Sarcoidosis

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Sarcoidosis

  1. 1. Sarcoidosis Sung Chul Hwang, M.D.Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine
  2. 2. DefinitionAn Idiopathic systemic disordercharacterized by accumulation oflymphocytes and monocytes inmany organs formingnoncaseating, epitheloidgranuloma and subsequentconformational changes in theinvolved organs
  3. 3. 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
  4. 4. Pathogenesis ( 1 )
  5. 5. Pathogenesis ( 2 )
  6. 6. Pathogenesis ( 3 )
  7. 7. Pathogenesis ( 4 )
  8. 8. Laboratory features• Lymphopenia• Hyperglobulinemia• Hypercalcemia• Hypercalciuria• Increased ESR• Abnormal LFT, ALP increase• Immunologic abnormality• PPD anergy• Circulating immune complex present
  9. 9. PFT• Typical DILD pattern• Restrictive pulmonary insufficiency• FVC decrease• RV decrease• DLCO decrease• Decreased lung compliance “ stiff Lung”
  10. 10. X-ray Findings• Bilateral Hilar or Mediastinal Lymphadenopathy• Interstitial pulmonary infiltrates• Fibrosis• Nodular changes• Gallium Scan : “ Panda-Eye Sign”
  11. 11. • BAL : Lymphocytic(T–helper) Alveolitis• TBLB : 90% diagnostic DILD• Kveim- Siltzbach Test : Rarely done• Serum ACE II level : elevated
  12. 12. 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
  13. 13. Differential Diagnosis• Lymphoma • Gout• Tuberculosis • Rheumatoid Arthritis• Eosinophilic • Primary hyper Granuloma PTH • Berylliosis
  14. 14. 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
  15. 15. 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
  16. 16. 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 ”
  17. 17. Sarcoidosis L/Ns
  18. 18. Sarcoidosis (L/N + Lung)
  19. 19. Sarcoid Uveitis Hypopion Posterior Synechiae : Iris deformity Cataract
  20. 20. Sarcoid Dactylitis
  21. 21. Sarcoid Skin Lesion
  22. 22. Lupus Pernio
  23. 23. Sarcoid Choreoretinitis
  24. 24. Infiltrative Sarcoids Bronchoscopy
  25. 25. BAL in Sarcoidosis
  26. 26. Grade I
  27. 27. Grade II A
  28. 28. Grade II B
  29. 29. Grade III
  30. 30. Advanced Chronic Sarcoidosis
  31. 31. Pathology of Sarcoidosis (1)
  32. 32. Pathology of Sarcoidosis (2)
  33. 33. Pathology of Sarcoidosis (3)
  34. 34. Pathology of Sarcoidosis (4)
  35. 35. Sarcodosis Pathology(TBLB)
  36. 36. Sarcoid Skin Lesions Papule or Plaque
  37. 37. Sarcoidosis F/39
  38. 38. Sarcoidosis

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