interstitial lung disease Flashcards
most common type of interstitial lung disease
WHAT ARE THE IMAGING FINDINGS?
idiopathic pulmonary fibrosis
CXR - bibasilar septal line thickening with reticular changes and volume loss and bronchiectasis when more severe. diffuse interstitial lung opacities and reduced lung volumes. CXR can also be normal.
NEED HRCT for diagnosis since CXR can be normal.
HRCT- shows bilateral, peripheral, and basal predominant septal line thickening with honeycomb changes
- will show usual interstitial pneumonia
- Biospy may not be necessary for diagnosis.
demographics of pts who have idiopathic pulmonary fibrosis
presentation of IPF
50-70 with history of smoking.
See dyspnea on exertion, progressive dypsnea, clubbing of fingers and chronic dry cough
CXR and physical exam of idiopathic pulmonary fibrosis
on CXR : see decreased lung volumes with lower posterior lung zone predominance
clubbing and early inspiratory velcro crackles, may be hypoxic
HRCT scan of idiopathic pulmonary fibrosis shows
honey combing, cystic changes, and traction bronchiectasis (at the bases and subpleural areas).
can be diagnosed by CT but if unclear can get lung biopsy which will show usual interstitial pneumonia
do we every use bronchoscopy with biopsy to make diagnosis of interstitial lung disease
no it’s not able to get adequate tissue if biopsy is needed. prefer a VATS.
ILD in ages 20-50 think of these differentials
sarcoidosis,
pulmonary histocytosis X (pulmonary langerhaan cell granulomatosis)
hypersensivity pneumonitis
LAM (if young woman)
if suspect ILD and silicosis, look at exposure history:
history of exposure to TB
job occupation
histoplasmosis
Subacute causes of ILD
chronic UIP
chronic hypersensitivity pneumonitis
asbestosis
rheumatoid arthritis, chronic scleroderma or other connective tissue disorder (dermatomyositis)
beryllosis
rocket and microchip making or fuorescent lighting
Why do we need to get HRCT everytime when we consider ILD?
because CXR is 15% negative for changes
pulmonary lymphangioleiomyomatosis (LAM)
cystic lung dx seen in young women
Presentation: present with pneumothorax (often recurrent) chylothorax, chylous ascites, hemoptysis and multiple thin walled cysts scattered diffusely in the lungs without nodules or fibrosis on imaging studies.
see elevated VEGF-D
pulmonary lymphangioleiomyomatosis (LAM) diagnosis
clinical but definitive is lung biopsy
pulmonary lymphangioleiomyomatosis (LAM) treatment
transplantation of lung
OCP and pregnancy worsen disease can also see luterine leiomyomas and renal angiomyolipomas as well
manfestation of Cystic fibrosis patients
bronchiectasis and chronic bronchitis
see _pancreatic insufficienc_y, cough, SOB and other signs of malnutrition and vitamin deficiency
Diagnosis: elevated sweat chloride testing
pulmonary langerhan cell histiocytosis X is
common in smokers seen in ages 20-50 yrs old.
HRCT findings: see diffuse thin walled cystic lung dx with upper lobe predominance
Commonly will have restrictive disease. Only see obstruction on PFTs with significant cystic dx. Will have lower DLCO
Tx: smoking cessation and can give steroids.
advanced sarcoidosis can have
cystic lung dx and bilateral hilar LAD.
cryptogenic organizing pneumonia (COP)
cough, fever, dyspnea, malaise, myalgias and presentation is similar to CAP or flu like illness. IT has an acute to subacute onset (<2 months) and see fever and they may get multiple rounds of abx
when do we see clubbing if the fingers?
IPF,
asbestosis,
CF
hypersensitivity pneumonitis
bronchiectasis
and occasionally in lung cancer.
