Sarcoidosis
Sung Chul Hwang, M.D.
Dept. of Pulmonary and Critical Care Medicine
Ajou University School of Medicine
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
Epidemiology
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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
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Lymphopenia
Hyperglobulinemia
Hypercalcemia
Hypercalciuria
Increased ESR
Abnormal LFT, ALP increase
Immunologic abnormality
PPD anergy
Circulating immune complex present
PFT
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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
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Lungs ( 94 %)
Upper airways ( 11.2 )
Lymph nodes ( 73 )
Skin
( 32 )
Eyes
( 21 )
Liver
( 21 )
Spleen
( 18 )
Bones
( 14 )
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Salivary gland
Heart
Nervous system
Joints
Endocrine
Kidneys
Lacrimal glands
Breast, Uterus
Differential Diagnosis
• Lymphoma
• Tuberculosis
• Eosinophilic
Granuloma
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Gout
Rheumatoid Arthritis
Primary hyper PTH
Berylliosis
Treatment and Prognosis
• Spontaneous Remission : Hilar or
Mediastinal L/Ns  80 – 90 %
• Lung Parechymal lesion is rarely selflimiting
• Treament of choice : Prednisone, others,
indomethacin, MTX, Cyclosporin,
Cytoxan, Allopurinol, etc
Indicators of Sarcoid Activity
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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