interstitial lung disease (ilD)

interstitial lung disease
ild

1. INTERSTITIAL LUNG
DISEASE
Mohammed Jamal
Guided By : Dr hasmik

2. Definition & Background
Interstitial lung disease (ILD) is a common term that includes more
than 150 chronic lung disorders. When a person has ILD, the
lung is affected in three ways. First, the lung tissue is damaged
in some known or unknown way. Second, the walls of the air
sacs in the lung become inflamed. Finally, scarring (or fibrosis)
begins in the interstitium(or tissue between the air sacs), and
the lung becomes stiff.
When the interstitiumbecomes scarred and thickened, it is much
more difficult for oxygen to travel from the air into the
bloodstream. Patients with interstitial lung disease, then, will
develop symptoms which are the result of lung malfunction,
like shortness of breath and cough.

3. The terms interstitial lung disease, pulmonary fibrosis, interstitial
pulmonary fibrosis, and diffuse parenchymal lung diseaseoften
used to describe the same condition.
Classification:
“Known” vs. “Unknown”
-”without” or “with” granulomas

4. Examples of KnownEtiology: withoutgranulomas
ARDS
Occupational and environmental inhalants
(e.g. asbestos, gases, aerosols)
Drugs
Aspiration
Radiation
Lymphangitic spread of carcinoma

5. Examples of KnownEtiology: withgranulomas
Occupational and environmental inhalants
-Beryllium
-Hypersensitivity pneumonitis (an allergic disorder)
-Talc
Infectious agents
-AFB (Acid-fast bacillus)
-Fungi

6. Examples of UnknownEtiology: without
granulomas
Ideopathic Interstitial Pneumonias (IIP):
Vasculitis
Pulmonary hemorrhage syndromes
Eosinophilic infiltration
Ankylosising spondylitis

7. Examples of UnknownEtiology: withgranulomas
Sarcoidosis
Histiocytosis-X
ANCA (+) Granulomatos vasculitis
-Wegener’s granulomatosis
-Churg-Strauss
-Lymphomatoid granulomatosis

8. Clinical Assessment
Check medication
Employment
Systemic Illnesses
Exposures

9. Signs & Symptoms
The most common symptoms are shortness of breath
with exercise and a non-productive cough. Some patients
may also have fever, weight loss, fatigue, muscle and
joint pain, and abnormal chest sounds,
depending upon the cause.
Clubbing can also be seen.

10. -PFT usually shows a restrictive pattern
-The FEV1/FVC ratio is usually normal
-It shows reduced lung volumes (low vital capacity, low total lung
capacity)
Pulmonary Function Test
Arterial blood gases typically show mild hypoxemia.
Patients tend to hyperventilate and have a reduced PCO2 and
compensated respiratory alkalosis, mostly as a result of increased
respiratory rate.

11. Check Medications
Immunosuppressive or chemo agents
-Bleomycin, methotrexate, cyclophosphmide
Antibiotics
-Macrobid
Cardivascular drugs
-Amiodarone
Vasoactive and neuroactive
-Sansert and dilantin

12. Occupational ILD
There are 3 categories of occupational lung disease
1.Hypersensitiviy pneumonitis
2.Organic dusts (byssinosis)
3.Inorganic dusts (asbestosis, silicosis, coal workers’
pneumoconiosis, and berylliosis)

13. Non-Occupational ILD
1.Idiopathic pulmonary fibrosis (IPF)
2.BOOP (bronchiolitis obliterans organizing pneumonia)
3.Collagen-vascular diseases
4.Sarcoidosis
5.Eosinophilic Granuloma
6.Lymphangioleiomyomatosis
(pronounced lim-fan-g-o-lie-o-my-o-ma-toe-sis)
7.Vasculitis causing ILD: Churg-Strauss
8.Goodpasture syndrome
9.Eosinophilic pneumonia

14. Idiopathic Pulmonary Fibrosis
Definition: (IPF) is an inflammatory lung disease of unknown origin
that causes lung fibrosis and restrictive lung disease. It
characteristically involves only the lung and has no extrapulmonary
manifestations except clubbing. Typically seen in decade 5 of life, it
affects men and women equally.
Etiology uncertain (autoimmune?) It is a diagnosis of
exclusion
Accounts for 50% of ILD’s
Male = female, average age = 55
Smoking worsen disease
10% may have low titers of ANA or RF

17. IPF presentation
Dyspneaand cough and diffuse infiltrative process on CXR
Clubbing is common
History of progressive exercise intolerance
Dry crackles at lung bases
Like many ILD’s, PFT’s show a “restrictive” pattern (low TLC,
normal FEV1/FVC, low DLCO)
Treatment: corticosteroids + or –cyclophosphamide or
azathioprine (20-30% of patients show improvement. This
disease progresses to death. Single-lung transplantation is an
option for some late-IPF patients. Pneumococcal and influenza
vaccines should be given.