Rarely seen in COPD or sarcoidosis
signs and symptoms of bronchiectasis
daily mucopurulent cough with significant sputum production, (cups of it)
can see rhinosinusitis, dyspnea, hemoptysis, crackles and wheezes
causes of bronchiectasis
irreversible enlargement of airways due to destruction of airway architecture;
Pathophysiology: injury to lung from prolonged irway inflammation which causes subsequent mucus stasis and leads to further airway obstruction, chronic infection and inflammation.
airway obstruction (cancer),
rheumatic dx (RA Sjogren’s)
toxic inhalation
chronic or prior infection (aspergillosis and mycobacteria)
immunodeficiency (hypogammaglobulinemia, congenital CF and
alpha 1 antitrypsin deficiency
ciliary dismotility - Kartagner’s
Evaluation of bronchiectasis
pathphysiology
diagnosis
labs:
irreversible enlargement of airways due to destruction of airway architecture;
Pathophysiology: injury to lung from prolonged irway inflammation which causes subsequent mucus stasis and leads to further airway obstruction, chronic infection and inflammation.
diagnosis of bronchiectasis is by HRCT scan of chest. See airway diameter that is greater than that of accompany vessel and lack of distal airway tapering. Can have thickened walls or cysts.
Need to figure out why bronchiectasis is present. Rule out underlying bacterial or mycobacterial infection.
Look at connective tissue dysfunction
fribrosis and ciliary dysfunction or alpha 1 anti-trypsin deficiency
CHECK:
immunoglobulin quantification
CF testing,
sputum culture (bacteria, fungai, mycobacterium and PFTs)
cyclophosphamide can cause
pulmonary toxicity with pneumonitis with symptoms of cough, fatigue and dyspnea.
NO longer use this to treat NSIP from diffuse scleroderma. Preferred to use mycophenolate mofetil for treatment
silicosis associated occupations are:
coal mining (underground and surface)
hard rock mining (Granite, slate, sandstone)
masonry,
construction glass manufacturing
ceramic production
clinical presentation of acute silicosis (less common)
symptoms within a few weeks or years after exposure rapid onset of cough, weight loss, fatigue and possible pleuritic chest pain CXR shows basilar alveolar filling pattern without rounded opacities or lymph node calcifications.
prognosis of acute silicosis?
poor prognosis with silicosis and cor pulmonale and respiratory failure and <4 year survival rate
clinical presentation of chronic silicosis (most common)
asymptomatic and slowly develops symptoms 10-30 years after silica exposure
CXR with <10 mm diameter nodular opacities that are rounded, irregular, and in mid to upper lung zones. Nodules can coalesce to form (progressive massive fibrosis) PMF with severe respiratory symptoms or cor pulmonale
Accelerated silicosis clinical presentation
asymptomatic, or slowly developing symptom within 10 years of exposure to high levels silica
It doesn’t show up within a few weeks or years (acute) but doesn’t appear after 10 or 30 years (chronic).
CXR similar to chronic silicosis increased risk for PMF (progressive massive fibrosis)
What is silicosis associated with?
increased risk for lung cancer
high risk for mycobacterial disease
associated with scleroderma and RA
airflow limitation and chronic bronchitis
respiratory protective devices can help
with prevention of disease but also can be seen in workers who use personal respiratory protection.
Chest imaging of chronic silicosis is
rounded and nodular opacities <10 mm in diameter in mid to upper lung zones
some nodules may coalesce in some pts to form progressvie massive fibrosis
see resulted retracted hila, upper lobe fibrosis, and lower lobe hyperinflation.
diagnosis of silicosis
clinical findgins, occupational history, and chest imaging and absences of other etiologies Spirometry shows mixed obstructive and restrictive impairment decreased FEV1 and FEV1/FVC ratio. PMF can be seen with sharper decline in PFTs. may need lung biopsy.
Treatment of silicosis
supportive treatment (bronchodilators and supplemental oxygen) with possible lung transplantation for end stage silicosis.
who gets asbestosis?
plumbers,
carpenters,
electricians
janitors.
presentation of asbestosis?
present 20-30 yrs post initial exposure with chronic dyspnea and see restrictive dx on spirometry without airflow obstruction
chest imaging findings for asbestosis?
see pleural plaques
when do pts with sarcoidosis present
10 or 40 yrs. See upper lobe involvement
fibrotic radiation pneumonitis
dyspnea, chest pain, and see CT with volume loss and coarse reticualr/dense opacities. Pt has history of radiation therapy 12 months ago
treatment of acute radiation pneumonitis:
prednisone 60 mg daily for 2 weeks followed by gradual taper
clinical presentation of acute radiation pneumonitis:
antibiotic non responsive pneumonia can have fever, dyspnea, pleurtic chest pain, and cough. labs show leukocytosis have history of radiation to neck and chest 4-12 weeks ago.
when does acute radiation pneumonitis develop?
