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Chronic Diffuse Infiltrative Lung Disease
Terms in this set (104)
Chronic diffuse infiltrative lung disease primarily involves?
alveoli and lung interstitum
Chronic diffuse infiltrative lung disease secondary causes involves?
airways and pulmonary vasculture
Most common disease process in Chronic diffuse infiltrative lung disease?
post-inflammatory pulmonary fibrosis and idiopathic pulmonary fibrosis
Chronic diffuse infiltrative lung disease is a combination of what two factors?
genetics and environmental exposures
Steps of disease?
initiation of tissue injury
maintenance of inflammation
resolution or development of fibrosis
Most common symptoms?
dyspnea of gradual onset (exertion) and nonproductive (dry) cough
length of symptoms- months to years
occupation & medications
exposure to silica asbestos, farming dusts tobacco
exposure to pets and humidifiers
symptoms of underlying collage vascular disease or vasculitis
Hx of Raynaud phenomenon, photosensitive, skin rashes, difficulty swallowing, muscle weakness, arthritis
collagen vascular disease- synovitis, telangiextasia, sclerodactyly, mala rash
sarcoidosis- uveitis, erythema nodosum, plaque like skin lesions
Heart exam- L side HF mimic pulm disease
CMP, CBC, ESR, ANA, RF, SPE, ANCA, anti GBM
Patients presents with cytopenia, hyperkalemia, and elevated liver functions?
Patients presents with chronic eosinophilic pneumonia?
Patient presents with cytopenia?
malignancy and collagen vascular diseases
Elevated ACE levels suggest?
Sarcoidosis, TB, berylliosis, asbestosis, silicosis
According to guest lecture what is the first imaging module for Chronic diffuse infiltrative lung disease?
high resolution CT- determines extent and severity of lung disease
What imaging module will have nonspecific findings of diffuse bilateral disease?
PFTs of a Chronic diffuse infiltrative lung disease patient will have?
restrictive patten (reduced lung volume), normal or increased ratio of FEV/FVC, reduction in diffusing capacity of lung for CO, and reduction in arterial O2 tension with normal or reduced CO2
What two diagnostic tools aid in diagnosis?
bronchoalveolar lavage (BAL) and transbronchial lugn biopsy
Lung biopsy will include?
tranbronchial or open biopsy
immunization against pneumococcal (every 5 years)
Chronic diffuse infiltrative lung disease of KNOWN etiology? (4)
Malignant diffuse infiltrative lung disease
Chronic diffuse infiltrative lung disease of UNKNOWN etiology? (12)
idiopathic pulmonar fibrosis
desquamative interstitial pneumonia/ respiratory bronchiolitis interstitial lung disease
acute interstitial pneumonia
lymphocytic interstitial pneumonia
bronchiolitis obliterans organizing pneumonia BOOP
collagen vascular disease with associated pulmonary involvement
eosinophilic granuloma of the lung
diffuse alveolar hemmorrhage
drug induced disease can be what type of drugs?
cytotoxic or non-cytotoxic drugs
cytotoxic drugs: definition, types of drugs, clinical presentation, Dx, Tx, prevention.
-major causes of morbidity and mortality in pts treated for malignancy
-bleomycin (O2 toxicity), amiodarone, nitrofurantoin, cyclophosphamide, methotrexate, nitrosoureas
-cough and SOB (COMMON)
-CXR: bilateral symmetrical interstitial infiltrates
-interstitial inflammatory cell infiltration and fibrosis, increased type II pneumocytes and fibrosis
-Tx: eliminate drug, corticosteroids
-Prevention: more early Dx than prevention, switch to alternative drug
nitrofurantoin, amiodarone, gold and penicillamine, and illicit drugs
MOST common causes of drug induced disease
acute form- 2-10 days after 1st drug exposure
symptoms: cough, SOB, fever, pleurisy (1/3)
CXR: alveolar or interstitial infiltrates- may have pleural effusion
Dx: Hx, resolve in 1-4d of stopping med
pulmonary toxicity in 5-7% cases
symptoms: cough, SOB, LOW grade fever
CXR: infiltrates- nonDx, may have pleural effusion
Dx: exclude other causes (infection & CHF)
Tx: w/draw drug- corticosteroids in severe cases
Gold and penicillamine induced...
