DR.OLA FAROUK RAMMAH Medical Specialist, Meeqat General Hospital. Madinah,KSA

1. UPDATES IN INTERSTITIALLUNG DISEASE
DR OLA FAROUK RAMMAH
Meeqat General Hospital,Madinah.KSA

2. DEFINITION
Encompasses a group of disorders of diverse aetiologies
with common features of varying degrees of fibrosis and
inflammation of the lung parenchyma orinterstitium.
It includes over 200 different diseases which, in spite of their
heterogenous nature have several common clinical,
radiological and histological manifestations.

3. “INTERSTITIAL”– AMISNOMER
•The interstitium of the lung spans the region between the alveolar
epithelial and capillary endothelial basementmembranes.
•This region includes a variety of cell types (fibroblasts, myofibroblasts,
and macrophages) and matrix components (collagens, elastin, and
proteoglycans).
alveolar spaceproximal tothe•Theinterstitiumextendsfromthe
terminal and respiratory bronchioles.
Thisgroup of pulmonary disorders frequently involves:
•alveolarepithelium,alveolarspace,pulmonarymicrovasculature,
respiratory bronchioles, larger airways & pleura

4. ILDshave been difficult to classify because>200 known
individual diseasesare characterized by diffuseparenchymal
lung involvement, either asthe primary condition or asa
significant part of amultiorgan process, asmay occur in the
connective tissue diseases(CTDs).
ILDsclassified into two groupsbasedon the majorunderlying
histopathology:
.1thoseassociatedwith predominant inflammationand
fibrosis and
.2those with apredominantly granulomatousreactionin
interstitial or vascularareas
•Eachof thesegroupscanbefurther clinicallysubdivided
according to known or unknownetiology

7. EPIDEMIOLOGY
ILDs wasconsidered to be rare in thepast.
Increasedawareness& wider availability of HRCTandother
diagnostic tests have led to increasedrecognition.
ILDsconstitute about 10%to 15%of the patients withrespiratory
diseases,seenat any large hospital in thecountry.
About 50%ofthe ILDsare idiopathic in origin while others are
associated with identifiable diseases, most commonly, aCTD.
UIP,also known asIPFis the most common form of IIP.

8. PATHOGENESIS
ILDsarenon-malignant disorders andarenot causedbyidentified
infectious agents.
The precise pathway(s) leading from injury to fibrosis is not known.
Although there are multiple initiating agent(s) of injury, the
immunopathogenic responses of lung tissue are limited, &the
mechanisms of repair have commonfeatures
Thetwo major histopathologicpatterns
inflammation andfibrosis
granulomatous

10. INFLAMMATION& FIBROSIS
Theinitial insult is aninjury to the epithelial surfacecausinginflammation in the
air spacesand alveolar walls.
If the diseasebecomeschronic, inflammation spreadstoadjacent portions of
the interstitium and vasculature and eventually causesinterstitial fibrosis.
Important histopathologic patterns found in the ILDsinclude UIP,NSIP,
respiratory bronchiolitis, organizing pneumonia (BOOP),diffuse alveolar damage
(acute or organizing), DIP,& lymphocytic interstitial pneumonia.
Thedevelopment of irreversible scarring (fibrosis)of alveolar walls, airways,or
vasculature is the most feared outcome in all of these conditions becauseit is
often progressive and leads to significant derangement of ventilatory function
and gasexchange.

11. CELLULARBASESFORTHEPATHOGENESIS

12. EPITHELIAL DAMAGE & ACTIVATION
•Multiple microinjuries damage and activate alveolar
epithelial cells, which in turn induce an antifibrinolytic
environment in the alveolar spaces, enhancing wound
clot formation.

13. FIBROBLAST MIGRATION & PROLIFERATION
•Alveolar epithelial cells secrete growth factors and
induce migration and proliferation of fibroblasts and
differentiation into myofibroblasts

14. ANGIOGENISIS & MYOFIBROBLAST FOCI FORMATION
•Subepithelial myofibroblasts and alveolar
epithelial cells produce gelatinases that may
increase basement membrane disruption and
allow fibroblast–myofibroblast migration.
•Angiogenic factors induce neovascularization.

