ILD DETAIL 2019

1. INTERSTITIAL LUNG DISEASE
Dr. Shivaom Chaurasia
Internal Medicine
First Year Resident

2. INTRODUCTION
 These are the large number of conditions that involves the parenchyma of the lung alveoli, alveolar epithelium the
capillary endothelium and space between these structures as well as the perivascular and lymphatic tissue.
 This region includes a variety of cell types (fibroblasts, myofibroblasts, and macrophages) and matrix components
(collagens, elastin, and proteoglycans).
• ILDs have been difficult to classify because >200 known individual diseases are characterized by diffuse
parenchymal lung involvement, either as the primary condition or as a significant part of a multiorgan process, as
may occur in the connective tissue diseases (CTDs).
• ILDs classified into two groups based on the major underlying histopathology:
• 1. those associated with predominant inflammation and fibrosis and
• 2. those with a predominantly granulomatous reaction in interstitial or vascular areas
• Each of these groups can be further clinically subdivided according to known or unknown etiology.

4. PATHOGENESIS
• ILDs are non-malignant disorders and are not caused by identified 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 common features
• The two major histopathologic patterns:
• inflammation and fibrosis
• granulomatous

5. INFLAMMATION & FIBROSIS
• The initial insult is an injury to the epithelial surface causing inflammation in the air spaces and alveolar walls.
• If the disease becomes chronic, inflammation spreads to adjacent portions of the interstitium and vasculature and
eventually causes interstitial fibrosis.
• Important histopathologic patterns found in the ILDs include UIP, NSIP, respiratory bronchiolitis, organizing
pneumonia (BOOP), diffuse alveolar damage (acute or organizing), DIP, & lymphocytic interstitial pneumonia.
• The development of irreversible scarring (fibrosis) of alveolar walls, airways, or vasculature is the most feared
outcome in all of these conditions because it is often progressive and leads to significant derangement of ventilatory
function and gas exchange.

9. DIAGNOSING ILD
• A clinical diagnosis is possible for many forms of ILD, especially if 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.
• HRCT scanning improves diagnostic accuracy as experience with histologic-image correlation is perfected.
1.HISTORY
a.Age
Ages of 20 and 40 years
Sarcoidosis, connective tissue disease-associated ILD, rare ILD such as lymphangioleiomyomatosis,
Pulmonary Langerhans cell histiocytosis,
over age 60
idiopathic pulmonary fibrosis
b.Gender-Less influential then age.
Female preponderance
Lymphangioleiomyomatosis, many connective disorder except rheumatoid arthritis
Men
Rheumatoid arthritis is more common in men.
Because of occupational exposures, men are also more likely to have a pneumoconiosis.

10. • Duration of symptoms:
• acute presentations (days to weeks) uncommon and are commonly misdiagnosed as
more common disease such as pneumonia, COPD exacerbation or heart failure.
• Some of the condition with acute onset includes- eosinophilic pneumonia, acute interstitial
pneumonia (m/c), Hypersensitive pneumonia and granulomatosis with polyangitis
• Chronic indolent presentation (months to years)-most common presentation
• Subacute form(weeks to months)- could suggest sarcoidosis, CTD associated ILD, Drug
induced ILD or COP

11. SIGN AND SYMTOMS
• A. RESPIRATORY SYMPTOMS:
• Progressive 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.
• Dry cough is common especially in IPF. However cough with hemoptysis may suggest Diffuse
pulmonary hemorrhage eg- Goodpasture Syndrome and also secondary pulmonary infection
with bronchiectiasis
• Wheezing is an uncommon manifestation of ILD but has been described in patients with
chronic eosinophilic pneumonia, Churg Strauss syndrome, respiratory bronchiolitis, and
sarcoidosis
• Clinically significant chest pain is uncommon in most ILDs. However, substernal discomfort is
common in sarcoidosis.
• Sudden worsening of dyspnoea, especially if associated acute chest pain, may indicate a
spontaneous pneumothorax, tuberous sclerosis, LAM, and neurofibromatosis.

