Interstitial Lung Disease ILD

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Interstitial Lung DiseaseILD
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General description

• ILDs represent a large and
  heterogeneous group of lower respiratory tract
  disorders.
• There are similar clinical signs and X-ray
  features.

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The characteristic of clinical signs including

• Dyspnea after exercising
• chest X-ray shows diffuse abnormality of
  pulmonary parenchymal,including
  nodules,linear(reticular) infiltrates
• pulmonary function tests shows restrictive
  hypoventilation reduced diffusing capacity
• tissue biopsy shows a variety pulmonary fibrosis
  and aveolar inflammation

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Clinical Classification of ILD

• known cause
• unknown cause (IIP,ects)

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Pathogenesis

• The pathogenesis of ILDs is unknown.
• But more and more facts have shown that immune
  cells and their cytokines play an important role
  in the course of ILDs.

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Nowadays the major courses of the ILDs including

• Intra-alveolar inflammation
• immune cells and their cytokines injure
  epithelial and endothelial cells
• intra-alveolar fibrosis/alveolar collapse

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• In the course of ILDs, many cytokines,
  including TGF-?, IGF-?, prostaglandin E2,
  platelet-derived growth factor, ects, involve in.

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Clinical manifestations

• Breathlessness, Progressive respiratory
  insufficency
• cough without sputum.
• Some patients may have fatigue, weight loss,
  joint pain.

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Physical examinations

• Bilateral basilar, crepitant velcro-like rale are
  found in most patients
• wheezing, rhonchi and coarse rales are
  occasionally heard
• with advanced disease, patients may have
  tachypnea and tachycardia
• clubbing of the fingers and toes is common
• At last, pulmonary hypertention and cor pulmonale
  may be exist

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Chest radiography

• It is important method to diagnose the ILDs.
  The majority of ILDs cause infiltrates in the
  lower lung zones.

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• A diffuse ground glass pattern is seen early in
  the disease
• when the disease progresses, a chest radiography
  demonstrates nodules, linear(reticular)
  infiltrates, or a combination of the two
• at last, the infiltrates become coarser and lung
  volume is lost
• honeycomb pattern may appear at the end of the
  disease

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nodular
linear
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nodular
linear
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honeycomb
ground glass pattern
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Pulmonary function tests

• Pulmonary function tests of ILDs shows
  restrictive hypoventilation.

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It includes

• Reduced lung volumes(vital capacity, total lung
  capacity)
• reduced diffusing capacity
• static lung compliance is decreased

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BALF examination

• the cell counts in BALF of ILDs is twice than
  that of normal humans
• cell complements of ILDs is difference from that
  of normal humans
• for example, the percents of neutriphils in BALF
  of IPF is higher than that of normal humans

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Blood examination
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Lung biopsy

• For example, TBLB(transbronchial biopsy), an
  open-lung or thoracoscopic biopsy are used to
  diagnose the ILDs

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Idiopathic pulmonary fibrosisIPF
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• IPF is an unknown chronic interstitial lung
  disease.Nowadays It has become a common disease.
• the clinical manifestations, and some
  experimental examination including pulmonary
  function tests,chest radiography examinations and
  lung biopsy are coincide to that of ILDs
  introduced before.

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• Pathology According to the pathologic
  classification, there are seven types of
  Idiopathic interstitial pneumonitis.
• IPF-UIP(usual interstitial pneumonitis)
• NSIP, nonspecific interstitial pneumonitis
• DIP, desquamative interstitial pneumonitis
• RBILD, respiratory brnchiolitis associated
  interstitial lung disease
• LIP, lymphocytic interstitial pneumonitis
• COP, concealed organizing pneumonia
• AIP, acute interstitial pneumonitis

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How to diagnose IPF

• According to the clinical signs and some
  experimental examinations, we can diagnose the
  IPF except some known cause ILDs
• lung biopsy is an only way to give a last
  diagnosis