15. FIBROSIS& IMPAIRED REEPITHELIAZATION
•Both intraalveolar and
interstitial myofibroblasts
secrete extracellular matrix
proteins, mainly collagens.
•An imbalance between
interstitial collagenases and
tissue inhibitors of
metalloproteinases provokes
the progressive deposit of
extracellular matrix.
•Myofibroblasts produces angiotensin IIprovoking
alveolar epithelial cell death, further impairing
reepithelialization.

17. GRANULOMATOUSLUNGDISEASE
Characterized by an accumulation of T-lymphocytes, macrophages
and epithelioid cells organized into discrete structures
(granulomas) in the lungparenchyma.
Thegranulomatous lesions can progress to fibrosis.
Many patients with granulomatous lung disease remain free of
severe impairment of lung function, or when symptomatic, they
improve after treatment.
DDx- sarcoidosis and hypersensitivity pneumonitis

18. DIAGNOSING ILD
Aclinical diagnosisispossible for manyforms of ILD,especiallyif
an occupational and environmental history is aggressively
pursued.
For other forms, tissue examination, usually, obtained by
thoracoscopic lung biopsy, is critical to confirmation of the
diagnosis.
HRCTscanning improves diagnostic accuracy asexperience with
histologic-image correlation isperfected.

19. SIGNS& SYMPTOMS
Dyspnoea is a common and prominent complaint in patients with
ILD, especially the idiopathic interstitial pneumonias,
hypersensitivity pneumonitis, COP,sarcoidosis & eosinophilic
pneumonias.
Some patients, especially patients with sarcoidosis, silicosis,
hypersensitivity pneumonitis, lipoid pneumonia, or lymphangitis
carcinomatosis, may have extensive parenchymal lung disease on
chest x-ray without significant dyspnoea, especially early in the
course of the illness.
Wheezing is an uncommon manifestation of ILD but has been
described in patients with chronic eosinophilic pneumonia,

20. Clinically significant chest pain is uncommon in most ILDs.
However, substernal discomfort is common in sarcoidosis.
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Fatigue and weight loss are common in all ILDs.

22. PHYSICAL EXAMINATION
The findings are usually not specific.
Most commonly, physical examination reveals tachypnoea and bibasilar
end-inspiratory dry crackles, which are common in most forms of ILD
associated with inflammation but are less likely to be heard in the
granulomatous lung diseases.
Crackles may be present in the absence of radiographic abnormalities
on the chest radiograph.
Scattered late inspiratory high-pitched rhonchi—so-called inspiratory
squeaks—are heard in patients with bronchiolitis.
The cardiac examination is usually normal except in the mid or late
stages of the disease, when findings of pulmonary hypertension and
cor-pulmonale may become evident.
Cyanosis and clubbing of the digits occur in some patients with
advanced disease.

23. INVESTIGATIONS
Antinuclear antibodies and anti-immunoglobulin antibodies
(rheumatoid factors) are identified in some patients, even in the
absence of a defined CTD.
An increased lactate dehydrogenase (LDH) level is a nonspecific
finding common to ILDs.
Elevation of the serum angiotensin-converting enzyme level is
common in sarcoidosis.
Serum precipitins confirm exposure when hypersensitivity
pneumonitis is suspected, although they are not diagnostic of the
process.
Antineutrophil cytoplasmic or anti–basement membrane
antibodies are useful if vasculitis is suspected.

24. electrocardiogram is usually normal unless pulmonary
hypertension is present; then it demonstrates right-axis
deviation, right ventricular hypertrophy, or right atrial
enlargement or hypertrophy.
Echocardiography also reveals right ventricular dilatation,
hypertrophy, or both in the presence of pulmonary
hypertension.