12. • B. NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
• Arthritis
• Ocular
• Skin and muscle
• GERD
• Lower GI symptoms
• Recurrent sinusitis
• Neurological symptoms
• Epilepsy & mental retardation
• Diabetes insipidus
• Fatigue and weight loss are common in all ILDs

13. • E . PAST MEDICAL HISTORY
• Any history of connective tissue disease, inflammatory bowel disease, or malignancy might
be a clue to an associated ILD
• A history of allergic rhinitis and asthma may implicate chronic eosinophilic pneumonia,
• Nasal polyposis and asthma may suggest eosinophilic granulomatosis with polyangitis
(EGPA, Churg-Strauss).
• An immunocompromised state due to an underlying disease or immunomodulatory therapy
may suggest an infectious cause of ILD.

14. F .Smoking history
• Occur largely among current or former smokers
• Pulmonary langerhans cell histiocytosis
• Desquamative interstitial pneumonitis
• Respiratory bronchiolitis-interstitial lung disease
• Idiopathic pulmonary fibrosis
• Among never or former smokers
• Sarcoidosis
• hypersensitivity pneumonitis.
• Active smoking can contribute to complications in patients with Goodpasture's syndrome, in which pulmonary
hemorrhage is far more frequent among current smokers. In this setting, it is thought that smoking damages
the alveolar wall, making the alveolar basement membrane more accessible to the circulating antibasement
membrane antibodies.
• G .Famlial History:
• An autosomal dominant pattern of inheritance has been reported for idiopathic pulmonary fibrosis (IPF),
tuberous sclerosis, and neurofibromatosis
• An autosomal recessive pattern of inheritance has been identified for Niemann-Pick disease, Gaucher's
disease, and Hermansky-Pudlak syndrome

15. 2.PHYSICAL EXAMINATION
• Crackles or "velcro rales" are present on chest examination in most forms of ILD, although they are less
likely to be heard in the granulomatous lung diseases, especially sarcoidosis
• 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 frequently heard on
chest examination in patients with bronchiolitis, but may also be heard in patients with traction
bronchiectasis due to pulmonary fibrosis.
• Clubbing — Clubbing of the digits is common in some pulmonary disorders (idiopathic pulmonary fibrosis,
asbestosis) and rare in others (sarcoidosis, hypersensitivity pneumonitis, pulmonary Langerhans cell
histiocytosis)

16. 3.LABORATORY TESTS

17. 4.PULMONARY FUNCTION TESTING:
• Restrictive defect
• Reduced total lung capacity (TLC), functional residual capacity, and residual volume
• Forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) reduced
• FEV1/FVC ratio is usually normal or increased
• Diffusing Capacity:
• Reduction in the diffusing capacity of the lung for carbon monoxide (DlCO)
• nonspecific finding in most ILDs.
• This is due to V/Q mismatch
• Arterial Blood Gas:
• hypoxemia
• Carbon dioxide retention

18. 5.CARDIOPULMONARY EXERCISE
TESTING:
• 6-min walk test is used to evaluate exercise capacity in patients with ILD.
• Walk distance and asses level of oxygen desaturation
• Serial assessment of resting and exercise gas exchange response
• Detects exercise-induced hypoxemia
• Indicates activity and responsiveness to treatment
• correlate with the patient’s baseline lung function and patient’s clinical course.

19. 6.INVESTIGATION
• A. CHEST X - RAY
• ILD may be first suspected based on an abnormal chest radiograph, which most commonly reveals a
basilar 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,
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 carries a poor prognosis.
• In most cases, the chest radiograph is nonspecific and usually does not allow a specific diagnosis.

20. • B. COMPUTED TOMOGRAPHY
• HRCT is superior to the plain chest x-ray for early detection and confirmation of suspected ILD.
• Coexisting disease (e.g., mediastinal adenopathy, carcinoma, or emphysema) is often best recognized on
HRCT scanning.
• In the appropriate clinical setting, HRCT may be sufficiently characteristic to preclude the need for lung
biopsy in patients with IPF, sarcoidosis, hypersensitivity pneumonitis, asbestosis, lymphangitic carcinoma
• When a lung biopsy is required, HRCT scanning is useful for determining the most appropriate area from
which biopsy samples should be taken.
C. BRONCHOALVEOLAR LAVAGE (BAL)
• useful in narrowing the differential diagnostic
NO role:
• in defining the stage of disease
• assessment of disease progression
• response to therapy