4-12 weeks after radiation therapy
how to diagnose acute radiation pneumonitis
after failing course of antibiotics. needs bronchoscopy to rule out infection and malignancy CT scan will show perivascular haziness and see patchy ground glass alveolar infiltrates or consolidative opacities. See “straight-line effects” of distinct boundaries where pneumonitis is from normal parenchyma.
Long term complications of acute radiation pneumonitis
see fibrotic radiation pneumonitis progression after 6 to 12 months time.
does smoking cause bronchiectasis?
no.
Bronchiectasis results from damage from bronchial walls from inflammation and infection.
hypersensitivity pneumonitis is
repetitive inhalations of antigens in a sensitized pt which results in immunologic response that forms noncaseating granulomas and peribronchial mononuclear cell infiltration with giant cells.
extrinsic allergic alveolitis and a form of interstitial lung dx that develops after inhalation of allergens (microbes, animal/plant proteins, dust, fungi, and chemicals)
Three forms of HP.
acute HP: large exposure to inciting antigen. see fevers, cough, fatigue within 12 hrs of exposure. CXR will have diffuse micronodular dx or be normal. Hear inspiratory crackles. HRCT- d_iffuse centrallobular micronodules and ground glass opacities_. TX: remove antigen and pt feels better within 48 hrs.
Recurrence of symptoms with repeat exposure is HALLMARK.
subacute and chronic HP : lower level prolonged exposure of antigen. See cough, fatigue, weight loss and SOB. HRCT shows micronodules and ground glass opacities but ALSO evidence of septal line thickening and fibrosis. Significant long exposure shows traction bronchiectasis and honeycomb changes on CT. TX: r_emove antigens essential for treatment._
Steroids are sued in pts with severe dx.
bird fancier’s lung is
chronic hypersensitivity pneumonitis - bird antigens cause problems and pt experiences cough, fatigue and weight loss and SOB.
lower level prolonged exposure of antigen.
HRCT shows micronodules and ground glass opacities but ALSO evidence of septal line thickening and fibrosis. S_ignificant long exposure shows traction bronchiectasis and honeycomb_ changes on CT.
TX: remove antigens essential for treatment.
presentation of hypersensitivity pneumonitis
acute or subacute or chronic depending on duration of exposure subacute and chronic forms a present with worsening sympotms
subacute form of hypersensitivity pneumonitis
resolves when antigen exposure ceases (with or without corticosteroids) people who are untreated develop interstitial fibrosis and chronic hypersensitivity pneumonitis. also 50% may have digital clubbing
subacute cough and dypsnea and see digital clubbing. has a new pet and symptoms stop when pt is away on vacation
hypersensitivity pneumonitis
history of antigen exposure and cessation of symptoms on removal of antigenic environment are major historical clues for
hypersensitivity pneumonitis otherwise it’s difficult to distinguish from other forms of interstitial fibrosis
CT findings of hypersensitivity pneumonitis
see ground glass opacities with emphysematous changes in mid upper lung fields
work up for hypersensitivity pneumonitis
need to get inhalation challenge test
if obtained BAL showing lymphocytosis
if obtained lung biopsy would show poorly formed noncaseating granulomas in the lung periphery (needed for diagnosis)
pulmonary alveolar proteinosis PAP is a
diffuse lung disease of accumulation of periodic acid schiff positive lipoproteinaceous material in distal air spaces
presents similar to chronic eosinophilic pneumonia (fever progressive dyspnea, cough and weight loss)
imaging shows “bat wing” distribution. no eosinophilia
asbestosis presentation
HRCT findings
plumbers, carpenters, electricians and janitors
present 20-30 years after initial exposure with chronic dyspnea (cough and wheezing are unusual)
spirometry shows restrictive pattern and no airflow obstruction
see bilateral nodular or reticular opacities and pleural plaques and clubbing of digits.
idiopathic pulmonary fibrosis demographics
Presentation?
