pft and interstitial lung diseases
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PFT andInterstitial lung diseases
Presenter Dr.CHAKRADHAR
Physiology of Breathing • inspiration-it is an active process, due to
contraction of diaphragm ,intercostals intrathoracic volume increases resulting in fall of intrapleural pressure from -2.5mmhg to -6 mmhg .this stretches the lung resulting in its expansion and inflow of air
• expiration – quite expiration at rest is a passive process while forceful expiration is an active process during quite expiration increase in intra alveolar pressure is due to relaxation of diaphragm ,elastic recoil of lung tissue ,surface tension due to air fluid interaction in alveoli
• Lung properties that determine lung volumes and capacities
Static property compliance/ elastic recoil
Dynamic property airway resistance
Compliance described as dispensability of the lungIt is defined by amount of volume change per unit
change in pressure over pressure volume curvesIt depends on elastic property of lung tissue and
surface tension
Decreased compliance in fibrosis increased compliance in emphysema
Effect of elastic property on compliance
In normal lung compliance is more during expiration than in inspiration this difference is called hysteresis it occurs due to presence of surfactant-ganong
Normal lung
hysteresis
Effect of surface tension on compliance
1.2L
1L
2L
0.5L
3.5L2.5L
2.5L
5L
Volumes
Residual volume(RV)Volume of air remaining in the lungs after maximalexpiration
Expiratory reserve volume(ERV) Maximal volume of air expired from the restingend-expiratory level
Tidal volume(TV)Volume of air inspired or expired with each breath duringquiet breathing
Inspiratory reserve volume(IRV)Maximal volume of air inspired from the restingend-inspiratory level
CAPACITIES
Inspiratory capacity(IC)Maximal volume of air inspired from the end-expiratorylevel (the sum of IRV and TV)
Vital capacity(VC) Maximal volume of air expired form the maximalinspiratory level
Inspiratory vital capacity(IVC) Maximal volume of air inspired form the maximalexpiratory level
Functional residual capacity(FRC)Volume of air remaining in the lungs at the end-expiratorylevel (the sum of RV and ERV)
Total lung capacity(TLC)Volume of air in the lungs after maximal inspiration (thesum of all volume compartments)
• Change in lung volumes and capacities in various disease states
Dynamic property – It is resistance offered by the airways to air flowDepends on the net diameter of the airwaysDuring quiet mouth breathing, the mouth, pharynx, larynx, and trachea constitute 20 to 30 percent of the airway resistance;
Most of the remainder of airway resistance is in medium sizedlobar, segmental, and subsegmental bronchi
the small peripheral airways, particularly those less than2 mm in diameter, constitute only about 10 to 20 percent ofthe total airway resistance. because the net total diameter of them is very large compared to proximal airways
Airway resistance varies with inspiratory and expiratory phases of respiration because of presence of collapsible walls in airways
resistance is less during inspiration compared to expiration due to pull by surrounding parenchyma during its expansion caused by more negative intrapleural pressure during inspiration.so air way disease like asthma affects the airflow rate more during expiration
Air way resistance is also inversely proportional to lung volumes
Pulmonary function tests(PFT)
it includes following objective methods to asses the lung function
SpirometryBlood gasesLung volumes by helium dilution or body
plethismographyDiffusion capacity Bronchial challenge test
Spirometry measures the pattern of air movement into and out
of lungs during controlled ventilatory maneuvers
Expiratory flow ratesForced vital capacity(FVC) total volume of air that can be exhaled from TLC forced expiratory volume in 1st second(FEV1)- 75%-80% of FVC
Grading of obstruction basing on FEV1/FVC% >75% normal60%-75% mild obstruction50%-59% moderate obstruction<49% sever obstruction
Forced mid expiratory flow rate-FEF (25%-75%) • It is flow rate between 25% and 75% of FVC
indicated by slope of FVC volume time curve. expresed as lit/sec
• It corresponds to smaller airway dynamics
Interpretation of FEF(25%-75%) with respect to % of predicted value
>60% normal 40%-60% mild obstruction 20%-40% moderate obstruction <10% sever obstruction
Forced mid inspiratory flow-FIF(25%-75%)• It is calculated similar to FEF(25%-75%).• It is dependent on the respiratory effort and
function of respiratory muscles a drop in mid inspiratory flow is more sensitive
indicator of respiratory muslce dysfunction or suboptimal effort
Forced inspiratory volume-time curve.
