Interstitial lung disorders

1. APPROACH TO INTERSTITIAL LUNG
DISEASE
Chair person – Dr. R.P. BUDEN
Co-chaiperson – Dr. BHARATH
Presenter- Dr.Sharan
Junior resident
General Medicine

2. Interstitial Lung Disease (ILD)
 Diffuse interstitial lung disease encompasses a group of disorders of
different etiologies with generalized involvement of the lung interstitium
 In includes over 200 different diseases which inspite of their heterogenous
nature ,have several common clinical,radiological and hpe manifestation
 Interstitium - space between the epithelial and endothelial basement
membranes in the lung

3. • In general, ILD implies the clinical manifestations of
inflammatory-fibrotic infiltration of the alveolar walls(septa)
resulting in profound effects on the capillary endothelium and
the alveolar epithelial lining cells.
• Interstitial fibrosis follows injury to the gas exchanging units,
that increases alveolar permeability, enabling the serum
contents to enter the alveolar spaces resulting in airspace
abnormalities in addition to interstitial changes.

4.  Global incidence 30-100 per 1,00,000 people per year with about 1/3 to
1/2 in the idiopathic category
 In India, ILD constitutes about 10-15% of patients with respiratory diseases
examined at any large hospital
 50% are idiopathic
 Chronic hypersensitivity pneumonia and sarcoidosis were found be the
most common causes,respectively ,in 2 large reports from india
EPIDEMIOLOGY

6. Presentation, diagnosis and
clinical course of the spectrum
of progressive-fibrosing
interstitial lung diseases
European Respiratory
Review 2018

7.  HISTORY
• AGE : >60yrs
20-40yrs: Sarcoidosis, CTD associated ILD, LAM, PLCH.
• SEX:
CTD associated ILDs are more common among women,
with the exception of RA associated ILD among men.
LAM more common in young women.
IPF and occupational/ exposure related ILDs are more common among men
CLINICAL FEATURES OF ILD
•Past medical history- CTD, Malignancy, Asthma & Allergic rhinitis

8. MEDICATION HISTORY
ANTIBIOTICS
NITROFURANTOIN
MINOCYCLINE
CEPHALOSPORINS
CHEMOTHERAPEUTICS
ATRA
ANTITHYMOCYTE GLOBULIN
BLEOMYCIN
BUSULFAN
CARMUSTINE
CHLORAMBUCIL
CYCLOPHOSPHAMIDE
CYTOSINE ARABINOSIDE
DOCETAXEL
OTHERS
BCG
MINERAL OIL
RADIATION
GEFTINIB
GEMCITABINE
IL2
IRINOTECAN
MELPHALAN
MITOMYCIN C
PACLITAXEL
PROCARBAZINE
VINORELBINE
NEUROLOGICAL/PSYCHIATRIC
CARBAMAZEPINE
PHENYTOIN
ANTIARRHYTHMIC
AMIDARONE
TOCAINIDE
ANTI INFLAMMATORY
AZATHIOPRINE
ETANERCEPT
GOLD SALTS
INFLIXIMAB
METHOTREXATE
NSAIDS
PENICILLAMINE
SULFASALAZINE

9. • Family history-
- The percentage of pulmonary fibrosis that is familial ranges from 5-20%
- Presence of a close relative with an IIP is among the strongest risk factors
for IPF
- Cystic lung diseases, LAM with mutation in TSC genes
• Environmental history-
Cigarette smoke in DIP, RB-ILD, LCH, IPF

10. Occupational history-
ORGANIC EXPOSURE
• Asbestosis- electrician, plumber, pipe fitter, construction workers, ship builder, insulation
installer
• Silicosis- stone cutter, miner, sand blaster
• Giant cell interstitial pneumonia, hard metal lung disease- metal grinder
• Berryliosis- metal worker, factory worker. Nuclear weapon, artifact, electronics, ceramics, golf
clubs, bicycle farmers
• Coal worker’s pneumoconiosis- coal worker
• Chemical worker’s lung- paint sprayer, plastic worker
INORGANIC EXPOSURE
• Bird breeder’s lung- bird breeder
• Farmer’s lung- haying
• Humidifier lung- office worker
• Hot tub HP- Lifeguard

