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 )

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

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

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

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

14. Organ Involvement 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

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

16. 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

17. 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

18. 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 ”

19. Sarcoidosis L/Ns

20. Sarcoidosis (L/N + Lung)

21. Sarcoid Uveitis Hypopion Posterior Synechiae : Iris deformity Cataract

22. Sarcoid Dactylitis

23. Sarcoid Skin Lesion

24. Lupus Pernio

25. Sarcoid Choreoretinitis

26. Infiltrative Sarcoids Bronchoscopy

27. BAL in Sarcoidosis

28. Grade I

29. Grade II A

30. Grade II B

31. Grade III

32. Advanced Chronic Sarcoidosis

33. Pathology of Sarcoidosis (1)

34. Pathology of Sarcoidosis (2)

35. Pathology of Sarcoidosis (3)

36. Pathology of Sarcoidosis (4)

37. Sarcodosis Pathology(TBLB)

38. Sarcoid Skin Lesions Papule or Plaque

39. Sarcoidosis F/39

40. Sarcoidosis