Flow volume relationships it is studying the variation in air flow with change in
lung volume
Expiratory flow volume curves
Inspiratory flow volume curveDuring inspiration the diameter of the airway remains
same throughout the inspiration(due to stretch by surrounding expanding parenchyma) and the flow predominantly depends on pressure gradient across the airway. this results in grossly symmetrical curve
Normal Flow volume loop
Fixed central air way obstruction eg trachial scarring after tracheotomy in this the obstruction remains same through
out respiratory cycles so both expiratory and inspiratory flows are equally affected
volume
Variable obstruction in this the degree of obstruction varies in different
phases of respiration the phase of respiratory cycle affected
depends upon the location of obstruction in tracheobronchial tree
variable extrathoracic(tracheomalacia) – inspiratory phase is affected
During expiration tracheal pressure is more than atmospheric pressure so it remains open during expiration resulting in normal expiratory flow
airfl
ow
variable intrathoracic obstuction obstruction is less during inspiratory phase as
airways remains patent during inspiratory phase due to pull by surrounding parenchyma
volume
airfl
ow
• Flow volume loop in obstructive diseases airway obstructive diseases-asthma/COPD both
expiratory and inspiratory flows are affected with relative preservation of expiratory flow pattern
volume
airfl
ow
• Flow volume loops in restrictive lung diseasesIn this the normal configuration of both expiratory
and inspiratory limbs are preserved but the peak flow and volumes are reduced
Diffusion capacity it is a measure of amount of gas exchanged across
the alveolocapillary membrane(interstitium)
CO gas is used because itdoes not reach equilibrium b/w alveoli and pulmonary capillary blood in a cardiac cycle duration(0.75sec), as it has high affinity to hemoglobin that prevents the Pco in capillaries to raise ,i.e CO exchange is diffusion-limited
Diffusion capacity of CO (DLco) it is dependent on the surface area available for diffusion thickness of interstitium haemoglobin in capillariesNormal DLco at rest is 25ml/min/mm hg it is decreased in pulmonary fibrosis(ILDs)-increas intertial thickness emphysema-decreases surface area pulmonary hypertension-decreases pulmonary flow anemia
FEV1/VC≥ LLN
VC ≥ LLN VC ≥ LLN
TLC ≥ LLNTLC ≥ LLN
Obstruction Mixeddefect
DL,co ≥ LLN DL,co ≥ LLN
DL,co ≥ LLN
NormalPVdisorders
CW and NMdisorders
ILDPneumonitis
AsthmaCB
Emphysema
yes
yes
yes
no
no
restriction
no
no
no
no
yesyes
no no
yesyes
Breath-Holding test of Sebrasez
Match Blowing Test
Valsalva Test
Single Breath Count
Ascultation over Trachea
Cough test
Bed side pulmonary function tests
Interstitial lung diseases (ILDs)
ILDS are defined as- collection of non neoplastic lung disorders both acute
and chronic ,that are not caused by known infectious agents presenting with variable degrees of inflammation and fibrosis-fishman
ILDs are also termed as diffuse parenchymal lung diseases(DPLD)
They predominantly involve interstitium of the lung (alveolar epithelium , pulmonary capillaries with its endothelium , stroma in between them along with lymphatics) but airways may also get involved occasionally
>200 known individual diseases involving pulmonary interstitium are present, these diseases can be classified together because of clinical , pathological and radiological similarities
When to suspect ILDs in all patients who present with constitutional symptoms of fever ,weight loss ,
asthenia and respiratory symptoms of cough ,dyspnea ,chest pain should be evaluated for known infectious/other hypersensitive respiratory disorders
when the features of respiratory involvement are progressive /relapsing and remitting type that do not respond to usual management than ILDs are suspected only after evaluating for other treatable causes
classification of ILDs
In to two major groups based on underlying histopathology
interstital FIBROSIS/ GRANULOMAEach major group into subgroups based on
etiological factor KNOWN cause/UNKNOWN cause
ILDsIntertial fibrosis
Known causeunknown cause
1.asbestos
2.fumes,gases3.drugs
4.radiation
5.smoking
a.Desquamative interstitial pneumoniab.respiratory bronchiolitis associated ildc.Langerhans cell granulomatosis
Granulomas in interstitium/vasculature
Intertial fibrosis with unknown cause1.Ideopathic interstitial pneumonia
a.Idiopathic pulmonary fibrosis UIP
b.Acute interstitial pneumonia AIPc.Cryptogenic organizing pneumonia BOOP
d.Non specific interstitial pneumonias
2.ILDs associated with connective tissue disordersSLE, RA, ankylosing spondylitis systemicsclerosis ,sjogrens ,polymyositis ,dermatomyositis
3.Pulmonaryhemorrhage syndromesGoodpastures,ideopathic pulmonary hemosiderosis, isolatedpulmonary capillaritis
4.Inherited diseasesTuberous sclerosis,NF,niemann pick,hermansky pudlak
5.gastointestinal/liver diseasesCrons,ulcerative colitis,primary biiar cirrosis,chronic active hepatitis6.Pulmonary alveolar proteinosis7.Eosinophilic pneumonias8.Lymphangio leomyomatosis9.amyloidosis
Granulomas in interstitium/vasculature
Known cause
unknown cause1.Hypersensitivity pnemonitis
Organic dusts Inorganic dustsBeryllium,silica
1.sarcoidosis
2.Granulomatous vasculitidisa.wegnersb.churg-straussc.Benign lymphocytic angitis
d.Necrotising sarcoid granulomatosis
3.Lymphomatoid granulomatosis4.Bronchocentric granulomatosis
Pathogenesis following initial injury to airway/alveoli either due