11. Duration of symptoms
• Acute presentations (days to weeks) of ILD are unusual and are commonly
misdiagnosed as more common diseases such as pneumonia, a chronic
obstructive pulmonary disease (COPD) exacerbation, or heart failure.
➢ Cryptogenic organizing pneumonia
➢ Acute eosinophilic pneumonia
➢ Acute hypersensitivity pneumonitis
➢ Diffuse alveolar hemorrhage
➢ Acute interstitial pneumonia
➢ Acute exacerbation of idiopathic pulmonary fibrosis or other ILDs
ACUTE ILD

12. ➢ Idiopathic pulmonary fibrosis
➢ Sarcoidosis
➢ Chronic hypersensitivity pneumonitis
➢ Occupational lung disease
➢ Nonspecific interstitial pneumonia
➢ Smoking associated ILDs
➢ Lymphocytic interstitial pneumonia
➢ Chronic eosinophilic pneumonia
SUBACUTE AND CHRONIC ILD
➢ Eosinophilic pneumonia
➢ Hypersensitivity
pneumonitis
➢ COP
➢ Vasculitides
➢ Pulmonary hemorrhage
EPISODIC ILD

13. SYMPTOMS-
• Chronic non productive cough with progressive
exertional dyspnoea(most common presentation)
• Substernal chest pain- frequent in sarcoidosis.
• Plueritic chest pain- serositis in patients with CTD or pneumothorax
in cystic lung diseases like LAM, LCH.
• Hemoptysis- Diffuse alveolar hemorrhage(Good Pasture’s
Syndrome, GPA, LAM).
• Fatigue is common to all ILDs.
• Wheezing -uncommon manifestation(found in eosinophilic
pneumonia, Churg-Strauss, respiratory bronchiolitis and
sarcoidosis)

14. SYSTEMIC SYMPTOMS
• Night sweats, fever, fatigue, weight loss
• Heliotrope rash, Gottron’s papules/ mechanic’s hands- Dermatomyositis
• h/o skin tightness & thickening, telengectasias, Raynaud’s phenomenon,
digital pitting- Scleroderma
• Papular eruptions, lupus pernio, erythema nodosum- Sarcoidosis
• Malar rash, photosensitivity, skin reaction, hair loss- SLE
• GI Symptoms- systemic sclerosis, polymyositis
• Chronic intermittent aspiration- progressive fibrotic lung disease
• Bloating, diarrhoea- systemic sclerosis

15. • Musculoskeletal-
Arthralgia, morning stiffness, joint swelling, erythema, deformities-
RA, Sjogren’s syndrome
Sausage digits- Systemic sclerosis, Polymyositis
Raynaud’s phenomenon with digital gangrene- Scleroderma, SLE, mixed CTD
• Ophthalmologic-
dry eyes- Sicca syndrome(Sjogren)
Uveitis- SLE, Sarcoidosis
• Long standing ILD- increasing edema, syncopal events, exertional chest
discomfort indicates severe pulmonary hypertension and Cor pulmonale in
advanced fibrotic lung disease and hypoxemia.

16. Examination :-
➢ Tachypnoea +/- Respiratory distress
➢ End inspiratory fine crackles- ‘Velcro’ crackles at the lung bases- earliest
signs of fibrosis
➢ Cyanosis and digital clubbing- advanced disease
➢ Wheezing, inspiratory squeaks( late inspiratory high pitched rhonchi)- HP,
Bronchiolitis, Sarcoidosis
➢ f/s/o pulmonary hypertension and cor pulmonale

17. LABORATORY STUDIES
• Leukopenia- Sarcoidosis, connective tissue disease, lymphoma, drug
induced
• Leukocytosis- systemic vasculitis, HP, lymphoma
• Eosinophilia- eosinophilic pneumonia, systemic vasculitis,
sarcoidosis, drug induced.
• Anti CCP antibody- RA, ANCA- Systemic vasculitis
• Raised LDH – common, nonspecific
• Elevated liver enzymes, hypercalcemia- sarcoidosis.
• Serum precipitins-hypersensitivity pneumonitis

19. ILD/DPLD- PFT and more
 PFT (spirometry) + DLCO) resting room air
SpO2/ABG
❖ Restrictive vs obstructive pattern useful in
narrowing diagnoses
❖ Defines severity of lung involvement in
ILD
❖ Helps in follow-up
❖ Biopsy + Therapy defining parameter
❖ Classically, ILD shows ‘Restrictive defect’
❖ Reductions in FVC, FEVI
❖ FEV1/FVC ratio is usually normal or
increased.
Diffusing capacity of carbon dioxide
(DLCO)
❖Commonly reduced
❖ V-Q mismatch + Effacement of lung
units, gas exchange area with further
destruction
❖ ABG: Hypoxemia, elevated A-a gradient
❖Hypercapnia rar- end stage disease
❖ Exercise testing can detect earlier gas-
exchange issues + better modality for
follow-up
❖CPET, 6MWT, Ex. oximetry

