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INTERSTITIAL LUNG DISEASE

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Title: INTERSTITIAL LUNG DISEASE


1
INTERSTITIAL LUNG DISEASE
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

6
Examples of Unknown Etiology without granulomas
  • Ideopathic Interstitial Pneumonias (IIP)
  • Vasculitis
  • Pulmonary hemorrhage syndromes
  • Eosinophilic infiltration
  • Ankylosising spondylitis

7
Examples of Unknown Etiology with granulomas
  • Sarcoidosis
  • Histiocytosis-X
  • ANCA () Granulomatos vasculitis
  • -Wegeners 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
Pulmonary Function Test
  • 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)

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
  • Hypersensitiviy pneumonitis
  • Organic dusts (byssinosis)
  • Inorganic dusts (asbestosis, silicosis, coal
    workers
  • pneumoconiosis, and berylliosis)

13
Hypersensitivity Pneumonitis
  • It is an immune-mediated reaction to organic
    agents
  • Typically it has poorly formed granulomas
  • Diagnosis is by history and chest x-ray may
    reveal recurrent infiltrates
  • Various causes include
  • Moldy hay --- farmers lung
  • Grain dust (works in a grain elevator)
  • Air conditioning systems
  • Hobbies such as bird breeding
  • Best treatment is to remove the patient from the
    offending antigent. Corticosteroids are
    beneficial only in acute disease.

14
Byssinosis
  • Caused by inhalation of cotton, flax, or hemp
    dust.
  • Not immune-related, no sensitization needed
  • Early stage has occasional chest tightness
  • Late stage has regular chest tightness toward the
    end of the 1st day of the work week (Monday
    chest tightness)
  • Therapy for early-stage focuses on reversing
    airway narrowing. Antihistamines may be
    prescribed to reduce tightness in the chest.
  • Bronchodilators may be used with an inhaler or
    taken in tablet form. Reducing exposure is
    essential.

15
  • Asbestos exposure
  • Causes parenchymal fibrosis with gt 10 years of
    moderate exposure
  • Features
  • Causes bilateral mid-thoracic pleural
    thickening/plaque/calcification formation.
  • Chest X-ray shows diaphragmatic calcification
    with sparing of the costophrenic angle
  • Malignant mesotheliomas are associated (80) with
    asbestos exposure
  • Asbestosis results from gt 10 years of moderate
  • exposure. Smoking has a synergistic effect
    with asbestosis in development of lung CA (adeno
    and squamous)

16
Asbestosis - Pleural Plaques
17
This long, thin object is an asbestos fiber
18
Silicosis
  • Requires years of exposure to crystalline silica
    to develop
  • (as in mining, glassmaking, ceramics,
    sandblasting, foundries,
  • and brick yardswith a latency of 20-30 years.
  • Acute form large exposures to fine particles
  • --- may develop in months, not years
  • Hallmark of chronic formsnodules
  • - simple small nodule lt1 cm, usually upper
    lung zones
  • - not associated with symptoms or pulmonary
    function
  • changes
  • - complicated leads to progressive massive
    fibrosis
  • nodules gt 1 cm

19
Silicosis
  • broncholiths 2 mm in diameter

showing calcified coalescence areas in upper
lobes
20
Berylliosis
  • Caused by a cell-mediated immune response that
    can
  • occur from gt 2 years exposure to even slight
    amount of beryllium. Suspect in people working
    in high-tech electronics, alloys, ceramics, and
    pre-1950 fluorescent light manufacturing.
  • Affects upper lobes (like silicosis, TB)
  • Have hilar lymphadenopathy that looks identical
    to that caused by sarcoidosis on chest X-ray.
  • Diagnose by beryllium lymphocyte transformation
    test.
  • Treat with corticosteroids.

21
Non-Occupational ILD
  • Idiopathic pulmonary fibrosis (IPF)
  • BOOP (bronchiolitis obliterans organizing
    pneumonia)
  • Collagen-vascular diseases
  • Sarcoidosis
  • Eosinophilic Granuloma
  • Lymphangioleiomyomatosis
  • (pronounced lim-fan-g-o-lie-o-my-o-ma-toe-si
    s)
  • Vasculitis causing ILD Churg-Strauss
  • Goodpasture syndrome
  • Eosinophilic pneumonia

22
Idiopathic Pulmonary Fibrosis
  • Etiology uncertain (autoimmune?) It is a
    diagnosis of exclusion
  • Accounts for 50 of ILDs
  • No extrapulmonary manifestation of the disease
  • Male female, average age 55
  • Smoking worsen disease
  • 10 may have low titers of ANA or RF

23
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 ILDs, PFTs show a restrictive
    pattern (low TLC, normal FEV1/FVC, low DLCO)
  • Diagnosis
  • 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.
  • 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.

24
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.

25
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.

26
Stage I (bilateral hilar adenopathy)
27
Stage IIReticular nodules and BHL
28
  • 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.
  • Prednisone 0.5- 1 mg/kg initially, then tapered
    and continued for 6 months to 1 year.

29
Mental Break
30
Hypersensitivity Pneumonitis
  • Also known as extrinsic allergic alveolitis.
  • Hypersensitivity reaction in the lung occurs in
    response to inhaled organic dust.
  • Example is farmers lung.
  • The exposure may be occupational or
    environmental.
  • The disease occurs from type III and type IV
    hypersensitivity reactions.
  • Farmers lung is due to thermophilic actinomyces
    in moldy hay.
  • Bird fanciers lung is caused by avian antigen.

