A seminar on Interstitial Lung Disease

1. Interstitial Lung Disease
Dr Aditya Nag

2. • The term interstitial lung disease (ILD) encompasses a large
group of > 200 parenchymal pulmonary disorders, of which
the majority are classified as rare that tend to be grouped
together because they share clinical, radiographic, and
pathologic features.
• These disorders are sometimes called diffuse parenchymal
lung disease (DPLD) to make the point that the interstitium is
not the only compartment of the lung affected.
INTRODUCTION

3. • Although these conditions are rare, a proportion of patients with
interstitial lung diseases (ILDs) may develop a progressive-fibrosing
phenotype. Progressive fibrosis is associated with worsening respiratory
symptoms, lung function decline, limited response to immunomodulatory
therapies, decreased quality of life and, potentially, early death.
• IPF is the most widely studied and most common ILD. It is characterized by
progressive fibrosis, lung scarring and a radiological pattern known as
usual interstitial pneumonia (UIP)

4. • There are a number of clinical and mechanistic parallels between IPF and
other fibrosing ILDs that may present a progressive phenotype . Given their
overlapping clinical, radiological and pathological presentations, the
terminology recently used to describe patients with fibrosing ILDs that may
present a progressive phenotype, despite currently available treatment, is
“progressive-fibrosing ILD (PF-ILD)”
• Progressive fibrosis of the lung parenchyma is self-sustaining and causes
progressive deterioration in lung function, respiratory symptoms and
quality of life. It therefore increases the risk of early death. Pulmonary
fibrosis is sometimes regarded as an indicator of disease progression, and
the prognosis associated with an ILD is generally worsened by its presence.

5. • ILD can be called progressive if patients meet any of the following criteria
within a 24-month period: a relative decline of ≥10% in forced vital
capacity (FVC); a relative decline of ≥15% in diffusing capacity of the lung
for carbon monoxide (DLCO); or worsening symptoms or a worsening
radiological appearance accompanied by a ≥5–<10% relative decrease in
FVC

6. CLASSIFICAT
ION
Several classification schemes for ILD have been
proposed.
• By Histopathologic & Clinical characteristics
• American Thoracic Society (ATS)/European
Respiratory Society (ERS) consensus panel
classification system (2001)

7. Types of interstitial lung disease (ILD) most likely to have a progressive-fibrosing phenotype
(indicated in bold).
Vincent Cottin et al. Eur Respir Rev 2018;27:180076
©2018 by European Respiratory Society

8. CAUSES
• Occupational and environmental factors
 Silica dust
 Asbestos fibers
 Grain dust
 Bird and animal droppings
 Radiation treatments
• Medications
 Chemotherapy drugs: methotrexate and cyclophosphamide
 Anti arrythmic medications: amiodarone or propranolol
 Antibiotics. Nitrofurantoin, ethambutol
 Anti-inflammatory drugs: Rituximab or Sulfasalazine

9. • Autoimmune diseases such as:
 Rheumatoid arthritis
 Scleroderma
 Dermatomyositis and polymyositis
 Mixed connective tissue disease
 Sjogren's syndrome
 Sarcoidosis

10. PATHOPHYSIOLOGY
In interstitial lung disease, the lung is affected in three ways:
 Lung tissue is damaged in some known or unknown way.
 The walls of the air sacs in the lungs become inflamed.
 Scarring (fibrosis) begins in the interstitium.
Fibrosis results in permanent loss of that tissue's ability to breathe and carry
oxygen. Air sacs, as well as the lung tissue between and surrounding the air
sacs, and the lung capillaries, are destroyed by the formation of scar tissue.

11. RISK FACTORS
•Age: Much more likely to affect adults, although infants and children
sometimes develop the disorder.
•Exposure to occupational and environmental toxins. Persons working in
mining, farming or construction or for any reason are exposed to pollutants
known to damage lungs, are at higher risk
•Gastroesophageal reflux disease
•Smoking
•Radiation and chemotherapy

12. CLINICAL
HISTORY
• Typical presentation of ILD -nonspecific
• Dyspnea on exertion or dry cough & abnormal chest
radiograph.
• Symptoms are usually progressive.
• two-thirds of patients with ILD are over 60 years of
age at diagnosis.