hard to dx due to other underlying disorders
dc drug- give Corticosteroids
Illicit drug induced...
talc induced granulomatosis & pulmonary fibrosis from injection of amphetamine or narcotic pills
Talc persist for years= fibrosis
Dx: biopsy of talc particles
pneumoconioses will manifest as what with what complications?
asthma, chronic bronchitis or diffuse parenchymal disease
complications: lung CA, pulmonary aspergillosis- INORGANIC
A patients presents with SOB over the past 20 years as an insulation installer, dry cough, fine end expiratory rales, digital clubbing, late- cor pulmonale, pleural plaques- this is suggestive of diffuse interstitial fibrosis? What is the clinical term of these symptoms?
Abestosis (type of pneumoconioses)
CXR, PFTs, biopsy, and Tx for Abestosis?
CXR: if early may be normal or small irregular shadows in lower lung
PFTs: resticitve pattern- decreased D(LCO), exercise induced hypoxemia
biopsy/DAL: excess asbestos
A 82 year old who use to be a quartz miner for 5 years when he was 18 y/o presents with normal Px, opacities in upper lode and hilar node enlargement on CXR is suggestive to have silicosis induced CDILD? What two forms may this patient have?
simple nodular silicosis or progressive massive fibrosis (PMF)
PFTs of a silicosis?
restricitve pattern- may have hypoxemia
Tx and further complications for a silicosis patient?
avoid exposure, corticosteroids & increase risk of TB
Coal workers pneumoconioses characteristics:
pulmonary fibrosis after exposure to coal dust/graphite with chronic cough with gray/black sputum with possible pMY w/ SOB
NO tx- avoid exposure
Chronic exposure to berylliosis can cause?
acute chemical bronchitis, pneumonitis, multi-systemic granulomatous disease (like sarcodosis)
Symptoms. CXR. PFTs. Lab, Dx, and Tx of berylliosis?
symp: SOB, cough, Cx pain, fatigue, wt. loss, arthralgia
CXR: ill defined nodular/ irregular opacities
PFTs: restrictive pattern with low D (LCO2)
Lab: hyperuricemia, hyperCa, hypercalcinuria, elevated ACE
Dx: hx, evidence on CXR/PFTs, granulomatous inflammation in lung
Rx: avoid exposure- corticosteroids
hypersensitivity pneumonitis defined:
interstitial lung disease = form inhalation of organic antigens
Farmer's lung causes by inhalation of proteins in thermophilic Actinomyces organisms in moldy hay and grain is also called?
Pathology of farmer's lung?
interstitial pneumonitis (2/x have granulomas), fibrosis, 50% bronchiolits obliterans, T lymphocytes of suppressor cytotoxic subclass
Patient with fever chills, cough, SOB< malaise 4-8 hours after antigenic exposure that improve after the removal of the exposure??
acute hypersensitivity pneumonitis
Patient with no systemic symptoms with chronic SOB and cough on exertion with a normal alveolar CXR, but BAL shows lymphocytosis and biopsy shows non specific pneumonitis with some granulomas/ bronchiolits obliterans?
chronic hypersensitivity pneumonitis
Treatment of chronic/acute hypersensitivity pneumonitis?
Stop exposure, add glucocorticoids use for acute and some chronic
Malignant diffuse infiltrative lung disease is causes by what and occurs with what?
causes by pulmonary lymphangitic carcinomatosis of adenocarcinomas (from breast, GI or lung) with cough, SOB and occurs with lymphangitic carcinomatosis, alveolar cell carcinoma, lymphoma, and leukemia
CXR shows bilateral reticular or reticulonodular infiltrates and Kerly B lines, pleural effusion, hilar lymphadenoapthy..?Dx with bronchoscopy and biopsy
Malignant diffuse infiltrative lung disease
Mutli-system disease of UNKNOWN etiology with non-caseating granulomas that is more common in blacks, 20-40 yo onset, and Male with organ involvement?