20. CHEST X-RAY
✓ Most common- bibasilar
reticular pattern.
✓ Others-Nodular or mixed
pattern of alveolar filling
and increased reticular
markings

21. UIP pattern
❖ Definite UIP
❖ Reticular opacities- interstitial fibrosis
❖ Basal, subpleural predominance
❖ Honeycombing with or without traction btasis
❖ Absence of other features suh as Ggos, cysts, air trapping, etc
❖ DDs:
❖ IPF
❖ CTD-RA/MCTD
❖ Asbestosis
❖ CHP

22. Diagnosis of Idiopathic Pulmonary Fibrosis
An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
Definite UIP
Probable UIP
Indeterminate for UIP
Alternative diagnosis
This was also based on Fleischner Society recommendations
DA Lynch. Fleischner Society White paper, Lancet Resp Med 2018

23. NSIP Pattern
2 types- cellular and fibrotic, usually with overlapping features
Common radiological manifestations include:
❖ Ground-glass opacities
❖ Tends to be a dominant feature
❖ Immediate subpleural sparing - a relatively specific sign
❖ Reticular opacities and irregular linear opacities (sometimes - minor subpleural reticulation)
mainly with fibrotic NSIP
❖ Thickening of bronchovascular bundles: with fibrotic NSIP
❖ Traction bronchiectasis: associated with fibrotic NSIP
❖ Lung volume loss: particularly lower lobes
❖ In advanced disease
❖ traction bronchiectasis
❖ consolidation
❖ microcystic honeycombing: a relatively less common feature

24. Hypersensitivity pneumonitis -Radiology
❖ GGOs: homogenous, may be symmetrical
❖ Ill-defined round centrilobular nodules: usually <5 mm in diameter
(occasionally these opacities have well-defined borders and soft-
tissue attenuation)
❖ Mosaic attenuation pattern- air trapping
❖ Head cheese sign: the combination of patchy ground-glass
opacities, normal regions, and air trapping

25. Sarcoidosis Radiology
❖ Common presentations:
❖ Mediastinal LNpathy
❖ Peri-bronchovascular predominance centrilobular nodules
❖ Upper and mid-zone predominance
❖ Fibrosis in later stages
❖ Less common
❖ Conglomerate masses in perihilar location
❖ Larger nodules>1cm
❖ Galaxy sign-grouped nodules surrounded by satellite nodules
❖ Ground glass nodules-alveolar sarcoidosis

26. DISTRIBUTION OF ILD
SARCOIDOSIS
SILICOSIS
COAL WORKER’S PNEUMOCONIOSIS
HYPERSENSTIVITY PNEUMONITIS
CHRONIC EOSINOPHILIC
PNEUMONIA
BERYLLIOSIS
LANGERHAN’S CELL HISTIOCYTOSIS
USUAL INTERSTIAL
PATTERN
NONSPECIFIC INTERSTIAL
PATTERN
CONNECTIVE TISSUE
RELATED ILD
ASBESTOSIS
DESQUAMTIVE INTERSTIAL
PATTERN
UPPER ZONES LOWER ZONES

27. PATTERN OF ILD
BERYLLIOSIS
LANGERHAN’S CELL HISTIOCYTOSIS
HYPERSENSTIVITY PNEUMONITIS
SARCOIDOSIS
SILICOSIS
METASTATIC DISEASE
TALCOSIS
RESPIRATORY BRONCHIOLITIS
GRANULOMATOUS POLYANGITIS
UIP
NSIP
PERIHERAL RETICULAR
NODULAR

28. LYMHANGIOLEIOMYOMATO
SIS
LANGERHAN’S CELL
HISTIOCYTOSIS
LYMPHOCYTIC INTERSTITIAL
PNEUMONIA
PNEUMOCYSTIS JIROVECI
PNEUMONIA
CYSTIC
NSIP
CRYPTOGENIC ORGANIZING
PNEUMONIA
HYPERSENSTIVITY PNEUMONITIS
EOSINOPHILIC PNEUMONIA
INFECTION
ALVEOLAR HEMORRHAGE
SARCOIDOSIS
DIP
PULMONARY ALVEOLAR
PROTEINOSIS
GROUND GLASS