21. IMAGING
• Upper zone lung disease is found preferentially in:
• Tuberculosis
• Fungal disease
• Sarcoidosis
• Pneumoconiosis (with the notable exception of asbestosis)
• Langerhans cell histiocytosis (previously eosinophilic granuloma)
• Ankylosing spondylitis
• Cystic fibrosis
• Cystic Pneumocystis jirovecii pneumonia
• Radiation fibrosis
• Endstage hypersensitivity pneumonia (ie, extrinsic allergic alveolitis)
• Basal lung disease is found in a different group of diseases:
• Bronchiectasis
• Aspiration
• Desquamative interstitial pneumonia (DIP)
• Nonspecific interstitial pneumonia and fibrosis
• Idiopathic interstitial pneumonia (IPF)/usual interstitial pneumonia (UIP)
• Drug reactions
• Asbestosis, scleroderma
• Central, perihilar lung disease occurs in chronic diseases
including:
• Sarcoidosis
• Lymphoma
• Kaposi's sarcoma
• Bronchiectases
• Central, perihilar lung disease also occurs acutely. As
examples:
• Pulmonary edema (appears as perihilar, batwing, or butterfly pattern)
• Pulmonary hemorrhage
• Bacterial pneumonia
• Peripheral lung disease is found in the following:
• Cryptogenic organizing pneumonia (COP)/bronchiolitis obliterans
organizing pneumonia (BOOP), I
• Asbestosis
• Eosinophilic lung disease
• Graft-versus-host disease

22. HRCT PATTERNS IN INTERSTITIAL LUNG DISEASE

23. 7.LUNG BIOPSY
• Lung biopsy is generally helpful in the evaluation of patients with ILD in the following situations:
• To provide a specific diagnosis. This is especially desirable in a patient with clinical features such as age <50 years, fever,
weight loss, hemoptysis, or signs of vasculitis; a progressive course; atypical or rapidly changing HRCT findings; unexplained
extrapulmonary manifestations; or pulmonary vascular disease of unclear origin.
• To exclude neoplastic and infectious processes that can mimic chronic, progressive ILD.
• To identify a more treatable process than originally suspected (eg, hypersensitivity pneumonitis versus idiopathic pulmonary
fibrosis).
• To predict the likelihood of response to therapy before proceeding with therapies that may have serious side effects (eg, cellular
nonspecific interstitial pneumonia or organizing pneumonia versus idiopathic pulmonary fibrosis).
• Rarely, to diagnose ILD in a patient with hypoxemia, pulmonary function tests strongly suggestive of ILD, a negative evaluation
for pulmonary vascular disease, and a normal HRCT.
• Relative contraindications to lung biopsy include:
• Radiographic evidence of diffuse end-stage disease, eg, "honeycombing," without areas of milder disease activity, as biopsy of
end-stage fibrosis is unlikely to reveal an etiology.
• Extensive pleural disease or emphysema that increase the risk of biopsy.
• Severe pulmonary dysfunction manifest by a diffusing capacity (DLCO) less than 35 percent predicted, severe hypoxemia
needing supplemental oxygen, or respiratory failure requiring mechanical ventilation.
• Serious cardiovascular disease, advanced age, or other major risks for surgery or general anesthesia.

28. 9.MANAGEMENT OF ILD
• 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.
• 3) Supplemental oxygen for Hypoxemia.
• 4) Treating underlying infections, cor-pulmonale.
• 5) Pulmonary rehabilitation to improve the quality of life in patients
•

29. • Glucocorticoids:
• First line therapy for suppression of the inflammation present in ILD, but the success rate is low.
• Glucocorticoid therapy is recommended for
• symptomatic ILD patients with eosinophilic pneumonias, COP, CTD, sarcoidosis,
• hypersensitivity pneumonitis, acute inorganic dust exposures,
• acute radiation pneumonitis, DAH, and drug-induced ILD.
• In organic dust disease, glucocorticoids are recommended for both the acute and chronic stages.
• Starting dose is prednisone, 0.5–1 mg/kg OD oral
• This dose is continued for 4–12 weeks, at which time the patient is reevaluated.
• If the patient is stable or improved, the dose is 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, a second agent (Cyclophosphamide,
azathioprine ) often is added and the prednisone dose is lowered to or maintained at 0.25 mg/kg OD
• An objective response usually requires at least 8–12 weeks to occur.
• Many cases of ILD are chronic and irreversible despite the therapy, and lung transplantation may then be
considered.