31
  • Pathology
  • There is infiltration of alveolar walls with
    lymphocytes, plasma cells and histiocytes.
  • There are loosely formed granulomas.
  • Fibrotic changes occur in advanced disease.

32
Clinical Features
  • The disease may occur in acute or chronic forms.
  • Acute Hypersensitivity Pneumonitis
  • Dyspnea, fever, malaise and cough appear 4-6
    hours after exposure.
  • These symptoms continue for 24-48 hours.
  • Physical examination shows fine crackles
    throughout the lungs.
  • These patients present with progressive dyspnea
    over a period of years.
  • Chest radiograph may be normal, but may show
    reticular nodular infiltration.

33
  • Chronic Hypersensitivity
    Pneumonitis
  • These patients present with progressive dyspnea.
  • Physical examination shows bilateral inspiratory
    crackles.
  • Chest x-ray shows reticular nodular infiltration
    and fibrosis predominantly in upper lobes.
  • Pulmonary function tests restrictive pattern.
  • Gas exchange shows hypoxemia which worsens on
    exercise.

34
Interstitial Disease Caused by Drugs, Poisons and
Radiation
  • Various drugs cause acute pulmonary reaction
    proceeding to interstitial fibrosis.
  • These drugs are busulfan, nitrofurantoin,
    amiodarone, bleomycin.
  • High oxygen concentration interstitial
    fibrosis.
  • Radiation exposure acute pneumonitis
    fibrosis.

35
Bronchiolitis Obliterans Organizing Pneumonia
(BOOP)
  • BOOP is bronchiolitis (inflammation of the small
    airways) and a chronic alveolitis (the organizing
    pneumonia). In adults, it is associated with
    penicillamine use.
  • Common presentation is an insidious onset (seeks
    to 1-2 months), of cough, fever, dyspnea,
    malaise, and myalgias. Multiple courses of
    antibiotics without effect. Rales are common.
    Chest X-ray shows some interstiial disease,
    bronchia thickening, and patchy bilateral
    alveolar infiltrate.
  • It has good prognosis and responds to steroids.
  • Open lug biopsy is the definitive means of
    diagnosing BOOP.

36
Eosinophilic granuloma
  • Previously called Histiocystosis X
  • Smokers and M gt F
  • It sometimes involves the posterior
    pituitary-leading to diabetes insipidus
  • In the lung, it causes interstitial changes and
    small cystic spaces in the upper lung fields,
    both of which are visible on chest X-ray giving a
    honeycomb appearance.
  • 10 initialy present with a pneumothorax and up
    to 50 get a pneumothrorax some time in the
    course of the illness.
  • Diagnosis is made by finding Langerans cells on
    lung biopsy or bronchoalveolar lavage.
  • Treatment stop smoking!

37
Vasculitis which causes ILD
  • Wegener Granulomatosis
  • - necrotizing granuloma which
  • 1. Affects the upper respiratory tract and
    paranasal sinuses
  • 2. Causes a granulomatous pulmonary
    vasculitis with large (sometimes cavitary)
    nodules
  • 3. Causes a necrotizing
    glomerulonephritis
  • Test ANCA test (90 sensitive and 90 specific)
    when positive, it virtually always c-ANCA
  • Diagnosis confirmed from either a biopsy of the
    nasal membrane or an open lung biopsy
  • Treatment cyclophosphamide with or without
    corticosteroids.

38
Wegener's Granulomatosis
  • chest xray showing bilateral lung nodules

CT scan from the same patient
39
Wegeners granulomatosis affecting other sites
in the body
  • Nasal crusting and frequent nosebleeds can occur.
  • Erosion and perforation of the nasal septum.
  • The bridge of the nose can collapse resulting in
    a saddlenose deformity.
  • This resulted from the collapse of the nasal
    septum caused by cartilage inflammation

Narrowing of the windpipe just below the vocal
cords, a condition called subglottic
stenosis. This narrowing, caused by inflammation
and scarring
40
Churg-Strauss
  • necrotizing small vessel vasculitis with
    eosinophilic infiltration.
  • it may affect the skin and kidney, and may cause
    neuropathy
  • Patients usually presents with preexisting asthma
    and have
  • eosinophilia (up to 80 of the WBCs!)
  • -Consider this with a progressively worsening
    asthmatic
  • -Treatment Aggressive therapy with cytotoxic
    agents and
  • corticosteroids.

41
Other ILDs
  • Goodpasture Syndrome
  • - Autoimmune young adult males M to F is 3
    1
  • - hemoptysis that usually precedes renal
    abnormalities
  • - May have Fe anemia
  • - Symptoms due to anti-glomerular basement
    memberrane antibodies which result in linear
    deposition of IgG and C3 on alveolar and
    glomerular BM.
  • - Treat with immunosuppressives and
    plasmapheresis

42
Idiopathic Pulmonary Hemosiderosis
  • causes intermittent pulmonary hemorrhage
  • It is similar to Goodpasture except IPH does not
    affect the kidneys
  • Fe deficiency anemia occurs
  • It may remit in young patients, but it is
    unrelenting in adults.

43
The End
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