INTERSTITIAL LUNG DISEASE

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INTERSTITIAL LUNG DISEASE
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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.

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• 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

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Examples of Known Etiology without granulomas

• ARDS
• Occupational and environmental inhalants (e.g.
  asbestos, gases, aerosols)
• Drugs
• Aspiration
• Radiation
• Lymphangitic spread of carcinoma

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• Examples of Known Etiology with granulomas
• Occupational and environmental inhalants
• -Beryllium
• -Hypersensitivity pneumonitis (an allergic
  disorder)
• - Talc
• Infectious agents
• - AFB (Acid-fast bacillus)
• - Fungi

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Examples of Unknown Etiology without granulomas

• Ideopathic Interstitial Pneumonias (IIP)
• Vasculitis
• Pulmonary hemorrhage syndromes
• Eosinophilic infiltration
• Ankylosising spondylitis

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Examples of Unknown Etiology with granulomas

• Sarcoidosis
• Histiocytosis-X
• ANCA () Granulomatos vasculitis
• -Wegeners granulomatosis
• -Churg-Strauss
• -Lymphomatoid granulomatosis

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Clinical Assessment

• Check medication
• Employment
• Systemic Illnesses
• Exposures

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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.
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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.
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Check Medications

• Immunosuppressive or chemo agents
• - Bleomycin, methotrexate, cyclophosphmide
• Antibiotics
• - Macrobid
• Cardivascular drugs
• - Amiodarone
• Vasoactive and neuroactive
• - Sansert and dilantin

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Occupational ILD

• There are 3 categories of occupational lung
  disease
• Hypersensitiviy pneumonitis
• Organic dusts (byssinosis)
• Inorganic dusts (asbestosis, silicosis, coal
  workers
• pneumoconiosis, and berylliosis)

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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.

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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.

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• 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)

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Asbestosis - Pleural Plaques
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This long, thin object is an asbestos fiber
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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

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Silicosis

• broncholiths 2 mm in diameter

showing calcified coalescence areas in upper
lobes
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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.

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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

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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

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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.

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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.

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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.

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Stage I (bilateral hilar adenopathy)
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Stage IIReticular nodules and BHL
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• 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.

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Mental Break
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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.

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

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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.

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• 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.

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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.

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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.

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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!

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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.

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Wegener's Granulomatosis

• chest xray showing bilateral lung nodules

CT scan from the same patient
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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
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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.

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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

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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.

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The End