Restrictive Pulmonary Disease

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Restrictive Pulmonary Disease

• March 19, 2003
• Dr. DArsigny

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Definition

• Restrictive Pulmonary Disease means a limitation
  (restriction) on ones ability to take a full
  deep breath and fill ones lungs to their normal
  volume
• ANALOGY like trying to breathe with a tight band
  around your chest. You can still breathe but only
  shallowly and you cannot expand the lungs and
  chest to take in a full breath

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Restrictive pulmonary disease

• There are 150 different disorders that can cause
  restrictive pulmonary disease and many share
  similar clinical, radiographic and physiologic
  abnormalities
• Unlike the obstructive pulmonary diseases that
  are diagnosed clinically, restrictive pulmonary
  diseases often require a lung biopsy for
  pathologic diagnosis
• Clinically, we try to narrow the differential by
  deciding if the patient cant breathe because of
  a non-lung parenchymal problem or due to lung
  (interstitial) disease

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Massive left effusion
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Causes of Constrictive disease

• Chest wall
• skin burns
• bones and joints kyphoscoliosis ankylosing
  spondylitis
• muscles muscular dystrophies myasthenia
  gravis
• nerves Guillain-Barre drugs
  succinylcholine, curare polio amyotrophic
  lateral sclerosis cervical cord
• Pleura
• effusion (blood, exudate, transudate, pus)
• fibrosis (infection, trauma, inflammation)
• tumor (mesothelioma)
• air (pneumothorax)
• Other
• intrabdominal contents within chest (hiatus
  hernia, perforated diaphragm)

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Interstitial disease
nodular
reticular
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Workup of interstitial lung disease

• History symptoms duration of symptoms any
  infectious symptoms extra-pulmonary
  symptoms work history exposures to
  dust/minerals/fibers drugs other
  diseases hobbies

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Causes of interstitial lung disease

• Drugs antibiotics (nitrofurantoin,
  sulfonamides, penicillin) chemotherapy
  agents (bleomycin, cyclophospamide) cardiac
  drugs (amiodarone,procainamide) immunosuppresants
  (methotrexate, azathioprine) other (heroine,
  interleukin-2)
• Idiopathic U.I.P., D.I.P., N.S.I.P.
• Infection mycoplasma, chlamydia, TB, fungal,
  parasites, nocardia, viral
• Collagen vascular disease SLE, RA, scleroderma,
  dermatomyositis
• Pneumoconiosis silicosis, coal-workers lung
• other inhalational diseases berylliosis,
  siderosis, asbestosis, hypersensitivity
  pneumonitis

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Causes on Restrictive lung disease

• Granulomatous diseases sarcoidosis eosinophili
  c granulomatosis hypersensitivity
  pneumonitis miliary TB
• Vascular pulmonary edema (high or low pressure)
• Miscellaneous
• lymphangioleiomyomatosis bronchiolitis
  obliterans with organizing pneumonia
  (BOOP) lymphangitic spread of tumor metastatic
  tumor (thyroid, melanoma, adenocarcinoma) radiati
  on pneumonitis chemical burn (chlorine,
  ammonia) Wegeners granulomatosis (rare)

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Work up for Restrictive pulmonary diseases

• History
• physical exam pulmonary auscultation
  (crackles) fever clubbing signs of other
  diseases connective tissue disease left
  heart failure cancer

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Work up for Restrictive pulmonary diseases

• Chest X-ray ?lung volume high
  diaphragms diffuse increase in density
  reticular/nodular more prominent at bases ?
  size of pulmonary arteries late stage, see
  large air spaces honeycombing, more
  prominent at the bases review old X-rays to
  assess acuity
• High resolution CT chest helps better assess
  interstitial disease and sometimes has classic
  findings sarcoidosis, LAM

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Work up for ILD

• Blood infection (serology, culture) allergy
  (serum antibody titres to organic
  antigens) other diseases
• Sputum
• PFTs ?VC, ?RV, ? TLC N or ? FEV1/FVC, N or ?
  flows ?DLCO ?PaO2, ?PaCO2 (? as compliance
  decreases) ?HCO3, ?pH

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Spirometry
normal
Restrictive
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Work up of ILD

• Bronchoscopy with bronchial lavage
• biopsy transbronchial surgical

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I.P.F.