due to autoimmune cause/known antigen-organic or inorgnaic surce ,drugs ,toxins in tobbaco
inflamatory cell infiltration ,exudatioin ,cytokine release causing metabolic and other functional abnormalities in alveolar interstitial components
If the insult continues to persist leading to chronicity
Lymphocyte infiltration progressive depositionEpitheloid cell formation of collagen leading to Surrounding areas of focal interstitial fibrosis
fibrosis and honeycombing
(GRANULOMA) formation
Most common ILDs 1. ideopathic interstitial pneumonias 40% a.ideopathic pulmonary fibrosis 55% 2.occupational and environmental 26 % Least common ILDs pulmonary haemorrhage syndromes <1%
Retrospective study of interstitial lung disease in a tertiary care centre in India.indian journal of chest diseases and allied sciences
Idiopathic pulmonary fibrosis (43%), sarcoidosis (22%), ILDs secondary to collagen vascular disease (19%)
and extrinsic allergic alveolitis, among others, were the
most common aetiological causes of ILD.
Clinical presentation
symptoms dyspnea cough-dry hemoptysis-ILDs associated with CTD pulmonary hemorrhagic syndromes chest pain/pleurisy fever weight loss Features of CTD- skin rash, raynauds phenomenon,
. arthritis , proximal muscle weakness sinusitis-wegeners granulomatosis
age at presentation most of ILDS present at 20-40 yrs 1.ILDs associated with connective tisssue disorders 2.ILDs with inherited causes- familial IPF gauchers hermansky-pudlak 3.lymphangioleiomyomatosis 4.langerhans cell granulomatosis
ILDs that manifest during infancy and childhood 1.follicular bronchitis 2.cellular interstitial pneumonia 3.acute idiopathic pulmonary hemorrhage of
infancy idiopathic pulmonary fibrosis (IPF) manifests after 60 yrs
Duration of symptomsAcute onset: days to weeks• Acute interstitial pneumonia• Acute pneumonitis from collagen vascular disease(especially SLE)• Cryptogenic organizing pneumonia• Drugs• Diffuse alveolar hemorrhage• Eosinophilic lung disease• Hypersensitivity pneumonitis
Subacute: weeks to months• Collagen vascular disease–associated ILD• Cryptogenic organizing pneumonia• Drugs• Subacute hypersensitivity pneumonitis
Chronic: months to years• Chronic hypersensitivity pneumonitis• Collagen vascular disease–associated ILD• Idiopathic pulmonary fibrosis• Nonspecific interstitial pneumonia• Occupation-related lung disease (e.g., silicosis,• asbestosis)
Gender variationCommon in females 1.ILDs associated with connictive tissue diseases
except in RA 2.lymphangioleomyomatosis 3.ILDs in tuberous sclerosis,hermansky pudlackCommon in males 1. ideopathic pulmonary fibrosis 2.pneumoconiosis
exposure history Hypersensitivity pneumonitis • Bird breeders• Farmers• Bark strippers/wood workersInorganic dustAsbestosis• Automotive mechanics , Electrician , Pipefitters ,
Shipyard workersberylliosis• Electronic and computer,industry workersCoal workers pneumoconiosissilicosis
Certain ILDs are also associated with smoking and drug intake so possibility of such association should be considered in history
In a patient with ILDS Family history of connective tissue disorders , sarcoidosis ,tuberous sclerosis should also be enquired
Signs on examination usually these are non specific tachypnea cyanosis clubbing velcro rales inspiratory squeaks- cryptogenic organising pneumo constrictive bronchiolitis hypersensitivity pneumonitis exta pulmonary signs in systemic diseases with lung involvement
Investigations 1.hematologicl/serological they provide supportive evidence CBC- eosionphilia , LFT , RFT, serum calcium , serum ACE, complement levels serum autoantibody- ANA ,anti dsDNA ,anti sm RF ,anti Jo ,p/c ANCA
radiological investigations Xray is basic – usually the findings are nonspecific it
may show reticular/nodular/mixed opacitis with varied distribution depending upon the disease
HRCT is gold standard for imaging as it reveals characteristic findings that are highly suggestive of diagnosis
Pulmonary function tests spirometry- most of the ILDs produce restrictive
pattern with - reduced TLC , FVC ,FRC ,RV normal/increased FEV1/FVC restrictive+obstructive pattern seen in lymphangioleomyomatosis sarcoidosis langerhans cell histiocytosis constirctive bronchiolits hypersensitivity pneumonia Spirometric values are proved to have prognostic
value in IPF, non specific interstitial pneumonia
Diffusion capacity most ILDs have reduction in CO diffusion.it is
attributed to- V/Q mismatch effacement of alveolar capillary unitsThe severity in reduction of diffusion capacity of lungs
does not correlate with disease stageCardio pulmonary exercise testing(6 minute walk test) these tests are used to identify the subtle
abnormalities in gas exchange in ILD patients who have normal gas exchange at rest
Bronchoalveolar lavage(BAL) it is of diagnostic importance in 1.sarcoidosis - lymphocytosis, CD4/CD8 >3.5 2.pulmonary alveolar proteinosis- Milky BAL fluid
with debris and foamy macrophages3.eosinophilic pneumonias-eosinophils >25%4.Pulmonar langerhans cell histiocytosis- CD1+
langerhans cells,birbeck granules in macrophages5.berylliosis- positive lymphocyte transformation test
to beryllium6.asbestosis-dust particles ferruginous bodies
tissue biopsyIt is the most effective method of confirming the
diagnosis and acessing the disease activityMultiple transbronchial biopsies are more sensitive in
sarcoidosis , hypersensitive pneumonitis , organising pneumonia ,respiratory bronchiolitis associated ILDs.