29. BRONCHOSCOPY-BAL FINDONG
Condition Bronchioalveolar Lavage Finding
Sarcoidosis Lymphocytosis; CD4:CD8 ratio >3.5 most
specific of diagnosis
Hypersensitivity pneumonitis Marked Iymphocytosis (>50%)
Organizing pneumonia Foamy macrophages; mixed pattern of increased cells
characteristic; decreased
CD4:CD8 ratio
Eosinophilic lung disease Eosinophils >25%
Diffuse alveolar bleeding Hemosiderin-Iaden macrophages, RBC
Diffuse alveolar damage, drug
toxicity
Atypical hyperplastic type 2 pneumocytes
Opportunistic infections Pneumocystitis carnii, fungi,cytomegalovirus
transformed cells

30. Lymphangitic carcinomatosis,
alveolar cell carcinoma,
pulmonary Iymphoma
Malignant cells
Alveolar protienosis Milky effluent, foamy macrophages and
lipoproteinaceous intraalveolar material
(PAS stain-positive)
Lipoid pneumonia Fat globule in macrophages
Pulmonary Langerhans cell
histiocytosis
Increased CD1 + Langerhans cells, electron
microscopy demonstrating birbeck granule in
lavaged macrophage
Asbestosis Dust particles, ferruginous bodies
Berylliosis Positive Iymphocyte transformation test to beryllium
Silcosis Dust particles by polarized light microscopy
Lipoidosis specific lipopigment in macrophages

31. Surgical Lung Biopsy
• SLB is recommended for diagnosis, if no UIP pattern
• Practically, 10–20% of fibrotic ILD pts undergo a SLB
– Issues: Advanced age, comorbidity, severe resp failure, PH
– Many do not consent for surgical biopsy.
• Stay: Hospitalization for several days
• Mortality can be observed in 2–6% at 90 days
• 30-day mortality can increase to 19%
• Patients ultimately diagnosed with IPF can have worse post-procedure outcomes than other
ILDs
• Some risk of persistent pain at 12 mths, prolonged air leak, and/or BPF (3–12%)
• long hospitalization &/or reoperatn
• Expensive….scant penetration in India

32. Cryobiopsy: The new kid on the block: Characteristics of transbronchial
cryobiopsy (TBCB)
Large samples (usually 5 mm diam) ,typically 20-100 alveoli
*Well preserved lung specimens/IHC feasible
*Complex morphologic patterns (i.e. UIP pattern) may be
identified
*Greater concurrence between pathologists
“ Transbronchial cryobiopsy might, therefore, be considered
an alternative to surgical lung biopsy in patients with DPLDs
and particularly in subjects with f-DPLDs” - Poletti et al,
Respirology (2014) 19, 645

33. UIP PATTERN
 Subpleural reticulation associated
with honeycomb changes
alternating with areas of preserved
normal lung architecture referred to
as temporal and spatial
heterogeneity
 Fibroblast foci, which are
subepithelial collections of
myofibroblasts and collagen

34. NSIP PATTERN
 varying amounts of interstitial
inflammation and fibrosis with a
uniform appearance
 Honeycomb changes are usually
absent
 fibroblast foci are rare.
 NSIP is often referred to
histopathologically as being either
predominantly cellular or fibrotic.

35. This is a primary option in ILD/DPLD
• Transbronchial biopsy
• Cryobiopsy (TBLC)- (transbronchial lung cryobiopsy)
• VATS (video assisted thoracoscopic surgery
* A definite role for Cryobiopsy in non-UIP, non CTD-ILD pts
* Primary role before surgical biopsy, with SLB for failed cryobiopsy

36. General recommendations
❖ No SLB/Cryo if
❖ HRCT chest- definite UIP and clinical possibility is IPF
❖ CTD-related ILD
❖ HRCT Sarcoidosis/suggestive of HP + typical exposure history
❖ Contraindications: Significant hypoxemia, severe PH, FVC low
❖ Biopsy can be considered in all others after a careful deliberation
❖ Surgical lung biopsy:
❖Early ILD, undifferentiated pattern
❖Peripheral predominant fibrotic ILD (to differentiate between fibrotic NSIP and IPF) – Can go either
way
❖Patient preference, inconclusive Cryobiopsy
❖ Others: Cryobiopsy
❖ Borderline patients: Cryobiopsy