18. Diagnosis :
Chest x-ray reveals reticular or reticulonodular disease.
High-resolution CT may show ground-glass
appearance. As IPF progresses, imaging will show
extensive fibrosis with honeycomb pattern.
confirmed in about 25% of patients by transbronchial
biopsy. If transbronchial biopsy is not sufficient, a
thorascopic-guided lung biopsy or open lung biopsy
should be done-especially when there is any
suggestion that there may be an infection involved or
in younger patients.
Note : lung biopsy to exclude vasculitis and infections

19. Treatment :
Pharmacologic treatment: includes pirfenidone, a new
small-molecule compound that has antifibrotic effects
(shown to significantly reduce a decline in lung
function and IPF disease progression) also
corticosteroids + or –cyclophosphamide or
azathioprine (20-30% of patients show improvement.
This disease progresses to death
Non-pharmacologic treatment for eligible patients
includes lung transplantation (shown to reduce the risk
of death by 75% as compared with those who remain
on the waiting list).

20. Sarcoidosis
A disease characterized by the presence of granulomatous tissue.
This is a systemic disease which involves eyes, brain, heart, lungs, bones and
kidneys, skin, liver and spleen.
On pathology a non-caseating granuloma composed of histiocytes, giant
cells and lymphocytes.
In advanced lung disease fibrotic changes are seen.

21. Clinical presentation :
Hypercalcemia or hypercalciuria due to increased
circulation of vitamin D produced by macrophages
• Elevated angiotensin-converting enzyme (ACE) (60% of
patients)
• Abnormalities in LFTs (30% of patients with liver
involvement, with 90% of patients being symptomatic)
• Skin anergy
• PFTs normal or showing a restrictive pattern
• Uveitis and conjunctivitis (>25% of patients) (give all
patients with suspected sarcoidosis an ophthalmologic
examination)

22. Etiology
Unknown, likely immunological basis.
Clinical Features
Four stages are identified:
Stage 0: No obvious intrathoracic involvement
Stage 1: Bilateral hilar lymphadenopathy, often
accompanied by arthritis, uveitis and erythema
nodosum.
Stage 2: Pulmonary parenchyma is also involved,
changes in mid and upper zones.
Stage 3: Pulmonary infiltrates and fibrosis without
adenopathy.

23. Stage I
(bilateral hilar adenopathy)

24. Stage II
Reticular nodules and BHL

25. Risk factor :black people , 20-40 age
Sarcoidosis can involve almost any organ system, but pulmonary
involvement is most common. Ocular, cutaneous, myocardial,
rheumatologic, GI, and neurologic manifestations can also occur.
Dermatologic manifestations occur in 25% of patients with
sarcoidosis; they include lupus pernio, erythema nodosum, non-
scarring alopecia, and papules. Commonly, sarcoidosis is
discovered in a completely asymptomatic patient, usually in the
form of hilar adenopathy on chest x-ray.
There are 2 distinct sarcoid syndromes with acute presentation:
• Lofgren syndrome includes erythema nodosum, arthritis, and
hilar adenopathy.
• Heerfordt-Waldenstromsyndrome describes fever, parotid
enlargement, uveitis, and facial palsy.

26. Lofgren syndrome

27. Heerfordt-Waldenstrom

28. Lung involvement in sarcoidosis occurs
in 90% of patients at some time in their
course. Hilar and left paratracheal
adenopathy is the most common
presentation. Interstitial lung disease
with or without hilar adenopathy can
also be a presentation of sarcoidosis.

29. Diagnosis :
The definitive diagnosis of sarcoidosis rests on biopsy of
suspected tissues, which show noncaseating granulomas.
Chest x-ray findings can show 4 stages of disease (the
stages are not progressive):
• Bilateral hilar adenopathy
• Hilar adenopathy with reticulonodular parenchyma
• Reticulonodular parenchyma alone
• Honeycombing of bilateral lung fields with fibrosis
Transbronchial biopsy

30. Pulmonary Function
No impairment occurs in stages 0 and 1.
In stages 2 and 3 restrictive changes are seen.
Treatment and Prognosis
85% of these patients improve spontaneously, but 15% may develop
progressive fibrosis and respiratory failure.

31. Treatment :
Generally in the setting of organ impairment, a trial of
steroids may be used, giving a high dose for 2 months
allowed by tapering the dose over 3 months. There are
certain scenarios in which steroids are mandatory: uveitis,
sarcoidosis involving the CNS and heart, and patients who
develop hypercalcemia. (Prednisone 0.5-1 mg/kg initially,
then tapered and continued for 6 months to 1 year.)
Eighty percent of patients with lung involvement from
sarcoidosis remain stable, or the sarcoidosis
spontaneously resolves. Twenty percent of patients
develop progressive disease with evidence of end-
organ compromise.

32. Mental Break

33. The End