if transbronchial biopsy does not suggest specific diagnosis lung biopsy by thoracoscopy or thoracotomy is necessary
Lung biopsy is contraindicated in end stage of disease,elderly ,cardiovascular compromise
Idiopathic pulmonary fibrosis(IPF)It is a specific form of fibrosis confined to lung with
characteristic radiological and histological appearance
Risk factors- age family history of IPF smoking clinical features Age >50 y chronic symptoms-dyspnea,cough Velcro rales, clubbingHRCT: Peripheral, subpleural,basal Reticular opacities. Honeycombing. Traction bronchiectasis
Idiopathic pulmonary fibrosis. This high resolutioncomputed tomogram of the chest demonstrates classic bilateralhoneycombing.
Bilateral, peripheral, and subpleural honeycomb changes (arrowhead) and traction bronchiectasis (arrow).
Histology- a. patchy interstitial infalmmation with lymphoplasmacytic infiltration. b. cystic fibrotic air spaces lined by
bronchiolar epithelium and filled with mucin
honeycomb change (arrowheads) present in the regionof dense fibrosis adjacent to the pleural surface. A fibroblast focus(arrow) is seen at the leading edge of advancing fibrosis.
Major criteria• Exclusion of other known causes of ILD, such as certaindrug toxicities, environmental exposures, and connective
tissue diseases• Abnormal pulmonary function studies that include
evidence of restriction (reduced VC often with an increased FEV1/FVC ratio) and impaired gas exchange [increased AaPO2 with rest or exercise or decreased DlCO]
• Bibasilar reticular abnormalities with minimalground glass opacities on HRCT scans
• Transbronchial lung biopsy or bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis
Minor criteria• Age greater than 50 years• Insidious onset of otherwise unexplained dyspnea
on exertion• Duration of illness greater than or equal to 3
months• Bibasilar, inspiratory crackles (dry or “Velcro” type
in quality) IPF is considered “likely” if the patient is an immunocompetent adult and all 4 major criteria
+ 3 minor criteria
TreatmentIt is not indicated for all IPF PatientsPatients whose clinical features suggestive of more
favourable outcome will have more benefit from therapy
Oxygen supplement for patients with hypoxemia at rest SPo2 <88%
Drugs –immunosuppressant's-
corticosteroids ,azathioprine ,cyclophosphamide ,interferon γ1b
Antioxidants-N acetyl cysteine
Criteria for lung transplantation• Pathologic or radiographic evidence of IPF and any of the following criteria
• DLCO <39%• Decrement in FVC 10% during 6 months of follow-
up• Decrease in SpO2 below 88% during a 6-min walk
test• Honeycombing on HRCT
• Secondary causes of IPF-pneumoconiosis ,radiation injury ,chronic aspiration ,organized chronic eosinophilic pneumonia
Nonspecific interstitial pneumonia It is a subacute process affects young women, non smokers HRCT Peripheral, subpleural,basal, symmetric Ground-
glass,opacities with Lower lobe volume loss. honey combing is rare
Peripheral ground glass opacities with tractional bronchiectasis
Histology
Treatment Immunosuppressents –glucocorticoids, azathioprine
uniform interstitial involvement with cellular infiltration or fibrosing pattern little or no honeycombing is found.