37. Treatment aspects
1.Removal from exposures
discontinue the drug,remove birds, clean window coverings and ventilation system
remove patient from that location
alter patient’s job or use protective respiratory equipment
2.Identification of the Underlying Cause
3. Medications:
- Corticosteroids
- Immunosuppressants
- Antifibrotic drugs: Nintedanib and pirfenidone

38. 4. Oxygen Therapy
5. Pulmonary Rehabilitation
6.Symptom Management/Palliation:
- Morphine
- Cough suppressants
- Managing adverse effects related to ILD drugs
7. Vaccination
8. Lung Transplantation: Few centers in the country, severely impaired DLCO
(<39%) with advanced fibrosis on HRCT
9. Supportive Care

39. GLUCOCORTICOIDS
 Prednisone 0.5 to 1 mg/kg ideal body weight per day up to a maximum of
60 mg/day for one month followed by 30 to 40 mg/day for an additional
two months
 For patients who respond or stabilize with treatment, the prednisone dose
is gradually tapered to reach 5 to 10 mg daily or on alternate days, by the
end of six to nine months
 For patients who relapse when prednisone is tapered or discontinued, low-
dose prednisone can be maintained for a longer period .
 Or, if relapse occurs at higher prednisone doses, another
immunosuppressive agent can be added as a glucocorticoid-sparing agent

40. Pulse methylprednisolone — For patients with severe NSIP
requiring hospitalization.
 A typical regimen is 1000 mg/day for three days followed by
oral prednisone
Additional immunosuppressive drugs — For patients with
more severe initial disease or an inadequate response to or
intolerance of glucocorticoids, therapy is usually expanded to
include an additional immunosuppressive drug, such as
azathioprine or mycophenolate

41. • Cyclophosphamide, calcineurin inhibitors, and rituximab are
usually reserved for refractory disease that has not responded
to prednisone in combination with azathioprine or
mycophenolate.

42. Pirfenidone
 Antifibrotic agent inhibits transforming growth factor beta (TGF-b)
 The dose ranges up to 40 mg/kg per day (to maximum of 2403 mg per day) in
three divided doses.
 Pirfenidone is initiated at a dose of 267 mg (1 capsule) three times a day. After
one week, the dose is increased to 534 mg (two capsules) three times a day, and
after the second week to the full dose of 801 mg (three capsules) three times a
day. Pirfenidone should always be taken with food.
 Side effects include rash (30 percent), photosensitivity (9 percent), nausea (36
percent), diarrhea (26 percent), abdominal discomfort (24 percent), dyspepsia
(19 percent), anorexia (13 percent), and fatigue (26 percent)

43. Nintedanib
 Nintedanib — Nintedanib may reduce the rate of disease progression in
patients with idiopathic NSIP who develop progressive fibrosis.
 Nintedanib has been approved by the US Food and Drug Administration
(FDA) and the European Medicines Agency (EMA) for use in patients with
idiopathic pulmonary fibrosis (IPF), systemic sclerosis-interstitial lung
disease (SSc-ILD), and progressive chronic fibrosing ILD of other origin
(eg, rheumatoid arthritis, fibrotic hypersensitivity pneumonitis) .
 The role of nintedanib in NSIP remains ill-defined,

44. The standard dose 150 mg BD
Liver function tests (LFTs; ALT, AST, bilirubin) should be
assessed prior to initiation of nintedanib;
Patients with moderate or severe hepatic impairment (Child
Pugh B or C) should not take nintedanib
After initiation, LFTs should be repeated monthly for three
months, every three months thereafter, and as clinically
indicated.

45. IDIOPATHIC PULMONARY FIBROSIS
CLINICAL SYMPTOMS Gradual onset of SOB ,Dry cough
Unusual in older adults
PHYSICAL EXAMINATION Frequent rales at lung bases
Digital clubbing is common
EXPOSURE Idiopathic ,but may be exposed to smoking
Genetics findings may expplain moe than 1/3 of
the risk of the disease
HRCT UIP pattern
HPE UIP pattern
CLINICAL COURSE 50% 3-5 yr mortality

46. NONSPECIFIC INTERSTITIAL PNEUMONIA
CLINICAL SYMPTOMS Subacute onset of SOB,dry cough
Frequently associated with other condition
PHYSICAL EXAMINATION Frequent rales,Clubbing is less common
EXPOSURE Can be idiopathic
But shoulf prompt considertion for other associated
condition
HRCT NSIP pattern
HPE NSIP pattern
CLINICAL COURSE 18% 5 year mortality