Pathogenesis of Sarcoidosis?
expansion of helper T cells in lungs activate by macrophages via interleuken and release of IL 2, B cells produce immuglobin (hypergammaglobilinemia)
Patient presents with bilateral hilar lymphadeopathy, diffuse infiltrative lung disease with SOB and Lofgren syndrome?
Lab studies of Intrathoracic sarcoidosis?
PFTs: reduced D(LCO2) and vital capacity w/o airflow obstruction
ACE: elevated 50-60%
Unusual others: pleural effusion, alveolar infiltrates, large nodular opacities, cavitation, atelectasis, calcifcation
Extrathoracic sarcoidosis manifestations:
erythema nodosum, lupus pernio, ocular involvement, GI, nervous system, cardiac, and endocrine
Most common skin rash in Extrathoracic sarcoidosis
erythema nodosum (non-granulomatous panniculitis) 10-20%
bluish-purple swollen lesion on nose, cheeks, earlobes, fingers toes, lips or knees...
Lupus pernio is seen in Extrathoracic sarcoidosis
Uveitis: anterior- tearing/photophobia, posterior- blurring vision
Ocular involvement in 25% seen in Extrathoracic sarcoidosis
GI involvements in Extrathoracic sarcoidosis?
stomach, esophagus, appendix, colon, rectum, liver, pancrease
Meningitis, encephalopahthy, hypothalamic lesions, cranial nerve involvement, seizures are seen in?
Extrathoracic sarcoidosis of nervous system involvement 5%
In Extrathoracic sarcoidosis what cardiac manifestations are seen?
arrhythmias and heart block- commonly seen on autopsy
In Extrathoracic sarcoidosis what endocrine manifestation seen?
hypercalcemia and pituitary dysfunction.
To diagnosis Sarcoidosis...
see non-caseating granulomas in tissue.. if there is pulmonary involvement perform brochoscopy with transbronchial biopsy
Differential dx for sarcoidosis...
granulomatous infections (TB)
Treatment for sarcoidosis..
glucocorticoids if symptomatic pulmonary involvemnt, with significant fever weight loss, hypercalcemia, and extra-pulmonary tissues that lead to func. impairment or risk of organ dysfunction
In a sarcoidosis patient, if glucocorticoids fail what is the next line of treatment?
methotrexate, azathiprine, myophenolate mofetil, hydroxychloroquine, cyclophosphamide or cyclosporine or LUNG transplant
Prognosis of sarcoidosis..
60-70% spontaneous remission
10-30% progressive course (lupus pernio, chronic uveitis, hyperCa, nephrocalcinosis, nasal mcuosal, cystic bone disease)
Patient presents with SOB on exertion and cough, fine inspiratory rales, and clubbing, what does this patient have?
idiopathic pulmonary fibrosis
Severe case of idiopathic pulmonary fibrosis presents with?
cyanosis and cor pulmonal- elevated JVP, edema, and prominent second heart sound
To diagnosis idiopathic pulmonary fibrosis, the patient will show?
diffuse pulmonary involvement w. no other organ involvement, no infection, hilar lymphadeopathy.
idiopathic pulmonary fibrosis prognosis and treatment...
2-3 survival rate- worse with smoking, sever SOB< low lung compliance, high # of fibroblastic foci
increase risk of lung carcinoma
Tx: corticosteroids with cyclophosphamide and azathiprine or lung transplant
desquamative interstitial pneumonia/ respiratory bronchiolitis interstitial lung disease....
cough, dyspnea, clubbing (50%), onset of 40 yo, men 2:1, SMOKERS, CXR- vague bibasilar opacities, Tx- stop smoking, steroids
20-30% mortality- 12 year survival rate
acute interstitial pneumonia...
similar to ARDS- no predisposing factor
<30 days= dyspnea, cough wit mucoid sputum fever evolves to respiratory failure
Dx: bronchoscopy with bronchoalveolar lavage
Tx: supportive O2, steroid
Mortality 33-78% in 6m onths
lymphocytic interstitial pneumonia....
diffuse lymphocyte infiltrates
occurs with autoimmune disease, dysproteinemias, immunodef., drug rxns, bone marrow transplant or idiopathic
onset 56 yo, women with cough SOB, rales and lymphadenopathy
Bronchoscopy on BAL- B cells
Tx: underlying issues, steroid and immunosuppressive therapy
bronchiolitis obliterans organizing pneumonia BOOP..
may be 2nd to proximal airway obstruction, rxn to drugs or inhalation of toxic fumes, viral infection, aspiration, lung transplant, inflamm bowel disease, collagen vascular disorders, or other lung diseases
Clinical manifestation of BOOP?
dry cough and SOB
fever, malaise, fatigue
Px: rales and some wheezes
Dx and Tx of BOOP?