25. CHEST X-RAY
ILD may be first suspected based on an abnormal chest radiograph,
which most commonly reveals a bibasilar reticular pattern.
A nodular or mixed pattern of alveolar filling and increased reticular
markings may also be present.
A subgroup of ILDs—sarcoidosis, chronic hypersensitivity pneumonitis,
silicosis, berylliosis, RA(necrobiotic nodular form), ankylosing
spondylitis— exhibit nodular opacities with a predilection for the upper
lung zones.
The chest x-ray correlates poorly with the clinical or histopathologic stage
of the disease.
The radiographic finding of honeycombing correlates with pathologic
findings of small cystic spaces and progressive fibrosis; when present, it
portends a poor prognosis.
In most cases, the chest radiograph is nonspecific and usually does not

26. COMPUTED TOMOGRAPHY
HRCTissuperior to the plain chest x-rayforearly detection and
confirmation of suspectedILD.
Coexistingdisease(e.g.,mediastinal adenopathy,carcinoma, or
emphysema) is often best recognized on HRCTscanning.
In the appropriate clinical setting, HRCTmay be sufficiently
characteristic to preclude the need for lung biopsy in patients with
IPF,sarcoidosis, hypersensitivity pneumonitis, asbestosis,
lymphangitic carcinoma, andPLCH.
When alung biopsy is required, HRCTscanning is useful for
determining the most appropriate area from whichbiopsy samples
should be taken.

27. PULMONARY FUNCTION TESTING
SpirometryandLungVolumes
Measurement of lung function isimportant in assessingthe extent of pulmonary
involvement in patients with ILD.
Most forms of ILDproduce arestrictive defect with reduced total lung capacity(TLC),
functional residual capacity, and residual volume.
Forcedexpiratory volume in 1second(FEV1)and forced vital capacity (FVC)are
reduced, but these changesare related to the decreasedTLC.
TheFEV1/FVCratio is usually normal or increased. Lungvolumes decrease as lung
stiffness worsens with diseaseprogression.
Afew disorders produce interstitial opacities on chestx-ray and obstructive airflow
limitation on lung function testing (uncommon in sarcoidosis and hypersensitivity
pneumonitis but common in tuberous sclerosis andLAM).

28. Arterial BloodGas:
Theresting arterial blood gasmay be normal or reveal
hypoxemia (secondary to amismatching of ventilationto
perfusion) and respiratoryalkalosis.
Anormal arterial O2tension (or saturation by oximetry) at
rest does not rule outsignificant hypoxemia during exercise
or sleep.
Carbondioxide (CO2)retention israre and isusuallya
manifestation of end-stagedisease.

29. CARDIOPULMONARY EXERCISETESTING :
useful to perform exercise testing with measurement of
arterial blood gases to detect abnormalities of gas
exchange.
Arterial oxygen desaturation, a failure to decrease dead
space appropriately with exercise ratio, and an excessive
increase in respiratory rate with a lower than expected
recruitment of tidal volume provide useful information
about physiologic abnormalities and extent of disease.
Serial assessment of resting and exercise gas exchange is
an excellent method for following disease activity and
responsiveness to treatment
Increasingly, the 6-min walk test is used to obtain a global
evaluation of submaximal exercise capacity in patients with
ILD.
The walk distance and level of oxygen desaturation tend
correlate with the patient’s baseline lung function and
mirror the patient’s clinical course.

30. FIBEROPTICBRONCHOSCOPYANDBRONCHOALVEOLARLAVAGE(BAL)

31. TISSUEANDCELLULAREXAMINATION
Lungbiopsyis the most effective method for confirming the diagnosisand
assessingdisease activity.
Thefindings mayidentify amore treatable processthan originally suspected,
particularly chronic hypersensitivity pneumonitis, COP,respiratory
bronchiolitis–associated ILD,or sarcoidosis. Biopsy should be obtained
before the initiation oftreatment.
Fiberoptic bronchoscopy with multiple transbronchial lung biopsies (4-8
samples)is often the initial procedure of choice, especially whensarcoidosis,
lymphangitic carcinomatosis, eosinophilic pneumonia, Goodpasture
syndrome, or infection issuspected.
If aspecific diagnosis is not made by transbronchial biopsy, surgicallung
biopsy by video-assisted thoracic surgery or open thoracotomy isindicated.
Adequate-sized biopsiesfrom multiple sites, usually from twolobes, should
be obtained.
Relative contraindications to lung biopsy include serious cardiovascular
disease,honeycombing and other roentgenographic evidence of diffuseend-
stage disease, severe pulmonary dysfunction, and other major operative
risks, especially in the elderly.