30. GUIDELINES FOR LUNG TRANSPLANTATION

31. IDIOPATHIC INTERSTITIAL PNEUMONIA
1.IDIOPATHIC PULMONARY FIBROSIS
• Also known as usual interstitial pneumonia(UIP)
• most common form of idiopathic interstitial pneumonia.
• IPF has a distinctly poor response to therapy and a bad prognosis.
• Type I pneumocytes are lost, and there is proliferation of alveolar type II cells.
• Clinical Manifestations:
• Exertional dyspnoea,
• Non-productive cough, and
• inspiratory crackles with or without digital clubbing may be present on physical examination.
• HRCT lung scans typically show patchy, predominantly basilar, subpleural reticular opacities, often associated
with traction bronchiectasis and honeycombing.
• A definite UIP pattern on HRCT is highly accurate for the presence of a UIP pattern on surgical lung biopsy.
• Atypical findings that should suggest an alternative diagnosis include extensive ground-glass abnormality,
nodular opacities, upper or midzone predominance, and prominent hilar or mediastinal lymphadenopathy.
• Pulmonary function tests often reveal a restrictive pattern, a reduced DlCO, and arterial hypoxemia that is
exaggerated or elicited by exercise.

32. Management of IPF:
• Untreated patients with IPF show continued progression of their disease and have a high mortality rate.
• There is no effective therapy for IPF.
• Thalidomide appears to improve cough in patients with IPF.
• Chronic microaspiration secondary to gastroesophageal reflux may play a role in the pathogenesis and
natural history of IPF.
• Gastroesophageal reflux (GER) therapy may be of benefit in IPF.
• Patients with IPF and coexisting emphysema (combined pulmonary fibrosis and emphysema [CPFE]) are
more likely to require long-term oxygen therapy and develop pulmonary hypertension
• Patients should be referred early for lung transplant because of the unpredictability of disease progression

33. 2.NONSPECIFIC INTERSTITIAL
PNEUMONIA
subgroup of the Idiopathic interstitial pneumonias
• Histologic Findings; There is less temporal and spatial heterogeneity than in UIP, and little or no
honeycombing is found
• predominantly cellular or fibrosing
• Treatment :
• The majority of patients with NSIP have a good prognosis
• glucocorticoids, often used alone or in combination with azathioprine or mycophenolate mofetil.

34. 3.ILD ASSOCIATED WITH CIGARETTE
SMOKING
1.Desquamative Interstitial Pneumonia:
• The chest x-ray and HRCT scans usually show diffuse hazy opacities.
• HISTOLOGY:
• A diffuse and uniform accumulation of macrophages in the alveolar spaces is the hallmark of DIP.
• The macrophages contain golden, brown, or black pigment of tobacco smoke
• Treatment:
• (10-year survival rate is 70%)
• smoking cessation.
2.Respiratory Bronchiolitis–Associated ILD
• subset of DIP
• HRCT lung :
• Bronchial wall thickening, centrilobular nodules
• ground-glass opacity, and emphysema with air trapping
• Histologic : alveolar macrophage accumulation in respiratory bronchioles
• Treatment RB-ILD appears to resolve in most patients after smoking
cessation alone.

35. • 3.Pulmonary Langerhans Cell Histiocytosis:
• primarily affects men between the ages of 20 and 40 years.
• Pneumothorax occurs in ~25% of patients.
• Hemoptysis and diabetes insipidus are rare manifestations.
• HRCT :combination of nodules and thin-walled cysts is virtually diagnostic.
• Histologic findings: presence of nodular sclerosing lesions that contain Langerhans cells accompanied by mixed
cellular infiltrates.
• Treatment: Discontinuance of smoking

37. 4.DRUG-INDUCED ILD
• A detailed history of the medications taken by the patient is needed to identify drug-induced disease
• The extent and severity of disease are usually dose-related.
• The onset may be abrupt and fulminant
• may be insidious, extending over weeks to months.
• several years before a reaction develops (e.g., amiodarone),
• weeks to years after the drug has been discontinued (e.g., carmustine)
• Histologic Findings: The patterns of lung injury vary widely and depend on the agent.
• Treatment :discontinuation of any possible offending drug and supportive care.

