ILD Flashcards
When type of granuloma do you get in the lung in ILD?
LOOSE
caseating= TB
non caseating=sarcoid/ beryliosis/ silicosis
how can you differentiate HSP with RBILD?
RBILD will be localised to the upper lobes.
What are the HRCT features of HSP?
Non-fibrotic- centrilobular nodules in all lobes, ground glass, mosaic attenuation, air trapping.
Fibrotic- honeycombing, traction bronchiectasis, reticulation, 3 density pattern (GG+airtrapping+nodule in same lobe), SPARES base of lungs
What are the poor prognostic factors in HSP?
cigarette smoking, lower baseline VC, lack of BAL lymphocytosis, persistent exposure to the inciting agent, and/or inability to identify an inciting agent
What is the diagnostic criteria for HSP?
1) exposure identification, serum IgG testing against potential antigens associated with HP, and/or specific inhalation challenge
2) imaging pattern
3) BAL lymphocytosis/ histopathological findings
What is the cell count in a normal BAL?
Alveolar macrophages >85%
Lo (CD4/CD8 0.9–2.5) 10–15% (higher in smokers) Neutrophils <3%
Eo <1%
Squamous epithelial <5%
What is the BAL differential for >15% lymphocytes?
Sarcoidosis
NSIP
HSP
Drug-induced pneumonitis
Collagen vascular diseases
Radiation pneumonitis
COP
Lymphoproliferative disorders
> 25% granulomatous ILDs- sarcoid/HSP/beryllium
> 50% HSP
What is the BAL differential for >1% eosinophils?
Eosinophilic pneumonia
Drug-induced pneumonitis
Bone marrow transplant
Asthma
bronchitis
Churg-Strauss
ABPA
Bacterial, fungal, helminthic, Pneumocystis infection
Hodgkin’s disease
> 25% eosinophilic pneumonia
What is the BAL differential for >3% neutrophils?
Collagen vascular diseases
IPF
Aspiration pneumonia Infection: bacterial, fungal Bronchitis
Asbestosis
ARDS
Diffuse alveolar damage
> 50% Acute lung injury, aspiration or suppartive infections
what differentiates Langerhans cell histiocytosis on BAL?
> 5% CD1a-positive cells
what differentiates Pulmonary alveolar proteinosis on BAL?
PAS-positive amorphous debris
What genes are associated with familial interstitial pneumonias (namely IPF)?
SFTPC (z1%), SFTPA2 (z1%), TERT (z15%), and TERC (z1%) are responsible for about 20% of all familial interstitial pneumonias
MUCB gene assoicated with IPF
What can fibrosing COP be associated with?
underlying polymyositis or antisynthetase syndrome
Which biomarkers are associated with rapidly declining LFTs?
SP-A, SP-D, KL-6 (Krebs von den Lungen-6), CCL18 (chemokine ligand-18), and MMP-7
These are all in IPF
What are the associated RF for IPF?
Male, smokers, GORD, chronic viral infections such as EBV/Hep C, FHx of ILD
When doing an HRCT when are the images taken?
end inspiration and end expiration, +/- prone
What are the HRCT findings of UIP?
traction bronchiectasis, subpleural reticulation/thickening, honeycombing- basal predominence, (GGO)
Which blood tests should be done in ILD?
CRP, ESR, ANA, RF, anti-CCP, anti-Jo1 etc
then if suspected can do anti-Scl70, anti-toperiomase, anti-centromere, Anti-Ro/La, ANCA
What are the histological findings on UIP?
Dense fibrosis with architectural distortion (i.e., destructive scarring and/or honeycombing)
subpleural/paraseptal distribution of fibrosis
Patchy involvement of lung parenchyma by fibrosis
Fibroblast foci
What defines progressive lung fibrosis in LFTs of ILD patients?
Absolute decline in FVC of >5% within 1 year of follow-up.
Absolute decline in TLCO >10% within 1 year of follow-up.
INBUILD and RELIEF are the studies that show that antifibrotics help in non IPF PPF setting too
criteria for antifibrotics?
ATS:
1. Absolute decline in FVC of >5% within 1 year of follow-up. Absolute decline in cDLCO >10% within 1 year of follow-up.
2. Radiological evidence of disease progression
3. Worsening respiratory symptoms
NICE:
FVC 50-80% predicted
and treatment stops if >10% decline in 1yr
What other malignancies is Langerhans associated with?
myeloid neoplasms
lymphomas
solid organ malignancies (especially lung and thyroid ca)
When should spiro be carried out according to NICE for IPF?
baseline, 6 months and 12months and earlier if three is deterioration
What are the treatments for cough in IPF?
treat co-morbs/underlying cause, opoids, (thalidomide- off license)
What are Birbeck granules seen in?
Langerhans
what sort of pattern of fibrosis do you get with prolonged nitrofurantoin use?
tends to be NSIP
What are the absolute contraindications to pirfenidone?
preg/breastfeeding
smoking (increases clearance!)
eGFR <30
severe hepatic impairment (Child Pugh C)
With which antibiotic does pirfenidone need to reduced?
ciprofloxacin
CYP1A2 inhibitors interact
which drugs are implicated in organising pneumonia?
rituximab, amiodarone, interferons, minocycline and statins