50-70 yrs old with 6 months of progressive dyspnea with exertion and chronic dry cough. No provoking factors, will have of history of smoking
see clubbing (50%) and late inspiratory velcro crackles at lung bases
History: treated for COPD without improvement, treated for CHF without improvement (crackles)
CXR - bibasilar septal line thickening with reticular changes and volume loss and bronchiectasis when more severe.
diffuse interstitial lung opacities and reduced lung volumes. CXR can also be normal.
NEED HRCT for diagnosis since CXR can be normal.
HRCT- shows bilateral, peripheral and basal predominant septal line thickening with honeycomb changes
- will show usual interstitial pneumonia - Biospy may not be necessary for diagnosis.
eosinophilic pneumonia is divided into
acute and chronic
acute eosinophilic pneumonia presentation
follows a rapid course (<3 weeks) of dyspnea and seen in smokers
see hypoxemic respiratory failure
see initial neutrophilic predominent leukocytosis and subsequent eosinophilia
see BAL>25% eosinophils - diffuse alveolar damage on lung biopsy
acute eosinophilic pneumonia
acute worsening of dyspnea due to high build up of eosinophils in lungs associated with smokers responds to steroids
treatment of eosinophilic pneumonia
steroids
both acute and chronic eosinophilic pneumonia respond well to steroids
keep them on it for 3 months followed by taping of steroids in subsequent three months.
labs of acute eosinophilic pneumonia
see initial neutrophilic predominent leukocytosis and subsequent eosinophilia
see BAL>25% eosinophils
- diffuse alveolar damage on lung biopsy
Difference between acute and chronic eosinophilic pneumonia
acute: <3 weeks, smokers, and see acute respiratory failure
chronic: >3 weeks and non smokers. don’t see respiratory failure
Chronic eosinophilic pneumonia presentation
Chronic Eosinophilic Pneumonia presentation: insidious onset of fever, cough, progressive dyspnea, wheezing, weight loss. NO respiratory failure unlike AEP. see peripheral eosinophilia>6% and elevated ESR and CRP, thrombocytosis. BAL>25% eosinophils suggests this lung biopsy will show interstitial and alveolar eosinophils with multinucleated giant cells and associated bronchiolitis obliterans with organizing pneumonia.
treatment of bronchiectasis
chronic productive cough and sputum - has irreverisble airway dilation. Tx of underlying cause may not lead to improvement in symptoms but could prevent further progression.
Tx is focused on airway clearance, treating infections, and preventing exacerbations. FOcus on preventing chronic or recurrent infections
Bronchodilators, inhaled steroids, and combo inhalers -help symptoms but don’t prevent future exacerbation or stop decline of lung function.
Can give antibiotics if needed
Cystic fibrosis definition
presentation
autosomal rescessive dx affecting CF transmembrane regulator (CFTR) gene and dx affects homozygous pts. See abnormally thick secretions that are difficult to clear.
Changes in respiratory secretions lead to bacterial colonization of airways and chronic bacterial infection resulting in chronic inflammation, sinusitis, chronic productive cough and bronchiectasis. Also not enough lung antiproteases to counteract neutrophilic elastases so worsening of lung damage
Thickened secretions cause problems for the GI tract with bile and pancreas and so see pancreatic exocrine and endocrine deficiency, liver dx, and malabsorption and maldigestion occurs; see b_owel obstruction, distal ileal obstructive syndrome, intussusception, and rectal prolapse. can also see kyphoscoliosis and airway hyperreactivity_
spirometry will show obstructive pattern.
CXR findings of CF pts
CXR: upper lobe predominant mucoid impaction
CF diagnosis
Diagnostic evaluation: SCREENING test is increased chloride in sweat in sweat chloride test
family history of CF is helpful for diagnosing.
sweat chloride is less sensitive in adults and so a negative test doesn’t rule out dx
DNA testing is what confirms diagnosis and provide prognosis.
if sweat chloride is elevated then needs genetic testing. if TWO mutations are present then diagnosis is made.
If genetic testing shows 0 to 1 gene then repeat sweat chloride and expanded genetic testing is indicated.
Diffuse parenchymal lung disease are characterized by:
initial work up
characteristics of presentation: non productive cough and dyspnea that is subacute or chronic without response to treatment
All suspected pulmonary parenchymal diffuse lung dx need to get: ANA, RF, anti -CCP and peptide antibodies in pts <40 yrs old.