majority of patients with NSIP have a good prognosis (5-year mortality rate estimated at <15%
There is a lymphoplasmacytic infiltration of the interstitium
Acute interstitial pneumonia(hamman rich syndrome)
considered as idiopathic cause of ARDSpresents asAbrupt on set of fever ,cough ,dyspnea after a
prodromal illness
HRCT b/l symmetrical patchy ground glass opacities in subpleural distribution
Histology leucocyte infiltration interstitial edema which
in later stages shows interstitial fibrosis
Diagnosis of AIP requires in clinical presentation like ARDS and pathological conformation of diffuse organising alveolar damage
Treatment as most have hypoxemia with respiratory failure
mechanical ventilation with supportive care is required. Benefit of glucocorticoid usage is unknown
Mortality rate is about >60% in first 6 months
cryptogenic organising pneumonia(COP)Clinical features presents during 40-50 yrs, common in non
smokers .has subacute course of fever, cough and other constitutional features with inspiratory crackles on examination
HRCT Reverse halo sign/attol sign it is central area ground glass opacity (distal air
way infiltration)surrounded by a more dense crescent or ring shaped shadow(granulation tissue in wall of air ways
Histology collagen deposition and narrowing of alveolar ducts.
formation of granulation tissue polyps(masson bodies)consisting of macrophages and fibrous tissue in small airways alveolar ducts,air spaces
Masson body
Treatment immunosupressents like glucocoricoidsIt induces remession in about 2/3rds but rest may
progress despite the therapy
Pulmonary langerhans cell histiocytosis(PLCH) langerhans cell is a reticuloendothelial cell derived
from macrophagePLCH is characterised by langerhans cell infiltration Etiological factor-smokingIt is also associated with malignancies like NHL,HL Presentation –young male (20ysr-40 yrs) chronic smoker nonproductive cough ,dyspnea ,weight
loss ,chest pain
Xray –illdefined nodular(2-10mm) ,reticular opacities with cysts in middle to upper zones and sparing of costphrenic angles
ill-defined nodules, reticulonodular opacities,cysts, costophrenic angle sparing, and preservation of lung volumes.
HRCT-combination of thin walled cysts with nodules
PFT-decreased DLco with restrictive/obsrtuctive patterns
Tissue biopsy poorly defined bronchiolocentric nodular sclerosing
lesions containing clusters of langerhans cells .langerhans cells show birbeck granules
central bronchiole ( ) is dilated, and adjacent ∗alveolar spaces have coalesced because of focal destruction of alveolar walls
Electron micrograph of Langerhans’ cell (Lg) of the lung. Typical X bodies (Birbeck granules)
ComplicationsPneumothraxHemoptysisPulmonary hypertension Treatment Stopping of smoking Corticosteroid therapy is less usefulAnti TNF α has evidence of improvement
Lymphocytic interstitial pneumonitis(LIP)Common in adult(>50yrs) femalesusually associated with CTDPresents with nonspecific respiratory featuresAbout 20% has salivary gland enlargementXRAY-b/l lower zone reticular opacitiesHRCT –lower lobe ground glass opacities with
centilobular/subpleural pulmonary nodules
Ground glass changes with pulmonary nodules
HistologyCharacterised by lymphocytic infiltration , germinal Center formation
within the interstitium
Treatment Corticosteroidsabout 30% can progress to end stage fibrosis
Pulmonary lymphangioleiomyomatosis affects young women, common in whitesCharacterised by atypical proliferation of pulmonary
inerstitial smooth muscle and cyst formationClinical features dyspnea ,cough ,chest pain accelarated during pregnency, abates after
oophorectomyIt should be suspected in young woumen with
emphysemaComplications-
hemoptysis ,pneumothorax ,chylothorax
PFT- obstructive/ mixed pattern
multiple thin-walled cystic airspaces.Surrounded by normal lung Pulmonary lymphangioleiomyomatosis causes
thin-walled emphysematous spaces leading to the distinctive type of honeycombing.