32. MANAGEMENTOFILD
Although the course of ILD is variable, progression is common
and often insidious.
Because therapy does not reverse fibrosis, the major goals of
treatment are
1)Permanent removal of the offending agent, when known
2)Early identification and aggressive suppression of the acute
and chronic inflammatory process, reducing further lung
damage.
(3Supplemental oxygen for Hypoxemia.
(4Treating underlying infections, cor-pulmonale.
5)Pulmonary rehabilitation to improve the quality of life in
patients

33. Glucocorticoids: First line therapy for suppression ofthe
inflammation present in ILD,but the successrate islow.
Glucocorticoid therapy is recommendedfor
symptomatic ILDpatients with eosinophilicpneumonias,
COP,CTD,sarcoidosis,
hypersensitivity pneumonitis, acute inorganicdust
exposures,
acute radiation pneumonitis, DAH,and drug-induced ILD.
In organicdust disease,glucocorticoids arerecommendedfor
both the acute and chronicstages.

34. Starting dose is prednisone, 0.5–1 mg/kg ODoral
Thisdoseiscontinued for 4–12weeks,at which time
the patient isreevaluated.
Ifthe patient is stable or improved, the doseis tapered
to 0.25–0.5 mg/kg and is maintained at this level for
an additional 4–12 weeks, depending on the course.
If the patient’s condition continues to decline on
glucocorticoids, asecond agent (Cyclophosphamide,
azathioprine ) often is added and the prednisone doseis
lowered toor maintained at 0.25 mg/kg OD.

35. Cyclophosphamide, azathioprine (1–2 mg/kg lean bodyweight
per day), and mycophenolate mofetil, with or without
glucocorticoids, have been tried with variable successin IPF,
vasculitis, progressive systemic sclerosis, and other ILDs.
Anobjective responseusually requires at least 8–12weeksto
occur.
In situations in which these drugs have failed or could not be
tolerated, other agents, including methotrexate andcyclosporine,
have been tried.
Many casesof ILDare chronic and irreversible despite thetherapy,
and lung transplantation may then beconsidered.

36. IDIOPATHICPULMONARY FIBROSIS
Also known asusual interstitial pneumonia(UIP)
most common form of idiopathic interstitial pneumonia.
IPFhasadistinctly poor response to therapy and abadprognosis.
TypeI pneumocytes are lost, and there is proliferation ofalveolar
type II cells.
Clinical Manifestations:
Exertional dyspnoea, Non-productive cough, and
inspiratory crackleswith or without digital clubbing maybe
present on physicalexamination.

38. IDIOPATHICPULMONARY FIBROSIS
HRCTlung scanstypically show patchy,predominantly basilar,subpleural
reticular opacities, often associatedwith traction bronchiectasis and
honeycombing.
Adefinite UIPpattern on HRCTis highly accurate for the presence of aUIP
pattern on surgical lungbiopsy.
Atypical findings that should suggestan alternative diagnosis include
extensive ground-glass abnormality, nodular opacities, upper or midzone
predominance, and prominent hilar or mediastinallymphadenopathy.
Pulmonary function tests often reveal arestrictive pattern, areducedDlCO,
and arterial hypoxemia that is exaggeratedor elicitedby exercise.

