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 %) • 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

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