There is no proven beneficial therapyProgesteron and luteinizing hormone analogues are
triedLung transplant is final consideration
Sarcoidosis a multisystem disease characterised by formation of
noncaseating granulomas Prevalence 20-60 per 1lakh population Common in females >40yrs,HLA DRB1*1101 smoking is associated with decreased riskClinical manifestations • Pulmonary (>90%) dyspnea,cough• Upper respiratory tract and oral cavity(5–10%)Hoarseness, laryngeal or tracheal obstruction, nasal
congestion, sinusitis
• Ocular (20–30%) - uveitis,retinitis, conjunctivitis, optic neuritis
• Skin (20–30%)- Erythema nodosum, lupus pernio, alopecia
• Hematologic (20–30%)- lymphadenopathy , splenomegaly , hypersplenism, anemia , lymphopenia
• Endocrine (10–30%)- Hypercalciuria, hypercalcemia, hypopituitarism, diabetes insipidus
• Neurologic (5–10%) -Facial and other cranial neuropathies, aseptic meningitis, seizures, obstructing hydrocephalus, myelopathy, polyneuropathy
• Cardiac (5–10%)- Arrhythmias, heart block, cardiomyopathy, sudden death
• Exocrine gland (10–20%)- lacrimal, and parotid gland enlargement, sicca syndrome
Liver,joints,muscles,renal and genitourinary systems are also involved
Investigations
Hematological –hypercalcemia ,raised ESR,hyper globulenima
Raised ACE levels in 60%-80%and is non specific
Kveim test-part of spleen of sarcoid patient injected intradermally and observed for granuloma formation after 4-6weeks
PFT restrictive pattern common than obstructive decreased diffusion capacity of CO
Radiological –Xray,HRCT staging
stage 1 hilar lymphadenopathy assosiated with right paratracheal adenopathy
stage 2 hilar lymphadenopathy+ pulmonar infiltrates
stage 3 pulmonry infiltrates alone
stage 4 fibrocystic changes
• BAL findings CD4/CD8 >3.5 to 4 supports a diagnosisTissue biopsy biopsy from easiest accessible site is used to confirm
a diagnosis of Biopsy of a skin or conjunctival nodule, enlarged
superficial lymphnode, or lacrimal gland will be sufficient if clinically involved if these sites are not involved trans bronchial biopsy(TBB) is needed
lungbiopsy showing extensive parenchymal involvement with granulomas, multinucleated giant cells, and mononuclearcell inflammation
Granuloma in lymphnode
Asteroid bodies
Diagnosis of sarcoidosis requires presence of clinical/radiological features suggestive of sarcoidosis with tissue biopsy showing noncasiating granulomas
Treatment Immunosuppressents - steroids azathioprine ,methotrexate hydroxychloroquin infliximab
EOSINOPHILIC PNEUMONIAIt includes disorders with eosinophilic pulmonary
infiltration with peripheral blood eosinophiliaIt is considered to be immunologically mediated with
varied precipitating eventsKnown causes unknown causesAllergic bronchopulmonary loefflers mycosisParasitic acute eosinophilic pneDrugs chronic eosinophilic pneEosinophilia myalgia syn hypereosinophilic syndr
Allergic bronchopulmonary mycosisIt is due to hypersensitive reaction to antigen of
inhaled fungi
Aspergillus fumigatus(mc)-ABPAPencillumCandidacurvularia ABPAIt usually presents as recurrent episodes of
cough,thick brown mucous ,dyspnoea ,wheez in a known asthmatic
• Diagnostic criteria of ABPA
Major
AsthmaPositive immediate hypersensitivity skin-prick test to AspergillusPrecipitating antibodies against AspergillusElevated total IgEElevated serum Aspergillus–specific IgE, IgGHistory of pulmonary infiltratesPeripheral blood eosinophilia+/− Proximal bronchiectasis
MinorMucous plugs containing AspergillusDual cutaneous reaction to Aspergillus
The imaging findings in ABPA• Tansient migratory pulmonary infiltrates usually in
upperlobes • Central bronchectas with mucoid impaction in
dilated bronchi appear as glove in finger opacity
ABPA. Extensive infiltrates withtubular configuration and ‘‘gloved finger” appearance are present, predominantly in the lower lobes
. Central bronchiectasisa patient with ABPA
• Basing on clinical ,radiological ,serological characters 5 clinical stages are recognised
Stage I: AcuteAcute asthma symptomsElevated serum IgE (>1000 IU/ml)Peripheral blood eosinophilia (may be absent in patients treated with oral coricosteroids)Fleeting infiltrates on chest x-ray (may be absent in patients treated with oral coricosteroids)Positive specific IgE, IgG, skin test reactivity, and precipitins to A. fumigatusResponds to steroids/antifungal therapy
Stage II: RemissionResolution of symptomsResolution of pulmonary infiltratesImprovement in eosinophilia and A. fumigatus specificblood abnormalities
Stage III: Exacerbation/recurrenceRecurrence/worsening of clinical symptomsRecurrent pulmonary infiltratesRising IgE levels
Stage IV: Steroid-dependent-asthmaRefractory steriod-dependent asthmaPersistently elevated serum IgE levelsPersistently elevated A. fumigatus–specific bloodabnormalities
Stage V: Fibrotic lung diseaseRefractory steriod-dependent asthmaFibrotic lung disease (irreversible obstructive andrestrictive defects with impaired diffusing capacity)Chronic bronchiectasis symptoms (sputumproduction, frequent infections)
Treatment of ABPAAcute stage- systemic glucocorticoid will reduce the
severity of symptoms duration of maintainence therapy is according to
stage early initation of therapy proved to reduce
progression to fibrosis
• Tropical pulmonary eosinophila this is due to hypersensitivity to antigens ofparasites
as Ascaris ,ankylostoma ,strongyloides ,toxocara ,Usually manifests as cough ,dyspnea , wheezing hyper infection syndrome results due to sever
autoinfection of parasites usually in immuno compromised patients presentation may be as ARDS
TREATMENT antihelminthic supportive measures
Drug induced eosinophilic pneumonia nitro furantoin penicillin sulfonamides isoniazid thiazides Tricyclic anti depressants indomethacin
hypersensitivity pneumonitis an ILD caused due to repeated inhalation of organic
agents by a susceptible host
A large number of agents are associated,presently common are
farmer’s lung disease, bird fancier’s disease, ventilator lung, Japanese summer-type HP
Disease Source AntigenFarmer’s lung disease Moldy hay
Thermophilic actinomycetesT. vulgarisAspergillus spp.