Dx: clinical with CT, may need biopsy
collagen vascular disease with associated pulmonary involvement
-up to 2/3 of cases of collagen vascular diseases
-PFTs: restriction, small airway obstruction, reduced DlCO with hypoxia at rest and worsening on exertion
seen in systemic lupus, RA, progressive systemic sclerosis, polymyositis and dermatomyositis, sjorgen syndrome, mixed CT disease
eosinophilic granuloma of the lung..
SMOKING!- may develop DI 2nd to pituitary involvement
-granulomas involves bones
-cough, SOB, 25% CXR no symptoms
can develop spontaneous pneumothorax, reduced D(LCO) and vital capacity w/ airflow obstruction
-Dx: lung biopsy
Tx: stop smoking, glucocorticoids, 12.5 survival after dx
poor prognosis increased age, airflow obstruct, reduced D (LCO) and hyperinflation
Alveolar proteinosis defined..
intra-alveolar accumulation of cellular lipoproteinaceous material that resemble surfactant
Pathogenesis of Alveolar proteinosis..
90% acquire and caused by autoantibodies to GM-CSF- congenital cases 2nd to defects in surfactant proteins or defects in GM-CSF receptors
Clinical manifestations of Alveolar proteinosis
asymptomatic with abnormal CXR
abrupt cough, fever, CX pain caused by disease and complication
SOB and cough of gradual onset
Lab: elevated LDH with other normal serum enzymes
Dx: BAL with bronchoscopy- large amounts of PAS-positive material- exclude PCP
Tx: 10% remission, best tx is whole lung lavage with sterile saline
future- GM-CSF SQ
eosinophilic pneumonia has how many forms?
7: simple pulmonary eosinophilia (Loffler syndrome), prolonged pulmonary eosinophilia w/o asthma, pulmonar eosinophilia with asthma, tropical eosinophilia, pulmonary vasculitis, hypereosinohpilic syndrome, acute eosinophilic pneumonia
simple pulmonary eosinophilia (Loffler syndrome)...eosinophilic pneumonia
transient, migratory infiltrates, absent symptoms or SOB and dry cough
intra-aveloar accumulation of eosinohpils, macrophages, and fluid
Tx: stop drugs that may cause it and tx parasitic infection
prolonged pulmonary eosinophilia w/o asthma...eosinophilic pneumonia
middle aged women
productive cough, SOB, malaise, wt. loss, night sweats, fever with progressive per. pulm. infiltrates
Dx: based on symptoms, CXR, peripheral eosinophilia, may BAL and broncoscopy
tropical eosinophilia...eosinophilic pneumonia
onset of asthma, fever, blood eosinophilia, CXR with basilar and alveolar infiltrates-
traveled Far East
represents form of filariasis
pulmonary vasculitis....eosinophilic pneumonia
multi-system disorder with vasculitis amnd granulomatous inflammation of lungs, nervous system, skin
eosinophilia w/ elevated IgE
acute eosinophilic pneumonia ....eosinophilic pneumonia
acute onset of cough, SOB fever, tachypnea, rales
No association with smoking or exposures or drugs
Associated with glomerulonephritis and grew. immunologically mediated, what is the disorder?
diffuse alveolar hemorrhage
Disease that may cause diffuse alveolar hemorrhage?
-anti-glomerular basement membrane antibody (Goodpasture syndrome)
-idiopathic and rapidly progressive glomerulonephritis w/ or w/o immune complexes
-SLE and other collagen vascular diseases
-antiphospholipid antibody syndrome
idiopathic pulmonary hemosiderosis
Dx of diffuse alveolar hemorrhage?
