Interstitial lung disease (ILD) is a term that includes over 150 chronic lung disorders where the lung tissue becomes damaged, inflamed, and scarred. This makes it difficult for oxygen to travel into the bloodstream, causing shortness of breath and cough. Idiopathic pulmonary fibrosis is a chronic, progressive ILD of unknown cause where lung tissue becomes thickened and stiff. Sarcoidosis is a systemic disease characterized by non-caseating granulomas that commonly involves the lungs, causing inflammation and fibrosis. It has various stages of involvement and generally improves spontaneously but can progress in 15% of patients.
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 interstitium becomes 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 disease often
used to describe the same condition.
Classification:
“Known” vs. “Unknown”
-”without” or “with” granulomas
4. Examples of Known Etiology: without granulomas
ARDS
Occupational and environmental inhalants
(e.g. asbestos, gases, aerosols)
Drugs
Aspiration
Radiation
Lymphangitic spread of carcinoma
5. Examples of Known Etiology: with granulomas
Occupational and environmental inhalants
-Beryllium
-Hypersensitivity pneumonitis (an allergic disorder)
- Talc
Infectious agents
- AFB (Acid-fast bacillus)
- Fungi
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)
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
15.
16.
17. IPF presentation
Dyspnea and 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.
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-Waldenstrom syndrome describes fever, parotid
enlargement, uveitis, and facial palsy.
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.
