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Sarcoidosis

Sarcoidosis. Sung Chul Hwang, M.D. Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine. Definition.

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Sarcoidosis

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  1. Sarcoidosis Sung Chul Hwang, M.D. Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine

  2. Definition An Idiopathic systemic disorder characterized by accumulation of lymphocytes and monocytes in many organs forming noncaseating,epitheloid granuloma and subsequent conformational changes in the involved organs

  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. Pathogenesis ( 1 )

  5. Pathogenesis ( 2 )

  6. Pathogenesis ( 3 )

  7. Pathogenesis ( 4 )

  8. Laboratory features • Lymphopenia • Hyperglobulinemia • Hypercalcemia • Hypercalciuria • Increased ESR • Abnormal LFT, ALP increase • Immunologic abnormality • PPD anergy • Circulating immune complex present

  9. PFT • Typical DILD pattern • Restrictive pulmonary insufficiency • FVC decrease • RV decrease • DLCO decrease • Decreased lung compliance “ stiff Lung”

  10. X-ray Findings • Bilateral Hilar or Mediastinal Lymphadenopathy • Interstitial pulmonary infiltrates • Fibrosis • Nodular changes • Gallium Scan : “ Panda-Eye Sign”

  11. BAL : Lymphocytic(T–helper) Alveolitis • TBLB : 90% diagnostic DILD • Kveim- Siltzbach Test : Rarely done • Serum ACE II level : elevated

  12. Lungs ( 94 %) Upper airways ( 11.2 ) Lymph nodes ( 73 ) Skin ( 32 ) Eyes ( 21 ) Liver ( 21 ) Spleen ( 18 ) Bones ( 14 ) Salivary gland Heart Nervous system Joints Endocrine Kidneys Lacrimal glands Breast, Uterus Organ Involvement

  13. Lymphoma Tuberculosis Eosinophilic Granuloma Gout Rheumatoid Arthritis Primary hyper PTH Berylliosis Differential Diagnosis

  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. 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. 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. Sarcoidosis L/Ns

  18. Sarcoidosis (L/N + Lung)

  19. Sarcoid Uveitis Hypopion Posterior Synechiae: Iris deformity Cataract

  20. Sarcoid Dactylitis

  21. Sarcoid Skin Lesion

  22. Lupus Pernio

  23. Sarcoid Choreoretinitis

  24. Infiltrative Sarcoids Bronchoscopy

  25. BAL in Sarcoidosis

  26. Grade I

  27. Grade II A

  28. Grade II B

  29. Grade III

  30. Advanced Chronic Sarcoidosis

  31. Pathology of Sarcoidosis (1)

  32. Pathology of Sarcoidosis (2)

  33. Pathology of Sarcoidosis (3)

  34. Pathology of Sarcoidosis (4)

  35. Sarcodosis Pathology(TBLB)

  36. Sarcoid Skin Lesions Papule or Plaque

  37. Sarcoidosis F/39

  38. Sarcoidosis

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