Get a high res CT chest. and if can’t make diagnosis then consider lung biopsy
acute dypsnea and cough of short duration= think: PNA, PE, CHF, asthma onr infection.
Pulmonary Langerhan cell histiocytosis clinical features
considered a part of the smoking related diffuse parenchymal lung diseases
CT scan: diffuse thin walled cysts and several pulmonary nodules in the mid and upper lung zone on HRCT. see development of pulmonary HTN.
See presence of langerhan cells with S100 and CD1a staining on tissue biopsy or transbronchial biospy
Tx: stop smoking and can give steroids.
Other smoking related ILD or diffuse parenchymal lung diseases
RB- ILD or respiratory bronchiolitis- combined restrictive and obstructive dx on PFTs
Pulmonary Langerhan cell histiocytosis (histocytosis X) - restrictive dx and can be obstructive if see lots of cysts.
Desquamative intersitial pneumonia-
All have lower DLCO.
Tx all with smoking cessation and steroids
medication that can cause ILD?
methotrexate can possible cause pulmonary ILD
See pulmonary fibrosis with
amiodarone
nitrofurantoin
busulfan
bleomycin
Connective tissues disorders that can cause ILD?
connective tissues disorders that can cause this -
rheumatoid arthritis: bronchiolitis, organizing pneumonia, rheumatoid nodules and nonspecific interstitial PNA (NSIP) and UIP (same as IPF)
diffuse scleroderma- can cause NSIP. Treat NSIP with mycophenolate mofetil, not cyclophosphamide due to side effects.
Prognosis with IPF?
IPF is progressive disorder and median survival time is 3-5 yrs.
Progression of dx is not linear but variable with periods of relative stability and then acute declines with exacerbations.
- acute exacerbation is acute worsening of dry cough, dyspnea, of <1 month duration and see new findings of CT scan (bilateral ground glass opacities) after a period of relative stability. Need to rule out CHF and pulm edema
- can see right sided heart failure and pulmonary HTN in severe dx.
treatment of IPF is:
- optimize comorbidities like obesity, heart failure, and sleep disordered breathing; this is common due to nocturnal hypoxemia and increased prevalance of OSA
- treat hypoxemia with supplemental O2 as needed based on pulse oximetry.
- with deconditioning, get pulmonary rehab to i_ncrease exercise tolerance and improve quality of life_
- treat with nintedanib and pifenidone which target fibroblasts and this delays the IPF progression but not curative.
Needs to be followed by pulmnologist.
IPF exacerbation is defined as:
acute exacerbation is a_cute worsening of symptoms (dry cough, dyspnea) <1 month duration and see new findings of CT scan_ (bilateral ground glass opacities) after a period of relative stability.
Events are triggered by infection or idiopathic.
Need to rule out pulm edema and CHF b/c of the ground glass opacities.
Lung transplantation for IPF is for:
life prolonging therapy for those without cormorbidities that may limit life expectancy
- those that don’t qualify are those who have untreatable end organ damage outside the lungs.
early referral for eligible pts is needed.
Most common cause of death in IPF pts
respiratory failure.
those who need mechanical ventilation with IPF have a terrible prognosis and increased mortality
Guidelines recommend against intubation with IPF if lung transplantation is not an option and have severe exacerbation or poor performance status.
need to have advanced care planning and end of life goals of care and palliative strategies.
NSIP or nonspecific interstitial pneumonia is
most common diffuse lung parenchymal disease associated with autoimmune disorders. NSIP affects younger population than IPF, can get lung transplant for this if no other end organ damage.
- two forms of NSIP: cellular and fibrotic
- cellular form (better prognosis and responds better to immunosuppression)
HRCT: bilateral lower lobe reticular changes and NO HONEYCOMB changes.
NSIP HRCT findings:
most common form of ILD with connective tissue disorders; seen in pts <50yrs
HRCT: bilateral lower reticular changes and NO honeycomb changes. See areas of ground glass opacification.
seen in systemic sclerosis, SLE, Sjogren’s sydnrome, dermatomyositis, polymositis and undifferentiated connective tissue disorder.
