interstitial ling disease Flashcards
which area exactly is the interstitium of the lung ?
the area between the alveolar epithelium and pulmonary vascular epithelium
what are the different groups of interstitial lung diseases?
ILD of known association
granulomatous ILD
Idiopathic interstitial pneumonia
miscellaneous ILD
what are the types of exposure related ILD?
pneumoconiosis
drug-induced
connective tissue disease
radiation pneumonitis
what kind of drugs can induce ILD ?
amiodarone
methotrexate
nitrofurantoin
what are the differential diagnosis with ILD ?
infectious causes :
community acquired pneumonia + atypical pneumonia
chronic or remote mycobacterial infections
non-infectious possibilities:
lymphoproliferative disorders, cardiac pathology, acute respiratory distress syndrome
what are the symptoms associated with ILD ?
dyspnea cough fever ( more in an acute attack) hemoptysis chest pain/pleurisy
what would a smoking history suggest in terms of ILD ?
Idiopathic pulmonary fibrosis
Respiratory bronchioloitis-associated interstitial lung disease (RBILD)
Desquamating interstitial pneumonitis (DIP)
pulmonary langerhans cell histocytosis (PLCH)
which ILD is less likely to be associated with smoking ?
Sarcoidosis and hypersensitivity pneumonitis
hypersensitivity pneumonitis
what ILD is associated with bird breeders and farmers ?
hypersensitivity pneumonitis
what ILD is associated with ceramic workers?
silicosis
what ILD is associated with pipe fitters and shipyard workers?
asbestosis
what ILD is associated with coal miners ?
coal workers’ pneumoconiosis
what pathology is amiodarone associated with ?
pulmonary fibrosis
what pathology is associated with nitrofurantoin?
alveolar haemorrhages
what symptoms are more suggestive of collagen vascular diseases ?
Raynaud’s phenomenon
proximal muscle weakness
joint swelling/pain
what does mild inspiratory squeak in physical examination suggest ?
suggests airway centred diseases:
constrictive bronchiolitis
hypersensitivity pneumonitis
what is the main finding on auscultation with ILD?
classic velcro rales or inspiratory crackles
what kind of Lab investigations are ordered for ILD ?
CBC liver function calcium, liver function test and a urine analysis along with immunological tests: ssDNA and complement levels in SLE Anti-SCL 70 for diffuse scleroderma
what is the current gold standard for imaging in ILD ?
HRCT
what are the radiological findings in idiopathic pulmonary fibrosis ?
1- X-ray: reticular opacities
2- HRCT : traction bronchiectasis
bilateral, basal honeycombing
bilateral, basal honeycombing
what are the radiological findings in sarcoidosis on X-ray and HRCT ?
HRCT: septal bleeding
nodules next to bronchovascular bundles
X-ray: hilar lymphadenopathy
nodules
which ILD show cystic changes with imaging ?
LAM
PLCH
what is the hallmark of ILD ?
restrictive changes in pulmonary physiology
what is the cause of the hypoxia inn patients with ILD?
ventilation perfusion mismatch
what to do if gas exchange appears normal at rest ?
do exercise testing ( 6 minute walk test) to unmask the defects
when can bronchoalveolar lavage be useful when it comes to ILD ?
useful in ruling out malignancies and infections
bronchoscopic biopsies are of limited utility in the evaluation of ILD except with ….
sarcoidosis
what is the treatment option for ILD?
providing oxygen to hypoxemic patients pulmonary rehabilitation pneumococcal and influenza vaccination remove offending environmental agents corticosteroids and immunosuppressants
what specific indications should be taken with LAM patients ?
avoid pregnancy , oophorectomy, progesterone, antiestrogens
give sirolimus
what is more commonly seen after the age of 50 ?
idiopathic pulmonary fibrosis
where is the affection in chronic hypersensitivity pneumonitis ?
upper lobe
where is the affection in acute hypersensitivity pneumonitis ?
lower lobe
when do we hear an expiratory wheeze refractory to inhaled bronchodilators ?
constrictive bronchiolitis
what is the sign seen on HRCT associated with hypersensitivity pneumonitis ?
head cheese sign
what is seen on HRCT of lymphangioleiomyomatosis LAM ?
thin walled cysts
what is seen on HRCT off PLCH ?
diffuse variable sized
bizarre shaped cysts
what is PAP ?
pulmonary alveolar proteinosis , accumulation of surfactants within the alveoli
what is seen on HRCT in PAP ?
ground glass opacities demarcated by thickened interlobular septae
crazy paving ( variable alveolar filling )
what is the appearance of BAL associated with pulmonary alveolar proteinosis ?
BAL of PAP appears as a milky fluid with debris and foamy macrophages
what are the results of flow cytometric analysis of lymphocytes in sarcoidosis vs Hypersensitivity pneumonitis ?
hypersensitivity pneumonitis : CD4/CD8 < 1
sarcoidosis : CD4/CD8 > 2.5
an increase in neutrophil count shows a worse prognosis in which diseases ?
sarcoidosis
hypersensitivity pneumonitis
idiopathic pulmonary fibrosis
what method of obtaining a tissue sample is preferred when it comes to ILD ?
thoracoscopy guided and open lung biopsy
when can we perform plasmapheresis ?
diffuse alveolar hemorrhage
what is the treatment for PLCH ?
cladribine
what is the treatment for PAP ?
whole lung lavage
GM-CSF therapy
definitive management for any end stage lung ?
lung transplant
what type of granulomatous disease is sarcoidosis ?
non caseating granuloma
what is the most common presentation associated with sarcoidosis ?
hilar and left paratracheal adenopathy
positive despine sign
what are the markers positive in pulmonary langerhans cell histocytosis
CD1a and S100
what is the management in LAM ?
avoid pregnancy and give sirolimus