47. • Primarily idiopathic but the morphological pattern can be seen in association with a
number of conditions:
• Connective tissue disorders
Systemic lupus erythematosus (SLE)
Scleroderma
Sjögren syndrome
Polymyositis
Dermatomyositis
• Other autoimmune diseases
Rheumatoid arthritis
Primary biliary cirrhosis
Hashimoto thyroiditis
Antisynthetase syndrome

48. • Drug-induced lung disease: especially chemotherapy agents – thalidomide
• Hypersensitivity lung disease
• Slowly healing diffuse alveolar damage (DAD)
• Relapsing organizing pneumonia
• Immunodeficiency (mainly HIV infection)
• Graft versus host disease (GVHD)
• Immunoglobulin G4 (IgG4)-related sclerosing disease
• Multicentric Castleman disease
• Myelodysplastic syndrome
If there is no underlying cause, it is termed idiopathic NSIP; which is now considered
a distinct entity.
Smoking is neither protective nor a risk factor for NSIP

49. CLINICAL SYMPTOMS Asymptomatic or have SOB ,cough
PHYSICAL EXAMINATION Rales common,Clubbing is rare
EXPOSURE Smoking
HRCT Diffuse patchy centrilobular ground glass nodules
HPE Respiratory bronchiolitis with adjacemt inflammatory
and fibrosing changes
Pigment laden macrophages
CLINICAL COURSE 25% 7 yearmortality
RESPIRATORY BRONCHIOLITIS ASSOCIATED ILD

50. CLINICAL SYMPTOMS Gradual onset of SOB,dry cough
Fatigue, tightening of skin,Exaggerated cold response
Difficulty swallowing
PHYSICAL
EXAMINATION
Rales in isolation
Skin thickening,joint swelling,telengiectasias
EXPOSURE Unknown, ?solvent / silicate exposure
HRCT UIP/NSIP pattern
Dilated esophagus,Mediastinal calcification,Pulmonary vascular
enlargement
HPE UIP/ NSIP pattern
CLINICAL COURSE 20-30 % 10 year mortality
SYSTEMIC SCLEROSIS ASSOCIATED ILD

51. CLINICAL SYMPTOMS Asyptomatic or SOB ,Dry cough
Fatigue ,palpitation,Eye,skin and joint changes
PHYSICAL EXAMINATION Normal,rale may be present
Skin findings,joint pain and enlarged lymph nodes
EXPOSURE Unknown,?silicate dust
HRCT Mediastinal and hilar lymphadenopathy
Peribronchovascular reticular –nodular pattern
HPE Noncaseating granuloma
CLINICAL COURSE Generaly low
SARCOIDOSIS

52. ACUTE EXACERBATIONS OF IIPS
• 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%)
• mean survival periods range from as little as days to months

53. • HRCT- Newly developing diffuse ground-glass abnormality and/or
consolidation superimposed on a background reticular or honeycomb
pattern consistent with the UIP pattern
• Treatment – Supportive
o There is some evidence that drug therapy (e.g., nintedanib) may reduce
the rate of acute exacerbations in patients with IPF.
o Immunosuppressive (e.g.prednisone) & cytotoxic (e.g., cyclophosphamide)
therapies are commonly used without proven benefit.

54. • Also known as Bronchiolitis obliterans organising pneumpnia (BOOP)
• 50–60s / Male = Female
• Presents as a subacute flulike illness & cough with sputum
• COP is commonly mistaken for pneumonia.
• Associated with CTD (e.g., polymyositis) or medications, or malignancy
• PFT- Obstructive pattern
• Treatment - Steroids
CRYPTOGENIC ORGANIZING PNEUMONIA

55. • HRCT Image –
Patchy, migratory, pulmonary
consolidation with GGOs.
Central GGO surrounded by
dense consolidation –
REVERSE HALO / ATOLL SIGN
(also seen in COVID 19)

56. • HPE –
 Patchy regions of organizing
pneumonia with granulation
tissue
 Commonly involves the small
airways, alveolar ducts, and alveoli
 Masson’s bodies – Polypoid plug
(granulation tissue) in bronchiole

57. Desquamative Interstitial Pneumonia and
Respiratory Bronchiolitis-Associated ILD
• Smoking associated ILDS
• <10% of the IIPs
• 3rd and 4th decades
• Progressive dyspnea cough
• Chest X-ray often normal
• Histopathology-Accumulation of pigmented macrophages- smoker’s macrophages
• HRCT- Centrilobular ground glass opacities -upper lobe predominant, or diffuse
• Treatment
Smoking cessation
Steroid in severe cases(6 -9 months)
BRONCHODILATORS

