ILD Flashcards
What factors indicate a poorer prognosis in UIP at diagnosis?
What radiological changes would you expect to find in a patient with Pleuroparenchymal Fibroelastosis (PPFE)?
Pleural thickening in the upper lobes with adjacent pulmonary fibrosis.
Around 50% of cases are idiopathic.
May get honeycombing/interlobular septal thickening.
Poor prognosis but no specific therapy. Progressive condition and may require referral for lung transplant.
Increased risk of pneumothoraces
What are the factors used to define PF-ILD?
FVC decline >10% over 24 months
FVC decline >5% and increased symptoms over 24 months
FVC decline >5% and radiological progression over 24 months
Increased symptoms and radiological progression over 24 months
What is the prevalence of ILD in Rheumatoid arthritis?
10%
What are the classic features of Hypersensitivity Pneumonitis on HRCT?
Centrilobular nodularity (inhaled allergen)
Ground glass changes/mosaicism
Air trapping
What is the most appropriate first line treatment for systemic-sclerosis related ILD?
Mycophenolate
Within which FVC range is treatment with antifibrotics indicated in IPF? Does this vary by agent?
FVC between 50 and 80% for pirfenidone.
Nintedanib has no upper limit for treatment (50-100%)
Nintedanib is associated with a higher risk of bleeding (use with caution if on DOAC)
What percentage of patients with sarcoidosis have pulmonary involvement (any, not specifically ILD)?
90%
What are the radiological features associated with LIP?
With which conditions is it associated?
Ground glass changes, lymphadenopathy, pulmonary nodules of varying size. MAy have some cysts.
Benign condition caused by lymphocytic infiltration of the lungs.
Associated with autoimmune conditions such as Sjogrens (most common lung presentation of Sjogrens), RA syndrome, SLE and conditions such as HIV.
If BAL is performed in a patient with sarcoid, what would you expect it to show?
Elevated lymphocyte count with an increased CD4:CD8 ratio (>4)
When can sarcoid be diagnosed without the need for biopsy?
If Lofgren’s syndrome is present then can make a confident clinical diagnosis:
Bilateral hilar lymphadenopathy
Erythema nodosum
Polyarthralgia
If not and aiming to treat then should look for confirmatory biopsy- often by EBUS
What is Lofgren’s syndrome? How common is it and what is the remission rate at 2 years? How is it treated?
Classic triad of bihilar lymphadenopathy, polyarthropathy and erythema nodosum associated with sarcoidosis.
Occurs in around 30% of sarcoidosis patients, with organ involvement rare
Usually responds well to steroids- higher doses of 30-40mg for a few weeks then taper and often shorter treatment duration (around 3 months)
Remission rate high- around 70-80% at 2 years
What are the indications for 2nd line therapy when treating sarcoidosis?
Pulmonary disease progression/unacceptable symptom burden despite adequate steroids
Unacceptable steroid side effects
Inability to wean steroids beyond 10-15mg/day maintenance
Major comorbidities adversely affected by steroids e.g. obesity, diabetes, osteoporosis
Strong patient aversion to the use of steroids
When may steroids be considered in the presence of sarcoid-related fatigue?
How should fatigue be investigated?
Usually only treat in the presence of organ involvement
Should always screen for alternative causes (Thyroid, iron, calcium, sleep disorder)
If treating then treat at the lowest possible dose of prednisolone +/- hydroxychloroquine.
Ginkgo Biloba is an alternative strategy alongside exercise and pacing.
Neurostimulants may be considered in extreme cases.
What is the UK 30-day mortality for surgical lung biopsy specifically for the diagnosis of ILD?
~2%
What effect do antifibrotics have upon the annual rate of FVC decline?
Reduce the annual rate of FVC decline by approximately 50%
What is anti-synthetase syndrome?
With which antibodies is it associated?
What is the typical ILD pattern?
Multisystematic autoimmune condition. Causes inflammatory myositis and ILD.
Common symptoms of mechanics hands, Raynaud’s and may get fevers and arthritis.
May get dermatomyositis-like features such as heliotrope rash/Gottron’s papules.
Most common antibody associated is anti Jo-1. Anti-histidyl-tDNA is commonly found in patients with ILD features
Organising pneumonia and NSIP are common presenting patterns on HRCT
What is the timeframe in which you may expect to see radiation pneumonitis?
Radiation injury usually a biphasic process:
Radiation pneumonitis usually 6 WEEKS to 6 MONTHS after radiotherapy then a more indolent process of fibrosis later on.
Often presents with dry cough, dyspnoea and fever.
What is the median life expectancy following diagnosis of IPF?
3 years
What is are the components of the GAP index? How is it used?
Used to predict mortality in patients with IPF. Incorporates:
Gender: M (1)
Age: 61+ (1) 66+ (2)
(Physiology)
Predicted FVC: 50-75 (1) <50 (2)
Predicted DLCO: 36-55 (1) <=35 (2) Unable to perform (3)
0-3 = Stage 1
Mortality 5.6% 1y 16.3% 3yr
4-5 = Stage 2
Mortality 16.2% 1y 42.1% 3yr
6-8 = Stage 3
Mortality 39.2% 1y 76.8% 3yr
What histological findings might you expect to find in HP?
Lymphocyte-rich infiltrate with a bronchogenic distribution (HP involves airways)
Multinucleated Giant Cells with cytoplasmic cholesterol clefts. May get granulomata
What is DIP? In whom is it more common? What are the histological/radiological findings?
Desquamative interstitial pneumonia. Closely related to respiratory bronchiolitis ILD (RB-ILD).
Associated with HEAVY SMOKERS (~90%) and more common in MALES (2:1)
Usually presents ages 30-60. May coincide with AI diseases like HIV or RA/scleroderma
Alveoli full with PIGMENT-LADEN MACROPHAGES
Usually bilateral symmetric DIFFUSE GROUND GLASS-OPACITIES
What is farmer’s lung and what is the likely causative organism?
Organic ILD presenting with recurrent chest infections. Improves with avoidance of the allergen. The causative organism is usually thermophilic actinomycetes that are found in the spores of hay or crops and animal feed. The condition is common in farmers.
Exposure causes an allergic reaction and the formation of circulating IgE.
What features may you expect to see in a biopsy of LCH?
Reniform (kidney shaped) Abundant eosiniphils and Birbeck granules (cytoplasmic organelles found only in Langerhans cells)
What is Erasmus syndrome? What is the common trigger?
Generalised progressive scleroderma following exposure to silica.
Can get classic features of scleroderma such as thickening of skin on the hands and fingers, Raynaud’s phenomenon and arthralgia accompanied by ILD.
Silicosis gives egg shell calcification of nodes and fibrosis
How should pirfenidone treatment be started (dosing)?
What should you do if there has been a gap in treatment?
Usually up-titrated. Start at 267mg TDS for 1 week then increase to 534mg TDS for 1 week then full dose 801mg TDS
If there is a gap in treatment (more than 14 days) then it should be up-titrated again with a similar regimen.
