Sarcoidosis
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Sarcoidosis

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Sarcoidosis Sarcoidosis Presentation Transcript

  • Sarcoidosis Sung Chul Hwang, M.D.Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine
  • DefinitionAn Idiopathic systemic disordercharacterized by accumulation oflymphocytes and monocytes inmany organs formingnoncaseating, epitheloidgranuloma and subsequentconformational changes in theinvolved organs
  • Epidemiology• Prevalence 10-40/100,000 mainly in Blacks• Europe 10-40/100,000 mainly white• Sweden 64/100,000• Irish female in London 200/100,000• Very Rare in Canadian Indians, Maoris, Southeast Asians• Familial : no specific patterns• Not related to HLA types• No Sexual predilection
  • Pathogenesis ( 1 )
  • Pathogenesis ( 2 )
  • Pathogenesis ( 3 )
  • Pathogenesis ( 4 )
  • Laboratory features• Lymphopenia• Hyperglobulinemia• Hypercalcemia• Hypercalciuria• Increased ESR• Abnormal LFT, ALP increase• Immunologic abnormality• PPD anergy• Circulating immune complex present
  • PFT• Typical DILD pattern• Restrictive pulmonary insufficiency• FVC decrease• RV decrease• DLCO decrease• Decreased lung compliance “ stiff Lung”
  • X-ray Findings• Bilateral Hilar or Mediastinal Lymphadenopathy• Interstitial pulmonary infiltrates• Fibrosis• Nodular changes• Gallium Scan : “ Panda-Eye Sign”
  • • BAL : Lymphocytic(T–helper) Alveolitis• TBLB : 90% diagnostic DILD• Kveim- Siltzbach Test : Rarely done• Serum ACE II level : elevated
  • Organ Involvement• Lungs ( 94 %) • Salivary gland• Upper airways • Heart ( 11.2 ) • Nervous system• Lymph nodes • Joints ( 73 ) • Endocrine• Skin • Kidneys ( 32 ) • Lacrimal glands• Eyes ( 21 ) • Breast, Uterus• Liver ( 21
  • Differential Diagnosis• Lymphoma • Gout• Tuberculosis • Rheumatoid Arthritis• Eosinophilic • Primary hyper Granuloma PTH • Berylliosis
  • Treatment and Prognosis• Spontaneous Remission : Hilar or Mediastinal L/Ns  80 – 90 %• Lung Parechymal lesion is rarely self- limiting• Treament of choice : Prednisone, others, indomethacin, MTX, Cyclosporin, Cytoxan, Allopurinol, etc
  • Indicators of Sarcoid Activity• Worsening clinical features• Worsening symptoms• Lung function deterioration• Elevated Serum Ca++• Elevated serum ACE level• Gallium scanning positivity increases• Worsening evidence of alveolitis in BAL
  • Radiologic Classification• Stage 0 : Absence of X-ray findings• Stage I : L/N enlargement without lung infiltrates• Stage II A : L/N + Lung parenchyme• Stage II B : Lung parenchyme without L/N• Stage III : changes indicating pulmonary fibrosis-> “ honey combing, hilar retraction ”
  • Sarcoidosis L/Ns
  • Sarcoidosis (L/N + Lung)
  • Sarcoid Uveitis Hypopion Posterior Synechiae : Iris deformity Cataract
  • Sarcoid Dactylitis
  • Sarcoid Skin Lesion
  • Lupus Pernio
  • Sarcoid Choreoretinitis
  • Infiltrative Sarcoids Bronchoscopy
  • BAL in Sarcoidosis
  • Grade I
  • Grade II A
  • Grade II B
  • Grade III
  • Advanced Chronic Sarcoidosis
  • Pathology of Sarcoidosis (1)
  • Pathology of Sarcoidosis (2)
  • Pathology of Sarcoidosis (3)
  • Pathology of Sarcoidosis (4)
  • Sarcodosis Pathology(TBLB)
  • Sarcoid Skin Lesions Papule or Plaque
  • Sarcoidosis F/39
  • Sarcoidosis