Bagassosis Moldy pressed sugarcane (bagasse
Thermophilic actinomycetesT. sacchariT. vulgaris
Bird fancier’s lung Domestic and wild bird products
Bird proteins
Ventilator lung
Contaminated humidifiers,dehumidifiers, air conditioners,heating systemsThermophilic
Thermophilic actinomycetes, T. candidus,T. vulgaris, Penicillium spp.,Cephalosporium spp.Amoebae Klebsiella spp.Candida spp.Basement lung Contaminated basement (sewage ormold)Cephalosporium spp.
Disease Source Antigen
Japanese summer house HP House dust, bird droppings(?)
Trichosporon cutaneum
Wood dust HP Contaminated wood dustBacillus subtilisAlternaria
Greenhouse lung Greenhouse soilAspergillus spp., Penicillium spp.,Cryptostroma corticale
Shell lung Oyster or mollusk shell Shell proteins
Basement lung Contaminated basement (sewage ormold)
Cephalosporium spp.Penicillium spp.
Pathophysiology susceptible host on repeated and prolonged
exposure to an antigen gets sensitised resulting in hypersensitivity reaction usually type 4 involving lung and formation of granulomas
Clinical features acute/subacute form – 6-8 hrs after exposure to
antigen cough , dyspnoea ,malaise ,fever with fine crackles
these features disappear in days to months if further exposure is avoided in few it can progress to chronic form with gradual worsening of cough ,dyspnoea
Investigations Raised ESR, CRP ,serum precipitins to suspected
antigenX RAY acute/subacute form –poorly defined/discrete
patchy/diffuse nodular infiltrates usually apical sparing
bilateral lower lobe 2- to 3-mmnodules.
Chronic stage-diffuse reticulonodular infiltrates with eventual honycombing
• HRCT ground-glass densities and diffusely increased pulmonary radiodensities.
patient with exposure to both birds and shells . Note the diffuse nodular lesions in the lower lobes, with areas of groundglassdensities posteriorly.
• PFT restrictive or obstructive or mixed pattern decreased diffusion capacityBAL – increased lymphocytic countLung biopsy in active stage- inflammatory cell infiltration with
granulomas in interstitium
lung biopsy from a patient with bird fancier’s disease.There is nonspecific interstitial mononuclear inflammation and loosely formed granulomatous lesions.
• Chronic stage –non specific interstitial and peribronchiolar fibrosis with or without granulomas
Treatment environmental control to reduce antigen exposure , glucocorticoids and supportive measures
• ILD associated with occupational inorganic dust exposure
AsbestosisAsbestos is a fibrous hydrated magnesium silicate
with more than 3000 commercial usesIt has 2 forms serpentine(chrysolite) commonly used
. amphibole less usedExposure occurs in cement industry,asbestos mining
Asbestosis refers to interstitial diseaseOther pulmonar manifestations arePleural plaque ,Benign pleural effusionMesotheliomasBronchogenic carcinoma
Asbestosis usally results after 20-30 yrs of exposureAnd presents as progressive cough and dyspnoea
XRAY in asbestosis b/l diffuse lowerlobe reticulonodular opacitie
bilateral calcified pleural plaques on top of the diaphragm. costophrenic angles are blunted. Mediastinalpleural calcification is present on the right.
• BAL –ferruginous bodies asbestos fiber surrounded by iron rich material
TreatmentStopping further exposure,avoiding smoking and other supportive measures
Silicosis exposure in stone quarries, sand blasting ,quartz
industriesPresents with nonspecific symptoms of
cough ,dyspnoea ,fatigue usually after several years of exposure
Acute silicosis symptoms occurs in few weeks to months after heavy exposure to dust with high quartz content
Silicotuberculosis –high prevalence of TB in silicosis
• XRAY small rounded opacities in upper zones ,calcified hilar lymphnodes appear as egg shell calcification
Anthracosis coal workers pneumoconiosis occurs due to
tissue reaction to inhaled coal dust Presents as nonspecific symptoms of cough with expectorant containing black particlesDyspnoea ,wheeze due to bronchiolitisIn advanced stages progressive massive fibrosis can
occurCaplans syndrome rheumatoid arhtritis with
pneumoconiosis (antracosis,silicosis)
• XRAY small rounded nodules in upper zones in case of simple CWP
In later stages larger nodules that progress to PMF
Bilateral masses of progressive massivefibrosis are seen.