40. Management of IPF:
Untreated patients with IPFshow continued progression of their diseaseand havea
high mortality rate.
There is no effective therapy for IPF.
Thalidomide appears to improve cough in patients withIPF.
Chronic microaspiration secondaryto gastroesophageal reflux mayplay arole in the
pathogenesis and natural history of IPF.
Gastroesophageal reflux (GER)therapy may be of benefit in IPF.
In patients with IPF,treatment with the three-drug regimen of prednisone,
azathioprine, and N-acetylcysteine (NAC)or warfarin (in IPFpatients who
lacked other indications for anticoagulation) hasbeen shown to increase the
risks of hospitalization and death.
Patients with IPFand coexisting emphysema (combined pulmonary fibrosis and
emphysema [CPFE])are more likely to require long-term oxygen therapy and
develop pulmonary hypertension and may haveamore dismal outcome than those
without emphysema.
Patients should be referred early for lung transplant becauseof theunpredictability
of diseaseprogression

41. Disease Age
M:F
C/F Imaging Prognosis REMARKS
Nonspecific
interstitial
pneumonitis
40-50 May be
indistinguishabl
e from UIP
Bilateral,
subpleural
ground glass
Prognosis
good but
depends on
But
Surgical
Biopsy is
(NSIP) opacities asso
with lower lobe
volume loss
the extent
of fibrosis
at
diagnosis
greater
than 10
years.
needed to
confirm.
Cryptogenic
organizing
pneumonitis
(bronchioliti
sobliterans
organizing
pneumonia
[BOOP])
50–60 Abrupt onset,
frequently
weeks to a few
months
following a flu-
like illness.
constitutional
symptoms are
common
areas of air-
space
consolidation,
ground-glass
opacities, small
nodular
opacities, and
bronchial wall
thickening and
dilation.
Good Rule out
infection
and treat
with
steroids

42. Disease Age
M:F
C/F Imaging Prognosis REMARKS
Respiratory
bronchiolitis-
associated
interstitial
lung disease
younger Heavy
smokers
with
similar
complains
Like UIP.
Bronchial wall
thickening,
centrilobular
nodules, ground
glass opacity
with
Airtrapping
Emphysematou
s change.
survival
greater
than 10 y
Spontaneo
us
remission
20%.
ILD with
Obstructiv
pattern
Acute
interstitial
pneumonitis
Hamman-
Rich
syndrome.
young Apparently
normal
indistinguis
hable from
that of
idiopathic
ARDS
ARDS
Diffuse b/l
airspace
consolidation
with patchy
symmetric areas
of ground-glass
attenuation
POOR Most
severe form
of ILD
Pneumonia

43. NONSPECIFIC INTERSTITIAL PNEUMONITIS
(NSIP)

44. CRYPTOGENIC ORGANIZING PNEUMONITIS
(BRONCHIOLITIS OBLITERANS ORGANIZING
PNEUMONIA [BOOP])

45. Smokingrelated ILD
Respiratory bronchiolitis- associated
interstitial lung disease

46. ACUTE INTERSTITIAL PNEUMONITIS

47. OCCUPATIONAL & RECREATIONAL
EXPOSURE RELATEDILD
Exposure Associated LungDisease
Bird breeders andfanciers Hypersensitivity pneumonitis
Automotive mechanics,Electricians
Pipefitters
Shipyardworkers
Asbestosis
Electronic and computer industry
workers
Berylliosis
Farmers Farmer’s lung (hypersensitivity
pneumonitis)
Hot tub, sauna,humidifiers Hypersensitivity pneumonitis
Metal workers (tool anddie) Metal-induced pneumoconioses
Miners, Sandblasters, Ceramic
workers
Silicosis
Miners (specificallycoal) Coalworkers’ pneumoconiosis

48. Coalminers
pneumoconioisis
Rounded opacities between 1 and 5 mm
(upper and middle zones)
small irregular and linear opacities
Progressive massive fibrosis
almost always starts in an upper zone
Calcification is not a feature
Cavitation ofPMF can occur
Caplan's syndrome is the name given to the combination
of rheumatoid disease and several round nodules (usually
1 to 5 cm in diameter) in the lungs of a coal miner.