38. DRUGS

39. CRYPTOGENIC ORGANIZING PNEUMONITIS (BRONCHIOLITIS
OBLITERANS ORGANIZING PNEUMONIA [BOOP])
• Clinical Manifestations
• typically involves patients in their 50–60s
• often presents as a subacute flu-like illness, with cough, dyspnea, fever, and fatigue. Inspiratory rales are often present on examination
and most patients are noted to have restrictive lung deficits on pulmonary function testing with hypoxemia.
• It is commonly mistaken for pneumonia. It is important to note that this syndrome can occur in isolation or can be secondary to an
underlying connective tissue disease (e.g., polymyositis), medications, or can result from an underlying malignancy.
• Laboratory testing for various connective tissue diseases is helpful as they can both be diagnostic and suggest the need for prolonged
medical therapy.
• HRCT Image Findings:
• include patchy, sometimes migratory, subpleural consolidative opacities often with associated ground-glass opacities.
• Peribronchiolar, or perilobar opacities can be present and sometimes a rim of subpleural sparing (often referred to as a reversed halo
or atoll sign) can be seen which can aid in the diagnosis
• Histopathology Surgical lung biopsy
• reveal patchy regions of organizing pneumonia with granulation tissue that commonly involves the small airways, alveolar ducts, and
alveoli with surrounding inflammation that can involve the alveolar walls
• Treatment
• Corticosteroids
• substantial clinical improvement in many patients but usually need to be continued for at least 6 months as relapse rates are high.
• Evidence is growing that alternate cytotoxic (e.g., mycophenolate, cyclophosphamide) or biologic (e.g., rituximab) therapies can be
helpful in both treating the disease and reducing the need for steroids. In some patients with secondary forms of the disease, long-term
therapy may be needed.

40. ACUTE OR CHRONIC IIPS
A.ACUTE INTERSTITIAL PNEUMONIA (HAMMAN-RICH SYNDROME)
• Clinical Manifestations:
• AIP is a rare and often fatal lung disorder that is characterized by an acute onset of respiratory distress and hypoxemia.
• A prodromal period of symptoms consistent with an acute upper respiratory infection is common. The mortality rate within 6
months of presentation can be quite high (>50%) and recurrences are common.
• In those that recover, lung function improvement can be substantial.
• AIP can be difficult to distinguish from acute respiratory distress syndrome (ARDS) and an acute exacerbation of an
unsuspected underlying pulmonary fibrotic process.
• HRCT Image Findings
• The most common imaging findings are patchy bilateral ground-glass opacities.
• Dependent regions of air-space consolidation are also common.
• Histopathology
• Similar to ARDS and acute exacerbations of underlying pulmonary fibrosis, AIP presents histopathologically as diffuse
alveolar damage (DAD) demonstrated on a surgical lung biopsy
• .Treatment
• often includes mechanical ventilation.
• There is no proven drug therapy for AIP.
• Glucocorticoids are often given but they are not clearly effective and have been demonstrated not to be beneficial in other
forms of DAD

41. B. ACUTE EXACERBATIONS OF IIPS
• Clinical Manifestations
• Acute exacerbations are not separate disorders, but rather an accelerated phase of lung injury that can occur in any ILD
resulting in pulmonary fibrosis.
• Acute exacerbations are characterized by an acute onset (<30 days) of respiratory distress and hypoxemia occurring in a
patient with underlying pulmonary fibrosis not explained by an alternate cause (e.g., pneumonia, left heart failure).
• Reported mortality rates are very high (>85%).
• HRCT Image Findings
• The most common imaging findings include patchy bilateral ground-glass opacities and dependent regions of air-space
consolidation.
• Sometimes these new changes can be appreciated on the background of the imaging findings typified by the underlying
IIP, although sometimes they obscure the preceding imaging findings.
• Histopathology
• Acute exacerbations of underlying pulmonary fibrosis present histopathologically as DAD, although sometimes organizing
pneumonia can also be demonstrated on a surgical lung biopsy.
• Treatment
• is mostly supportive. Mechanical ventilation, when not being used as a bridge to lung transplantation, is controversial as
the survival rate in these patients tends to be poor.

