4 accumulation + IL2 release)
possibly environmental antigens
no evidence to suggest its caused by an infectious agent
increased incidence in sweden, denmark, Ire, African-Americans, non-smokers, females x2
onset age = 25-40
often asymptomatic, incidental finding on CXR
bilat hilar lymphadenopathy
right paratracheal strip enlargement
bilat mid zone predominant infiltrates
presentations
30% with resp symptoms (SOB, dry cough)
non-specific: fever, fatigue, weightloss, anorexia, night sweats
peripheral lymphadenopathy
cut lesions
eye involvement
neurosarcoid (involves CNS)
Lofgren syndrome: fever, erythema nodosum, polyarthritis (esp ankles), bilat hilar lymphadenopathy
Mikulicz syndrome: benign enlargement of parotids and/or lacrimal glands
investigations
CXR
CT-thorax
PFTs (restrictive defect)
ECG
bloods
look for increased Ca, alkaline phosphatase, serum ACE
do renal, liver + bone profile
EBUS-TBNA to sample hilar nodes
non-caseating granulomas (rim of lymphocytes around epithelium macrophages)
send to micro to rule out infection (esp before giving steroids - would make infection worse)
unpredictable clinical course
remain asymptomatic
progress
remission with relapses
10-15% of those with resp involvement progress to diffuse interstitial fibrosis pul hypertension + cor pulmonale
usually steroid responsive
Langerhan's cell histiocytosis (rare)
usual interstitial pneumonia (UIP)
confusing name - NOT INFECTIOUS
unknown aetiology, possible immune
diffuse interstitial fibrosis
affects males more
2/3 cases > 60 y/o
all other potential causes (e.g. asbestosis, connective tissue disorders) must be excluded before this can be Dxed
alv wall injury
response = inflamm (alveolitis aka pneumonitis) + healing by fibrosis
interstitial oedema
if persistent it progresses
hallmark = patchy fibrosis/inflamm alternating with normal tissue
honeycombing if advanced (lung spaces separated by inflamm fibrous tissue - seen on CT)
investigations
CXR (bilat interstitial shadowing)
HRCT-thorax
PFTs
ECG
FBC, U+E, LFTs, Ca, ABG
+/- bronchoscopy
+/- Bx of lung tissue
via VATS (video-assisted thoracic surgery)
difficult
done by thoracic surgeon under general anaesthetic
sub pleural/lower zone predominant
usually insidious gradual onset
dry cough, dyspnoea, clubbing, velcro crackles on inspiration, cyanosis, cor pulmonale (increased JVP + parasternal heave), peripheral oedema