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Clinical Diagnostic Standard of IPF

• Except known cause ILDS
• Lung function
• HRCT
• TBLB and BAL
• Age
• Unexplained dyspnea after exercise
• Period
• Physical examination

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Chest radiography
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Chest radiography
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Pathologic Diagnosis

• The pathologic diagnosis of IPF is coincide with
  UIP

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Treatment of IPF

• Nowadays, the treatment ways of IPF are lack of
  effective ways
• corticosteroids are the main therapy
• the initial treatment of choice is prednisone
  0.5mg/kg of ideal body weight per day. For 1
  month, the dose is gradually tapered over several
  months to a maintenance dose of 0.125 mg/kg per
  day

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• Immunosuppressive agents,including CTX, MTX
• lung transplantation

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Treatment

• Some common therapies, including oxygen therapy,
  antibiotic therapy when pulmonary infections
  exist.

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prognosis
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Sarcoidosis
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Definition

• Sarcoidosis is a disease of unknown cause and is
  characterized by the presence of non-caseating
  granulomas in one or more organ, system. It is
  considered a systemic disease
• Usually lungs and the lymph nodes in the
  mediastinum and hilar regions are the most site
  of involvement
• The clinical course is quite variable
  asymptomatic

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The cause of sarcoidosis is unknown. But
many researchers have suggested that immune
mechanisms are important in disease
pathogenesis.Genetic factor may also play an
important role.
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Other factors including infectious and
environmental or occupational may also involve in
the sarcoidosis.
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Pathogenesis

• Antigen processing by macrophages is believed to
  trigger an oligoclonal expansion of
  CD4(helper-inducer) lymphocytes of the Th1
  phenotype with production of IL-2 and IFN-?.
• IL-2 cause proliferation of more CD4 cells,
  elaborate more cytokines.
• Many cytokines, mainly IL-2,adhension molecules
  and growth factors are released from lymphocyte
  and macrophages.

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The basic pathogenesis includes three main stages

• Pulmonary alveolus inflammation
• formation of non-caseating granulomas
• the stage of interstitial fibrosis

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Clinical manifestations

• The clinical course is variable
• the respiratory system is the most commonly
  affected
• approximately 90 of patients demonstrate
  intrathoracic involvement on a chest radiograph
• sometime with or without extrathoracic disease

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Clinical manifestations

• Almost 30 to 60 per cent of patients have no
  symptoms at the time of presentation
• sometimes the disease is identified because of
  abnormalities on a chest radiograph
• some patients present with respiratory symptoms
  such as dyspnea and cough, which may or may not
  be accompanied by constitutional symptoms, such
  as fever and malaise

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Specific signs and symptoms depend on the
particular organ system(s) involved

• Respiratory system disease
• Intrathoracic nodal involvement and parenchymal
  lung disease are the most common ways in which
  sarcoidosis affeccts the respiratory system
• Hilar and medistinal lymph nodes may be affected
• The pulmonary parenchyma demonstrates well
  defined,non-caseating granulomas with the
  pulmonary interstitium
• Usually upper lobes of the lung tend to be more
  involved

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Extrapulmonary sarcoidosis

• Including
• skin disease about 20-25 of patients are
  involved lesions include papules, plaques,
  nodules and lupus pernio, erythema nodosum
• eye disease and neurologic disease, liver and
  spleen, peripheral lymph nodes, bone lesions and
  myopathy

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Experimental examinations and some specific
examinations

• elevations in the level of angiotensin-converting
  enzyme(ACE) (the normal level is 17.6-34u/ml).
  The measurement of serum ACE might be a useful
  diagnostic and prognostic test in sarcoidosis
• hypercalcemia a potentially important
  complication of sarcoidosis