58. • AIP is a rare and fatal lung disorder that is characterized by an acute onset of
respiratory distress and hypoxemia
• Often preceded by viral URTI
• Hamman crunch sound due to mediastinitis
• The mortality rate within 6 months of presentation is high (>50%), and recurrences
are common.
• Difficult to distinguish from ARDS and acute exacerbation of IPF
• Treatment - supportive and often includes mechanical ventilation,there is no proven
drug therapy for AIP.
ACUTE INTERSTITIAL PNEUMONIA
(HAMMAN-RICH SYNDROME)

59. PULMONARY LYMPHANGIOMATOSIS(LAM)
• Young female,non smoker
• Associated with Tuberous
sclerosis,Angiomyolipoma,recurrent
pneumothorax and chylous effusion
•
• HRCT - Multiple uniform cyst in lower
lobe
• Treatment - Evorolimus

60. PULMONARY LANGERHAN’S CELL HISTIOCYTOSI (LCH)
• Young male , smoker
• Recurrent spontaneous
pneumothorax
• HRCT – Bilateral centrilobular
nodules and cyst predominantly
in upper lobe
• Treatment - steroids

61. LYMPHOID INTERSTITIAL PNEUMONIA (LIP)
• WOMEN / 5th decade
• Slowly progressive cough and dyspnea over years
• Variant of NSIP
• Lymphadenopathy, Arthritis .
• Associated with Sjogren syndrome , HIV and common
variable immuno deficiency
• HRCT – GGO and cyst

62. EOSINOPHILIC PNEUMONIA
PRIMARY PULMONARY EOSINIPHILIC SYNDROMES-
 Acute eosinophil ic pneumonia
 Chronic eosinophilic pneumonia
 Eosinophilic granulomatosis with polyangiitis (Churg-
Strauss syndrome)
 Hypereosinophilic syndrome
PULMONARY DISEASES OF KNOWN CAUSE
 Asthma and eosinophilic bronchitis
 Allergic bronchopulmonary aspergillosis
 Drug/toxin reaction
 Infection

63. LUNG DISASES ASSOCIATED WITH EOSINOPHILIA-
 Cryptogenic organizing pneumonia
 Hypersensitivity pneumonitis
 Idiopathic pulmonary fibrosis
 Pulmonary Langerhans cell histiocytosis
SYSTEMIC DISASES ASSOCIATED WITH EOSINOPHILIA-
 Postradiation pneumonitis
 Rheumatoid arthritis
 Sarcoidosis
 Sjögren's syndrome

64. Acute eosinophilic pneumonia
 Males (20 and 40 years) with no history of asthma.
 Smokers (after restarting smoking)
 Presentation-ARDS like
 Cough, dyspnea, malaise, myalgias, night sweats, pleuritic chest pain
 7 to 30 days -peripheral eosinophilia (mean eosinophil counts of 1700)
 ESR,CRP, and IgE levels high
 Treatment-Steroids
 Complete clinical and radiographic recovery without recurrence or residual sequelae

65. • A febrile illness of short duration (one month or less, but often less than
one week)
• Hypoxemic respiratory failure (eg, pulse oxygen saturation [SpO2] <90
percent on room air or arterial oxygen tension [PaO2] <60 mmHg)
• Diffuse pulmonary opacities on chest radiograph
• BAL differential cell count showing eosinophilia >25 percent
• Absence of known causes of eosinophilic pneumonia, including drugs,
infections, asthma, or atopic disease
Diagnostic criteria for acute eosinophilic pneumonia

66. Chronic eosinophilic pneumonia
 Pulmonary infiltrates and eosinophilia in both the tissue and blood.
 Female non smokers mean age 45 years
 Majority have asthma, history of allergies
 cough ,low-grade fever, progressive dyspnea, weight loss, wheezing,
malaise and night sweats
 CXRay – “Photographic negative pulmonary edema“/ reverse bat
wing appearance on (<25% patients)
 Peripheral eosinophilia-mean eosinophil >30% of TLC
 BAL eosinophil >40%
 Prolonged courses of corticosteroids for months to year
 Relapses present