ILDs associated with connective tissue disorders
There is varied response to immunological damage of lung tissue in various CTDs
1.Diffuse alveolar damage(DAD)Seen in acute pneumonia in SLE Polymyositis/dermatomyositis
Interstitial involvement is a common respiratory manifestation of the collagen vascular disorders
Nonspecific interstitial pneumonitis (NSIP)
seen in patients with rheumatoid arthritis, polymyositis/dermatomyositis, mixed connective-tissue disease, and scleroderma.
•Lymphocytic interstitial pneumonitis
Seen in primary Sj¨ogren’s syndrome secondary Sj¨ogren’s syndrome, associated with rheumatoid arthritis.
Seen in –rheumatoid arthritis SLE systemic sclerosis
Idiopathic pulmonary fibrosis
Bronchiolitis obliterans organizing pneumonia(BOOP)
Seen in - rheumatoid arthritis polymyositis/dermatomyositis
Parenchymal nodules
Noninfectious inflammatory parenchymal nodules occur in rheumatoid arthritis Sj¨ogren’s syndrome.
In rheumatoid arthritis they are called as necrobiotic or rheumatoid nodules. Seen in both pleura and lung parenchyma identical in appearance to a subcutaneous rheumatoid nodule.
In the lung parenchyma, these nodules are located in the interlobular septa and in the subpleural parenchyma.
These nodules comprise of histocytes, giant cells, and other mononuclear cells surrounding an area of fibrinoid debris
Necrobiotic rheumatoid nodule. There is a central area of fibrinoid debris surrounded by palisading histiocytes.
ILDs with diffuse alveolar hemorrhageIt includes good pastures syndrome ,ideopathic
pulmonary hemosiderosis other auto immune diseases that involve pulmonary capillaries
it is characterised by intertitial infiltration of inflamatory cells with capillarities,fibrinoid necrosis of interstitium , bleed in alveoli
Presentation-acute onset of cough, dyspnea ,fever ,hypoxemia ,hemoptysis ,impared renal function
BAL-hemorrhagic fluid
Xray-non specific diffuse alveolar opacities
diffuse bilateral alveolar infiltrates representing massive alveolar hemorrhage.
Lung/renal biopsy with immunoflurosence –specific pattern of involvement
linear deposition-good pastures granular deposition –HSP
Treatment-methyl prednisolone/plasmapheresis
large numbers of red blood cells within alveolar spaces in a patient with alveolar hemorrhage due to Wegener’s granulomatosis
Pulmonary alveolar protenosis(PAP)It is not a true ILD as there is no lung inflamation and
the architecture is well preserved.as it resembles ILD so is discussed here
Surfactent it is produced by type 2 pneumocytesIt is a mixture of
dipalmitoylphosphatidylcholine ,other lipids and proteins
The surfactant proteins help to maintain the phospholipid layer
• Surfactant proteinsSP-A it has regulatory function SP-BSP-CSP-D maintains other proteins in lipidlayer PAPIt occurs due to defective clearance of surfactant by
alveolar macrophages
They are key proteins for surfactant action
3 causes of PAP>90% is acquired –antibody to GM-CSFCongenital -due to mutation in SPB proteinSecondary-llysinuric pretein intoleranceAcquired varient occurs at 30yrs-50yrsClinical features dyspnea cough occasionally with gelatinous expectoration fatigue ,weight loss
InvestigationsBlood/serum - polycythemia ,increasd SP-A ,SP-
B ,anti GM-CSF antibodyBAL -anti GM-CSF antibody
b/l central middle and lower zone opacities-batwing appearance
ground-glass opacification with interlobular thickening.
ILDs associated with Smoking 1.desquamative interstitial pneumonia 2.respiratory bronchiolitis associated pneumonia 3.langerhans cell granulomatosis/oesinophilic granuloma ILDs associated with drugs
ACE inhibetros - eosinophilic pneumonia
β blockers- pulmonary fibrosis acute hypersensitivity pneumonitis organising pneumonias eosinophilic/infiltrative pneumonias
Amiodarone- pulmonary fibrosis pulmonary nodules organising pneumonias oesinophilic pneumoniasBleomycin- pulmonary fibrosis pulmonary nodules organising pneumoniasCarbamazepine- organising pneumonia acute hypersensitivity pneumonitis diffuse alveolar damage alveoler hemorrhage
Other drugs-cyclophosphamide , phenyton , pencillamine , propyl thiouracil,
ReferencesFishman 4th editionGanong 24th editionHarrison 18nth edition
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