13. • H/o wheezing- hypersensitivity pneumonitis,
eosinophilic pneumonia or sarcoidosis.
• H/o pleuritic chest pain- serositis in a patient with CTD,
or pneumothorax from Lymphangioleiomyomatosis
(LAM)
• Hemoptysis- diffuse alveolar hemorrhage

14. Time Course of Disease
Onset
Acute:
• Cryptogenic organizing pneumonia (COP)
• Acute eosinophilic pneumonia (AEP)
• Acute hypersensitivity pneumonitis
• Diffuse alveolar hemorrhage
• Acute interstitial pneumonia (AIP)
• Acute exacerbation of idiopathic pulmonary fibrosis
or other ILDs

15. Subacute to Chronic
• Connective tissue disease–associated ILD
• Idiopathic pulmonary fibrosis (IPF)
• Sarcoidosis
• Chronic hypersensitivity pneumonitis (CHP)
• Occupational lung disease
• Nonspecific interstitial pneumonia (NSIP)
• Desquamative interstitial pneumonitis (DIP)
• Respiratory bronchiolitis interstitial lung disease (RB-
ILD)
• Lymphocytic interstitial pneumonia (LIP)

16. SYSTEMIC
SYMPTOMS
• Connective tissue disease is a frequent cause of ILD
• Nonspecific symptoms such as night sweats, fever,
fatigue, or weight loss suggest an underlying
inflammatory condition.

17. • Increasing edema, syncopal events, or exertional
chest discomfort may indicate severe pulmonary
hypertension.
• presence of palpitations or syncope in a patient with
sarcoidosis - Cardiac sarcoidosis.
• pleuritic chest pain, leg swelling & increasing
dyspnea- consideration of acute pulmonary embolism.
OTHER SYMPTOMS

18. PAST MEDICAL
HISTORY
• Prior diagnosis of connective tissue disease
• Case of HIV disease- lymphocytic interstitial
pneumonia (LIP) are common.
• H/o acute or chronic kidney disease might suggest
underlying vasculitis, pulmonary– renal
syndromes, or CTD.
• H/o liver disease could suggest sarcoidosis, primary
biliary cirrhosis.

19. OCCUPATIONAL
HISTORY
• Inorganic
Exposure
• Organic
Exposure

20. Inorganic
Exposure

21. Organic
Exposure

22. MEDICATION
HISTORY
• Nitrofurantoin
• Amiodarone
• NSAIDs
• h/o recent chemotherapy
• h/o immune-modulating drug use

24. PHYSICAL
EXAMINATION
• Typical‘velcro’ crepts - IPF, crepts frequently absent in
sarcoidosis.
• Inspiratory squeaks – COP.
• Clubbing- IPF, DIP, IBD.
• Skin involvement- Sarcoidosis, CTD, Vasculitis
• Arthritis- CTD, sarcoidosis.
• Uveitis, conjunctivitis- Sarcoidosis, CTD.
• Muscle weakness- Polymyositis, dermatomyositis.
• Neuropathy- Sarcoidosis, CTD.
• Lymphadenopathy- Sarcoidosis, CTD.

25. CHEST
IMAGING
• Abnormal chest radiograph is often the first
indication of underlying ILD.
• Normal : Sarcoidosis, CTD, RB-ILD

26. Diagrams illustrating the four types of
ILD.

27. Cardiogenic edema and Kerley lines. A: PA chest radiograph shows an enlarged cardiac silhouette
and bilateral reticular and linear ILD. B: Close-up view of (A), lower right lung, shows short, linear
opacities perpendicular to the lateral pleural edge, representing Kerley B lines. C: Close-up of (A), right
upper lung, shows linear opacities (arrow) radiating outward from the hila, representing Kerley A
lines. D: CT shows interlobular septal thickening (arrows), representing Kerley lines.