49. SILICOSIS
Clues to diagnosis
Micronodular pattern
Simple silicosis :
Upper lobes
Small multiple nodules
Egg shell calcification
Complicated :
>1 cm nodules
Acute silicosis :
small nodular pattern with ground glass
appearance( crazy paving )
PMF : nodules coalesce to large masses
BAL : dust particles on polarised light

50. X Ray:
reticular interstitial pattern
pleural plaques ( lower lung field , cardiac
border and diaphragm )
Irrregular linear opacities first noted in
lower lung fields.
HRCT :
Distinct subpleural curvilinear opacities 5-
10 mm length parallel to pleural surface
BAL:
Asbestos bodies
ASBESTOSIS
Clues to diagnosis

51. HYPERSENSITIVITY PNEUMONITIS
Hypersensitivity pneumonitis (HP),or extrinsic allergic
alveolitis, is aspectrum of interstitial, alveolar, and
bronchiolar lung diseasesresulting from immunologically
induced inflammation in response to inhalation of awide
variety of different materials that are usually organic or low-
molecular weight chemical antigens (or haptens) that may
lead to irreversible lungdamage.
Despite the terms hypersensitivity and allergic, HPisnot an
atopic disease and is not associated with increased IgEor
eosinophils.

52. Hypersensitivity pneumonitis (HP)hasAcute, subacute& chronic
manifestations.
Acute manifestations include:
transient fever, hypoxemia, myalgias, arthralgias, dyspnoea, and
cough that occur 2 to 9 h after exposure and resolve in 12 to 72 h
without specific treatment (sometimes longer after aparticularly
intense exposure).
Patients exhibit tachypnea, bibasilar rales, and occasionally cyanosis.
Thereis usually peripheral blood leukocytosis with neutrophilia and
lymphopenia (without eosinophilia), and bronchoalveolar lavage
(BAL)neutrophilia.
Subacute or intermittent diseasemay result from repeatedexposures,
and manifest as
productive cough, dyspnea, fatigue, and weight loss.
There may be BALlymphocytosis, frequently (although notalways)
with apredominance of CD8+Tlymphocytes.

53. ChronicHPisclinically more insidious, and patients maylacka
history of acuteepisodes.
present with agradual onset of cough,dyspnea,fatigue,and
weight loss.
Symptoms are usually present for months toyears.
Thereistypically no fever,but tachypnea andbibasilardry
rales are usually present.
Thisform of the diseasemaybe difficult to distinguish from
idiopathic pulmonary fibrosis.
symptomsandsignsof cor-pulmonale arenot uncommonat
presentation.

54. bilateral lower lobe 2- to 3-mm
nodules
diffuse nodular radiodensities in the lower
lobes, with areas of ground- glass densities
posteriorly.

55. INTERSTITIAL LUNG DISEASE IN CTD
Suspect a CTDif patient has:
Musculosketetal pain
Weakness
Fatigue
Joint pains and swelling
Photosensitivity
Raynaudsphenomenon
Pleuritis
Dry eyes or mouth

56. ProgressiveSystemicSclerosis (PSS)
•Clinical evidenceof ILDispresent in about one-half of patientswith
progressive systemic sclerosis (PSS),and pathologic evidence is
present in three-quarters.
•Pulmonary function tests show arestrictive pattern and impaired
diffusing capacity, often before any clinical or radiographic evidence
of lung diseaseappears.
•Pulmonaryvasculardiseasealone or in association withpulmonary
fibrosis, pleuritis, or recurrent aspiration pneumonitis is strikingly
resistant to current modes oftherapy.

57. RheumatoidArthritis
•ILDassociated with RAis more common inmen.
•Pulmonary manifestations of RAinclude
•pleurisy with or withouteffusion,
•ILDin up to 20%of cases,
•necrobiotic nodules (nonpneumoconiotic
intrapulmonary rheumatoid nodules) withor
without cavities,
•Caplan’ssyndrome(rheumatoid
pneumoconiosis),
•pulmonary hypertension secondaryto
rheumatoid pulmonary vasculitis,
•BOOP,and
•upper airway obstruction causedby
cricoarytenoid arthritis.

