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Sarcoidosis
Sung Chul Hwang, M.D.Dept. of Pulmonary and Critical Care Medicine
Ajou University School of Medicine
Definition An Idiopathic systemic disorder character
ized by accumulation of lymphocytes and monocytes in many organs forming noncaseating, epitheloid granuloma and subsequent conformational changes in the involved organs
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
Pathogenesis ( 1 )
Pathogenesis ( 2 )
Pathogenesis ( 3 )
Pathogenesis ( 4 )
Laboratory features• Lymphopenia• Hyperglobulinemia• Hypercalcemia• Hypercalciuria• Increased ESR• Abnormal LFT, ALP increase• Immunologic abnormality• PPD anergy• Circulating immune complex present
PFT
• 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
• 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
Differential Diagnosis
• Lymphoma• Tuberculosis•Eosinophilic Granu
loma
• Gout• Rheumatoid Arthritis• Primary hyper PTH• Berylliosis
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
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
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