28. Diagnosis of fibrosing interstitial lung diseases (ILD) that may present a progressive phenotype.
Vincent Cottin et al. Eur Respir Rev 2018;27:180076
©2018 by European Respiratory Society

29. HRC
T• More sensitive than chest radiograph
Radiographic Characteristics of the UIP Pattern
“Definite UIP”
• Peripheral, subpleural distribution
• Basilar predominance
• Reticular markings and traction bronchiectasis
• Honeycombing
• Absence of inconsistent features

30. • peripheral reticular markings
• small subpleural
cysts/honeycombing Idiopathic

31. • Ground-glass opacities in a peripheral
distribution
• reticular markings
• Subpleural sparing is evident

32. • Ground-glass opacities and areas of consolidation
• A 58-year-old woman with organizing pneumonia (OP)
secondary to radiation for breast cancer

33. Nonspecific interstitial pneumonia: high-resolution computed tomography images from a 46-
year-old male patient who underwent lung transplantation. a) The initial scan, taken at first
admission, shows ground-glass attenuation and consolidation with reticulat...
Vincent Cottin et al. Eur Respir Rev 2018;27:180076
©2018 by European Respiratory Society

34. Distribution of
ILD

35. Pattern of
ILD

37. LABORATORY
TESTING
• Elevated liver enzymes or hypercalcemia-
Sarcoidosis
• Renal insufficiency- Pulmonary renal
syndromes-
• Peripheral eosinophilia- chronic eosinophilic
pneumonia, Churg–Strauss syndrome, drug reaction.

38. Serologic
testing

39. PULMONARY FUNCTION
TESTS
• Most forms of ILD demonstrates a restrictive
ventilatory defect due to decreased compliance and
increased recoil of the lung parenchyma.
• Presence of obstruction suggests either concomitant
obstructive lung disease, or the presence of an airway-
centered lung ILD such as LAM or sarcoidosis.

40. BRONCHOSC
OPY• Useful in the diagnosis of DPLD.
• inspection of the upper and lower airways,
bronchoalveolar lavage (BAL), and the
performance of transbronchial lung biopsy.
BAL:
• Cell count and differential,
• Cytology,
• Viral assays
• Microbiologic cultures

41. • bloody lavage specimens- diffuse alveolar
hemorrhage
• milky white BAL fluid- pulmonary alveolar
proteinosis
• BAL eosinophilia (>25%)- acute eosinophilic
pneumonia
• BAL lymphocytosis - granulomatous ILD, suggestive
of hypersensitivity pneumonitis, drug reaction, or
cellular NSIP

42. SURGICAL LUNG
BIOPSY
• Despite a high yield in certain forms of lung disease, the
utility of transbronchial biopsy for most of the IIP (such
as IPF, NSIP, and LIP) is low and surgical biopsy is often
required for accurate diagnosis.
• The usual technique is video-assisted thoracoscopic
surgery (VATS) that has a low morbidity and mortality
in selected populations.

43. TREATME
NT
• REMOVAL FROM EXPOSURES
• IMMUNOSUPPRESSIVE THERAPY
• ANTIFIBROTIC DRUGS
• TREATMENT OF COMORBIDITIES
• PALLIATIVE CARE
• LUNG TRANSPLANTATION

44. Treatment objectives in
ILD
1. Provide symptom-relief
2. Slow down disease progression
3. Prevent complications
4. Improve quality of life
5. Prolong survival
6. Prevent treatment-complications
7. End-of-Life care and palliative treatment

45. REMOVAL FROM
EXPOSURES
• Drug reaction is suspected- should be
discontinued
• mold growth, removal of birds from the home, extensive
cleaning of upholstery, window coverings, and ventilation
systems.
• occupational exposures- avoided

46. IMMUNOSUPPRESSIVE
THERAPY• Some forms of ILD, including COP, CTD– associated
ILD, and sarcoidosis, shows favorable response to
steroids and other immunosuppressive agents.
• when a more prolonged course of therapy is
anticipated, azathioprine or cyclophosphamide, permit
low dose of steroids.
• If no clinical improvement is seen after 3 to 6 months
of therapy, discontinuation of immunosuppressive
therapy should be strongly considered.

47. ANTIFIBROTIC
DRUGS
• Useful in progressive fibrotic lung diseases.
 Pirfenidone: a small-molecule drug which have
antifibrotic properties
 Nintedanib: a tyrosine kinase inhibitor, is one of
two drugs approved for the treatment of IPF. In
clinical trials, Nintedanib slowed disease
progression by reducing the rate of decline in
forced vital capacity (FVC) in patients with IPF
and mild or moderate lung function impairment

48. • Most patients with ILD referred for lung
transplantation have IPF- advanced stage.
• a severely impaired DLCO (<39%) as well as
advanced fibrosis on HRCT predict poor survival and
are considered to be triggers for active listing.
• Early referral to a lung transplant center is useful
LUNG TRANSPLANT

49. Thank You