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• PPD test about 2/3 patients with sarcoidosis has
  no reaction
• Kveim antigen test we cant usually used the
  test we have not standard antigen
• bronchial-alveolar lavage fluid
  examination(BALF) the lymphocyte percent of the
  BALF is elevated. Usually, the percent of
  lymphocyte of BALF is more than 28.It
  demonstrates that the disease is active

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• tissue biopsy
• an affected organ or tissue are generally
  used to diagnostic biopsy, including skin, lymphy
  node,ects.
• Flexible electric bronchoscopy with
  transbronchial lung biopsy(TBLB)
• Mediastinoscopy is sometimes performed in the
  presence of isolated mediastinal adenopathy

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X-ray examination

• The plain chest radiography is an important way
  to diagnose sarcoidosis.
• The major abnormalities seen on the chest
  radiograph include lymphadenopathy,usually
  involving both hila and mediastinal,and
  involvement of the pulmonary parenchyma

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• Computed tomography(CT) of the chest is used to
  evaluate of suspected sarcoidosis, especially
  there is need for better definition of
  mediastinal lymph node involvement.
• High-resolution CT is used to demonstrate that
  pulmonary parenchymal involvement is localized
  around bronchovascular structures, producing an
  appearance resembling budding branches on a tree.

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• Usually, The features of HRCT of patient with
  sarcoidosis shows numerous small nodular in a
  predominantly bronchovascular distribution

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Scanning with gallium citrate-67 is a more
sensitive method to evaluate the activity of
sarcoidosis
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Diagnosis and differential diagnosis

• According to the clinical signs, chest X-ray,
  tissue biopsy, we can diagnose the sarcoidosis.
• Nowadays the standard to diagnose the sarcoidosis
  includes
• chest X-ray shows that bilateral hilar and
  mediastinal adenopathy, accompany with(or
  without) small nodules in pulmonary parenchymal

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• Tissue biopsy shows that characteristic of
  affected organ and tissue is conincide to
  sarcoidosis
• The Kveim test is positive
• The PPD test is negative or weak positive
• Hypercalcemia and hypercalciuia
• The lymphocytes of BALF is elevated
• The level of SACE is elevated
• Among them, the first and the second are more
  important. If a patient has both the first one
  and the second one, we can diagnose

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Differential diagnosis including

• The tuberculosis of hilar lymphy node. Usually,
  the patient with tuberculosis may have clinical
  signs of tuberculosis.PPD test is positive.
  Biopsy shoud be done if we couldnt diagnose

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Differential diagnosis including

• lymphoma.Usually we must do biopsy
• The transferred tumor of hilarthe patient with
  transferred hilar adenopathy usually shows the
  clinical signs caused by primary tumor. For
  example, the chest X-ray of patient with lung
  cancer sometimes shows not only pulmonary
  parenchymal involvement but also hilar and
  mediastinal adenopathy

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Treatment

• Most of patients, especially those with no
  clinical signs need no therapy.
• If patients with clinical signs, the need to
  therapy. For example, parenchymal lung disease is
  a potential indication, depending on its effects
  on pulmonary function and symptoms, not on the
  severity of radiographic involvement.Nowadays
  corticosteroids are also a major treatment
  method. They can alter its long-term natural
  history, improve the clinical signs promptly,
  prevent progressive pulmonary fibrosis

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• Usually prednisone is started at 40mg/d.The dose
  can be tapered with the good of using lowest dose
  that keeps the disease under adequate control.
• Treatment durations usually is one year
• Another drugs, including cytotoxic agents(for
  example methotrexate), hydroxychloroquine

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Prognosis
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• Table 1
• Radiographic staging of intrathoracic sarcoidosis
• stage hilar adenopathy parenchymal
  disease
• 0 No
  No
• 1 Yes
  No
• (both hilar and
  mediastinal right paratrachcal)
• 2 yes
  yes(usually
• 
  shows
  small
• 
  nodules
  
• 
  
  interstitial fibrosis)
• 3(or 4) No
  yes
• 
  (with fibrosis)

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