58. SystemicLupus Erythematosus
•Lungdiseaseis acommon complication in SLE.
•Pulmonary manifestations”
•Pleuritis with or without effusion is the most common.
•atelectasis,
•diaphragmatic dysfunction with lossof lungvolumes,
•pulmonary vasculardisease,
•pulmonary hemorrhage,
•uremic pulmonaryedema,
•infectious pneumonia,and
•BOOP.
•Acutelupuspneumonitis characterizedbypulmonary capillaritis leadingto
alveolar hemorrhage isuncommon.
•Chronic,progressive ILDis uncommon.It is important to excludepulmonary
infection.
•Although pleuropulmonary involvement maynot be evident clinically,
pulmonary function testing reveals abnormalities in many patients withSLE.

59. Polymyositisand Dermatomyositis
•ILDoccursin ∼10%of patients with polymyositisand
dermatomyositis (PM/DM).
•Diffuse reticular or nodular opacities with or without analveolar
component occur radiographically, with apredilection for the
lung bases.
•ILDoccursmore commonly in the subgroupof patientswith an
anti–Jo-1 antibody that is directed tohistidyl tRNAsynthetase.
•Weaknessof respiratory musclescontributing toaspiration
pneumonia may be present.
•Arapidly progressive illness characterized bydiffuse alveolar
damage may causerespiratory failure.

60. HRCT in RA
bibasilar peripheral reticular pattern,
intralobular interstitial thickening
distortion of the lung parenchyma
Bilateral is present, predominantly on the
left side
bibasilar peripheral reticular pattern,
pleural effusion
thickening of the interlobular septa,

61. DRUG REACTIONSCAUSING ILD
Alveolar Haemorrhage:
Amiodarone,Amphotericin B,Clopidogrel, Cocaine,
Cyclophosphamide, Haloperidol, Heparin, Hydralazine, Mitomycin,
Nitrofurantoin, Penicillamine, Statins, Streptokinase,Sulfonamides
BOOP(bronchiolitis obliterans organizing pneumonia )
Carbamazepine,Cephalexin,Chloroquine, Cocaine,
Cyclophosphamide, interferon-a Mecamylamine Mesalamine
Methotrexate,Nitrofurantoin, Phenytoin, Statins,Sulfasalazine
Constrictivebronchiolitis
Busulfan, penicillamine

62. EOSINOPHILIC PNEUMONIAS
Classified as:
(1simple eosinophilic pneumonia (Loeffler syndrome),
(2acuteeosinophilic pneumonia (AEP)-aprocessthat
clinically resemblesARDS,
(3chronic eosinophilic pneumonia (CEP)that hasa
longer time course and does not result in
respiratory failure.

65. AEP CEP

66. RADIATION PNEUMONITIS
evident 6 weeks to 6 months followingradiotherapy
radiographs showalveolar opacities that generally conform tothe
treatment portals.
In most cases,the pneumonitis isasymptomatic
If symptomatic, characterizedby the abrupt onset of fever,cough,
and dyspnea.
Managedwith supportive carein conjunctionwith
Glucocorticoids.

68. Vasculitic
Disorders
Lung
Involvement
ANCA Interstial Pattern
seen
Wegener
granulomatosis
Common c-ANCA >> p-
ANCA
80–
90%
Diffuse Alveolar
Hemorrage with
nodules
,cavitation
Microscopic
polyangiitis
Common Common p-
ANCA > c-ANCA
80%
DAH
Churg-Strauss
syndrome
Common p-ANCA > c-
ANCA
30–50%
DAH with
transient infiltates
Goodpasture
syndrome
Common p-ANCA
10%
DAH
Takayasu arteritis Common Negative DAH
ILD WITH A GRANULOMATOUS OR VASCULAR STRUCTURES

