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