COP or cryptogenic organizing pneumonia
Disorder that results from injury to lung, see patchy proliferation of granulation tissue that affects the terminal bronchiole and alveolar ducts and spaceis.
-seen after a infection, radiation exposure, drug induced pneumonitis, and autoimmune diseases
presents as a “pneumonia that doesn’t respond to course of antibiotics” as it’s misdiagnosed. Has a CXR with consolidation
Need HRCT chest- see ground glass opacities or areas of alveolar consolidation resembling an infectious pneumonia and infiltrates can be migratory on repeat imaging but also see peripheral nodules and nocules along bronchovascular bundle. Don’t need to have lung biopsy.
Tx with steroids and COP improves. Relapses of COP need taping of steroids and recurrence may need a immunosuppresive therapy.
2nd line is cyclophosphamide for pts who dont’ respond with steroids or for those who are critically ill from severe COP.
acute intestitial pneumonia is
rapidly progresses in days to weeks and basically cannot be distinguished from ARDS
HRCT: has pulmonary edema and open lung biopsy shows diffuse alveolar damage.
The main difference for knowing that this is acute interstitial pneumonia is that there is no precipitating reason for ARDS.
high mortality >50%, tx like ARDs with low tidal volume ventilation and give steroids but it doesn’t really help.
What is Lofgren’s syndrome?
bilateral hilar LAD
migratory polyarthralgia
erythema nodosum
fever
presentation of sarcoidosis that doesn’t need biopsy
Tx with NSAIDs, no steroids.
What does not require a biopsy for diagnosis of sarcoidosis?
asymptomatic bilateral hilar LAD - no fevers, malaise, night sweats to suggest malignancy
Lofgren’s sydrome - bilateral hilar LAD, migratory arthralgias, erythema nodosum and fever
Heerfordt syndrome - anterior uveitis, parotiditis, fever and facial nerve palsy
what is Heerfordt syndrome
sarcoidosis that doesn’t need a biopsy for diagnosis- anterior uveitis, parotiditis, fever and facial nerve palsy
Treatment of Sarcoidosis
treat with steroids but many pts don’t need to be treated.
Stage 0, I, will resolve spontaneously.
Stage II 50% will need steroids, and Stage III 80% need steroids
Decision to treat sarcoidosis depends on involvement and this is not based on radiographic findings.
Treatment is required low to mdeium dose steroid therapy on alternate days is needed.
Sarcoidosis is
granulomatous disease of unknown cause that affects several organs. Seen more in AA and younger pts. 90% of pts have lung involvement.
can be asymptomatic but can have lung involvement
HRCT findings: pulmonary parenchymal dx, intrathoracic LAD alone or in combination. There can be multiple appareances they can have small nodules alongside bronchovascular bundles.
PFTs: obstructive, restrictive or both
Diagnosis: non-caseating granulomas with exclusion of infections like TB and fungi
Complication of Sarcoidosis
Sarcoidosis can develop pulmonary HTN through multiple ways:
- chronic hypoxemia
- destruction of capillary bed so less capillary surface area
- granulomatous inflammation of pulmonary arteries
- compression of pulmonary arteries secondary contiguous LAD
- pulmonary veno-occlusive dx from granulomatous inflammation and LV ventircular dysfunction from cardiac involvement
If we see pulmonary HTN see prognosis and survival time of about 3 years.
Asbestos is associated with
increased risk for lung cancer regardless of smoking status.
what causes cryptogenic organizing pneumonia COP or formly known as BOOP (bronchiolitis obliterans organizing pneumonia)
infection - bacterial viral PCP or mycoplasma
drugs - cocaine or amiodarone
connective tissue dx: SLE< RA, Sjogren’s and scleroderma
idiopathic pulmonary fibrosis
hypersensitivity pneumonitis
diffuse alveolar hemorrhage.
Desqumative interstitial pneumonia
is one of the smoking related ILDs
- tx stop smoking and if they are exposed to notoxious air particles.
On CT: see bilateral hazy opacities with diffuse basilar subpleural ground glass opacities. can see cysts in areas of ground glass opacities
Bronchoscopy: numberous pigmented machrophages and distal airspaces and moderate infiltration of alveolar septum by lymphocytes and plasma cells and mild alveolar septal fibrosis.