• Diffuse fibrotic disease of the lungs of unknown
  etiology
• represents 30-40 of all cases of ILD
• diagnosis of exclusion
• prevalence of 3-4 cases/100 000 population
• mean age 40-70 years with no sex predominance
• 60 are clubbed
• rare family clusters
• mean survival is 4-6 years, but clinical course
  varies

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IPF

• Insidious onset of breathlessness, occasionally
  heralded by flu-like illness
• CXR has reticular or reticular-nodular
  infiltrates, predominately at the bases
• PFTs show rapid shallow breathing pattern with
  small lung volumes, hypoxemic as disease worsens
• BAL shows increased lymphocytes if increased
  eosinophils, poor prognosis if increased
  neutrophils or eosinophils, better response to
  cyclophosphamide

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X-Rays
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(No Transcript)
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Treatment

• Oxygen if needed
• steroids
• immunosuppressives
• transplantation
• treat right heart failure
• rehabilitation

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I.P.F.

• UIP DIP
• Dead at 5 yrs 45 4.8
• Dead at 10 yrs 72 30
• Course untreated
• improved 0 22
• no change 15 16
• worse 85 62
• Course with corticosteroids
• improved 12 62
• no change 19 12
• worse 69 26

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Sarcoid

• systemic disease of unknown etiology, with
  non-caseating granulomas in the tissues-
  especially lung and lymph nodes
• may involve any organ or tissue
• involves the lung in 90 of cases
• unlike IPF, it is often a clinical or
  radiographic diagnosis
• must exclude mimics such as TB, lymphoma,
  histoplasmosis, coccidiomycosis, beryliosis,
  lung cancer

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Sarcoid

• Most patients with sarcoidosis are
  asymptomatic CXR much worse than patient
• CLASSIC young, female, with erythema nodosum
• may have shortness of breath or cough
• Other malaise, fatigue, joint pain, uveitis,
  muscle, liver and and/or lung
  involvement, hypercalcemia, heart and/or brain
  involvement anergy negative skin tests for TB,
  mumps, candida, trichophyton

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Sarcoid-CXR

• Bilateral hilar adenopathy
• reticulonodular infiltrates predominately in
  upper and mid-lung zones

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CXR
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Sarcoid

• Other investigations CT chest skin anergy
  tests blood calcium, Ace level, WBC,
  IgG bronchoscopy BAL and transbronchial
  biopsy mediastinoscopy lymph node biopsy

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Sarcoid

• Treatment
• for all other organ involvement
• for hypercalcemia
• for lung involvement if it is getting
  worse smaller lungs on CXR smaller lung
  volumes on PFT hypoxemia worse
  breathlessness
• does not alter eventual outcome
• steroids, chloroquin, azathioprine

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Hypersensitivity pneumonitis

• not an infection
• an allergic response to inhaled organic material
• delayed reaction
• usually clears when removed from allergen
• repeated exposure may lead to fibrosis
• develop symptoms 4-6 hours after dust/antigen
  inhalation
• develop shortness of breath, cough, chills, fever
  and malaise x 12 hours and abate spontaneously
• on exam, high temperature, pulmonary crackles,
  high WBC
• Chronic exposure progressive SOB and cough

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X-Ray

• Reticular infiltrates
• fine alveolar fillling
• predominately in mid-upper lung zones

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Causes of hypersensitivity pneumonitis

• Farmers thermophilic actinomyces
• Workers with birds (pigeons), cotton, mushrooms,
  cork
• Other sources of spores basements, grain, trees
• further work-up history and physical
  X-ray PFTs blood
  eosinophilia serology for allergen
  bronchoscopy with BAL and biopsy

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Hypersensitivity pneumonitis

• Treatment
• avoid exposure
• masks
• steroids
• cyclophosphamide
• azathioprine