67. HYPEREOSINOPHILIC SYNDROME
• Persistent elevation of eosinophils >1500 cells/mcl for >6
months with end organ damage like myocarditis,peripheral
neuropathy etc
• No infiltrates in lung
• Steroids - not responsive

68. Interstitial Pneumonia with Autoimmune Features (IPAF)
 A significant proportion of patients with interstitial lung disease (ILD) manifest
autoimmune features, but do not fulfill the diagnostic criteria for a definite
connective tissue disease (CTD)
 In 2015, ERS/ATS task force proposed classification criteria for IPAF
 These classification criteria were based on a combination of features from
three domains:
❖ Clinical domain consisting of extra-thoracic features;
❖ Serologic domain with specific autoantibodies;
❖ Morphologic domain with imaging patterns,

69. UPDATE: PF-ILD  PPF!
Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and
Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice

70. Anti-synthetase Syndrome(AS)
❖ Uncommon and under-recognized CTD
❖ Presence of antibodies to anti-aminoacyl t-RNA synthetase
❖ Features of interstitial lung disease (ILD), myositis and
arthritis.

73. • This guideline provides evidence-based recommendations for the treatment of
Interstitial Lung Disease (ILD) in adults with Systemic Autoimmune Rheumatic
Diseases (SARDs) at greatest risk of ILD, including systemic sclerosis (SSc), rheumatoid
arthritis (RA), idiopathic inflammatory myopathies (IIM including polymyositis,
dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy),
mixed connective tissue disease (MCTD), and Sjögren's Disease (SjD)

74. Summary of recommendations for screening of SARD-ILD
• For people with SARDs at increased risk of developing ILD, conditionally recommend
screening with
PFTs
HRCT of the chest
HRCT chest and PFTs over PFTs alone.
• For people with SARDs at increased risk of developing ILD, conditionally recommend against
screening with
6MWD
Chest radiography
Ambulatory desaturation testing.
Bronchoscopy.
Surgical lung biopsy.

75. Summary of recommendations for monitoring of SARD-ILD
• For people with SARDs at increased risk of developing ILD, conditionally recommend
monitoring with
PFTs
HRCT of the chest
HRCT chest and PFTs over PFTs alone
Ambulatory desaturation testing
• For people with SARDs at increased risk of developing ILD, conditionally recommend against
monitoring with
6MWD
Chest radiography
Bronchoscopy.

76. • For people with IIM-ILD and SSc-ILD, PFTs for monitoring every 3-6
months rather than either shorter or longer intervals, for the first
year, then less frequently once stable.
• For people with RA-ILD, SjD-ILD, and MCTD-ILD, PFTs for
monitoring every 3-12 months rather than shorter or longer
intervals, for the first year, then less frequently once stable.
• For people with SARDs-ILD, no guidance about frequency of
routine HRCT chest for monitoring ILD but suggest HRCT when
clinically indicated.

77. Summary of Recommendations for Management of SARD-
ILD: First-line ILD Treatment

78. Recommendations for Management of SARD-ILD
Progression Despite First-line ILD Treatment

79. Recommendations for Management of SARD with
Rapidly Progressive ILD (RP-ILD)

80. Pulmonary Rehabilitation
Exercise
training
Patient
Education
Psychosocial
support
Nutritional
counselling
Individualised
care
Breathing
retraining and
Techniques
Behavioural
support
Continuous
monitoring
and Follow up

81. Multi-Disciplinary Diagnosis (MDD)
❖ Rationale:
❖ More than 200 types of DPLDs described with overlapping features and varied prognosis
❖ Diagnosing with highest degree of confidence may avoid biopsy, esp. in elderly & those
with multiple comorbidities
❖ In association with pulmonologist, pathologist, radiologist and rheumatologist
❖ Two of the largest, recent studies suggest:
❖ In patients with working diagnosis, MDD leads to change in diagnosis > 40% patients
❖ In patients without working diagnosis, MDD gives a confident diagnosis in 45-80%
patients
Laurens J. De Sadeleer et al. Chest 2018
Grewal et. al. Annals of American Thoracic Society 2019

82. ILD..knowledge, understanding and approach challenging for both
patient and provider
Interstitial Lung Disease

83. References-
1. Fishman’s Pulmonary Diseases and Disorders 5th edition
2. Harrison’s Principles of Internal Medicine 19th Edition
3. API textbook of Medicine 10th Edition
4. Crofton’s and Douglas’s Respiratory Disease 5th Edition
5. Murray & Nadel’s textbook of Respiratory medicine –
6th edition

84. THANK YOU