69. X ray : consolidation, typically resolving within a matter of days,multiple
abcesses
HRCT : ground-glass partial alveolar filling.
Hb : anaemia( iron defeciency )
BAL :- frank blood-staining in sequential lavage (acute presentation) and
numerous macrophages containing iron, identified by Perl's stain
Dlco :- may be increased in acute conditions but is chronically low
ILD in VASCULITIC
DISORDERS
Suspect if
Mononeuritis mutiplex
Renal involvement
Skin lesions
haemoptysis

70. SARCOIDOSIS
Probably one-third of sarcoid patients who come to medical
attention are asymptomatic, and the disease is picked up as an
incidental finding on chest imaging.
Another one third have fever, malaise, and weight loss, and an
equal proportion have shortness of breath (SOB), cough,and
sometimes chest pain.
Pulmonary function varies by radiologic stage.
Low-stage disease patients may have normal pulmonary
function, but airflow obstruction (because sarcoid granulomas
can narrow the large and small airways and restriction are also
seen.
High-stage (i.e., diffuse fibrotic) disease shows a restrictive
pattern with decreased diffusing capacity; pulmonary
hypertension may also be present.

71. •Stage0:Nodemonstrableradiographic
abnormality
•
Stage1: Hilar and mediastinallymph
node enlargement
•without radiographicparenchymal
abnormality
•Stage2: Hilar and mediastinal. lymph
node enlargement plus parenchymal
abnormality
•Stage3: Parenchymalabnormality
alone
•Stage4: Advanced fibrosis.

73. SARCOIDOSIS CTD….
BAL :-lymphocytosis
CD4 : CD8 > 3.5 is most specific
PFT :-Restrictive pattern
ButObstructive componentpresent in many
Biopsy :-non caseatinggranulomas
lymphocytosis
Sr.ACE levels:-
Hyper calciuria orHypercalcemia

74. LYMPHOCYTICINFILTRATIVEDISORDERS
Thisgroup of disordersfeatures lymphocyte andplasmacell
infiltration of the lungparenchyma.
Are benign or canbehave aslow-grade lymphomas.
characterized by diffuse lymphadenopathy, fever, hepato-
splenomegaly, and hemolytic anemia, with ILDin somecases.
Lymphocytic Interstitial Pneumonitis
Lymphomatoid Granulomatosis
histologicfindings: angiocentric malignant (Tcell) lymphoma
characterized by apoly- morphic lymphoid infiltrate, anangiitis,
and granulomatosis.

76. PULMONARY ALVEOLAR PROTEINOSIS
Theclinical features of (PAP)are nonspecific
cough, shortness of breath (SOB),malaise, and
sometimes chest pain or weight loss, usually
developing in an indolent fashion.
Chestexaminationisfrequently unremarkable,
although crackles, clubbing, and cyanosis are
reported.

77. PULMONARY ALVEOLARPROTEINOSIS
diffuse reticulo-alveolar infiltrates
BAT WING distribution
BAL:- milky effulent foamy
macrophages with lipoproteinous
intraalveolarmaterial
thickened interlobular septa
“crazy paving” ground glass
fashion, sharply demarked from
normallung creating a
“geographic” pattern.

78. PROGNOSIS
Someforms of interstitial lung diseaseresolve
completely, while others lead to long-termand
irreversible scarring and lung damage with
accompanying respiratory failure .
Pulmonary hypertension candevelop in casesof long-
standing interstitial lung diseaseand canlead to cor
pulmonale
Theprognosis isdependent upon the type and severity
of interstitial lung diseaseaswell asthe underlying
health status of thepatient.

79. PREVENTION
Avoid or limit exposure to toxins or treatments that can lead to ILD
Proper diet and exercise reduces chance of developingILD.
Quitting smoking and avoiding exposure to substances known to
causeILDcanprevent the disorder from developing or worsening.
Peoplewho areemployed in jobs where they maybeheavily
exposed to known causesof lung disease in the workplace
typically should undergo routine screening forlung disease.