-anti-GBM antibody, antinuclear, antiDNA, ANCA complement levels, immune complexes
-BAL with iron stain
Is BAL iron stain is hemosiderin laden macrophages what is the Dx?
alveolar hemorrhage confirmed
If BAL iron stain is not hemoisderin laden macrophages what is the next step in Dx?
biopsy of kidney, lung, or other involved sites
Anti-GBM antibody disease defined?
young men who SMOKE
glomerulonephritis and diffuse alveolar hemorrhage caused by cytotoxic antibody against alpha3 chain of type IV collage in glomerular and alveolar basement membrane
Anti-GBM antibody disease presentation?
hemoptysis, complain of SOB, gross hematuria, Iron def anemia, renal failure (50%)
Anti-GBM antibody disease CXR & PFTs?
CXR: early fluffy perihilar acinar shadows- later interstitial changes
PFTs: restrictive pattern, D(LCO) may be increased due to CO uptake by extravascular hemoglobin
Anti-GBM antibody disease Dx:
confirmed by anti0GBm antibodies (95%) or linear deposits of IgG (rarely IgA) on glomerular or alveolar basement membrane
Anti-GBM antibody disease Tx:
immunosuppressive agents, glucocorticoids, plasmapheresis
50% die or require LT dialysis
40 year old male presents with cough and dyspnea. Hx of smoking. Clubbing of digits. CXR reveals vague bibasilar opacities. Lung biopsy is indicated for dx.
Desquamative interstitial pneumonia/respiratory bronchiolitis interstitial lung disease
Tx: quit smoking, steroids
(mortality:20-30% with mean survival 12 years, yikes)
A patient presents with a rapid onset (under 30 days) and worsening of dyspnea and cough with mucoid sputum. It appears to be ARDs but there is no predisposing factor. Bronchoscopy with bronchoalveolar lavage is indicated. Likely diagnosis and treatment?
Acute interstitial pneumonia
treatment is supportive (steroids not proven effective)
mortality: 33-78% in 6 months, super yikes
56 year old female with a history of Sjogren syndrome presents with cough and dyspnea. Rales and lymphadenopathy are found on exam. Bronchoscopy reveals B cells on BAL. Also, diffuse lymphocyte infiltrates are present. Dx and Tx?
Treat the underlying disease: steroids and immunosuppressive therapy
55 year old patient presents with dry cough, dyspnea, fever, malaise and fatigue. Rales and wheezes are heard on exam. Patient has a Hx of proximal airway obstruction. CXR show alveolar infiltrates. Biopsy is indicated. Dx and Tx?
Bronchiolitis obliterans organizing pneumonia (BOOP)
DDx: IPF has clubbing of digits
35 year old patient with a Hx of smoking has an abnormal CXR and has developed a spontaneous pneumothorax. Granulomas are found in the bones. PFT: reduced Dlco and vital capacity with airflow obstruction. Dx and Tx?
Eosinophilic granuloma of the lung
Tx: smoking cessation, glucocorticoids
DDx: spontaneous pneumothroax also occurs with AIDs-related PCP
What are the 3 presentations of alveolar proteinosis?
- asymptomatic with abnormal CXR
- abrupt onset of cough fever, and chest pain caused by disease and complicated by opportunistic infection
- dyspnea and cough of gradual onset
Diagnosis and treatment of alveolar proteinosis?
BAL with bronchoscopy find large amounts of PAS-positive material (must exclude PCP)
Tx: if your patient is not the lucky 10% that undergo spontaneous remission then lavage the whole lung with sterile saline
Young patient with no hx of smoking, environmental or drug allergy has a sudden onset of cough, dysnea, fever, tachypnea and rales. Radiographic "negative" of pulmonary edema present. Dx and treatment?
Acute eosinophilic pneumonia
Treat with steroids
What would show up on CXR in chronic eosinophilic pneumonia?
in addition to "negative" pattern of pulmonary edema
Loffler syndrome is the result of
intra-alveolar accumulation of eosionphils, macrophages and fluid from drugs and/or parasitic infection
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