42. OCCUPATIONAL & RECREATIONAL EXPOSURE RELATED ILD

44. 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 )
• BAL : dust particles on polarised light

45. ASBESTOSIS
• Clues to diagnosis
• 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
• BAL: Asbestos bodies

46. 1V.INTERSTITIAL LUNG DISEASE IN
CONNECTIVE TISSUE DISEASE
• Suspect a CTD if patient has :
• Musculosketetal pain
• Weakness
• Fatigue
• Joint pains and swelling
• Photosensitivity
• Raynauds phenomenon
• Pleuritis
• Dry eyes or mouth

47. A. PROGRESSIVE SYSTEMIC SCLEROSIS
(PSS)
• Clinical evidence of ILD is present in about one-half of patients with progressive systemic
sclerosis (PSS), and pathologic evidence is present in three-quarters.
• • Pulmonary function tests show a restrictive pattern and impaired diffusing capacity, often
before any clinical or radiographic evidence of lung disease appears.
• • Pulmonary vascular disease alone or in association with pulmonary fibrosis, pleuritis, or
recurrent aspiration pneumonitis is strikingly resistant to current modes of therapy.

48. B. RHEUMATOID ARTHRITIS
• ILD associated with RA is more common in men.
• Pulmonary manifestations of RA include
• pleurisy with or without effusion,
• ILD in up to 10% of cases,
• necrobiotic nodules (nonpneumoconiotic intrapulmonary rheumatoid nodules) with or without cavities,
• Caplan’s syndrome (rheumatoid pneumoconiosis),
• pulmonary hypertension secondary to rheumatoid pulmonary vasculitis,
• upper airway obstruction caused by cricoarytenoid arthritis
• HRCT in RA bibasilar peripheral reticular pattern, intralobular interstitial thickening distortion of the lung
parenchyma
• Bilateral is present, predominantly on the left side
• .

49. 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.
• Stage 0: No demonstrable radiographic abnormality
• Stage 1: Hilar and mediastinal lymph node enlargement • without radiographic parenchymal abnormality
• Stage 2: Hilar and mediastinal. lymph node enlargement plus parenchymal abnormality
• Stage 3: Parenchymal abnormality alone
• Stage 4: Advanced fibrosis
• BAL :- lymphocytosis CD4 : CD8 > 3.5 is most specific
• PFT :- Restrictive pattern But Obstructive component present in many
• Biopsy :- non caseating granulomas lymphocytosis
• Sr. ACE levels:Hyper calciuria or Hypercalcemia

50. D. POLYMYOSITIS AND
DERMATOMYOSITIS
• ILD occurs in ∼10% of patients with polymyositis and dermatomyositis (PM/DM).
• • Diffuse reticular or nodular opacities with or without an alveolar component occur radiographically,
with a predilection for the lung bases.
• • ILD occurs more commonly in the subgroup of patients with an anti–Jo-1 antibody that is directed to
histidyl tRNA synthetase.
• • Weakness of respiratory muscles contributing to aspiration pneumonia may be present.
• • A rapidly progressive illness characterized by diffuse alveolar damage may cause respiratory failure.

51. F. RADIATION PNEUMONITIS
• evident 6 weeks to 6 months following radiotherapy
• radiographs show alveolar opacities that generally conform to the treatment portals.
• In most cases, the pneumonitis is asymptomatic
• If symptomatic, characterized by the abrupt onset of fever, cough, and dyspnea.
• Managed with supportive care in conjunction with Glucocorticoids.

53. PROGNOSIS
• Some forms of interstitial lung disease resolve completely, while others lead to long-
term and irreversible scarring and lung damage with accompanying respiratory
failure .
• Pulmonary hypertension can develop in cases of longstanding interstitial lung
disease and can lead to cor pulmonale
• The prognosis is dependent upon the type and severity of interstitial lung disease as
well as the underlying health status of the patient.

54. PREVENTION
• Avoid or limit exposure to toxins or treatments that can lead to ILD
• Proper diet and exercise reduces chance of developing ILD.
• Quitting smoking and avoiding exposure to substances known to cause ILD can prevent
the disorder from developing or worsening.
• People who are employed in jobs where they may be heavily exposed to known causes
of lung disease in the workplace typically should undergo routine screening for lung
disease.

55. REFERENCES
• Harrison’s_Principles_of_Internal_Medicine,_Twentieth_Edition_(Vol.1_&_Vol.2
• Davidsons Principles and Practice of Medicine 23rd ed

56. •Thank You