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Sarcoidosis. Sung Chul Hwang, M.D. Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine. Definition.

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sarcoidosis

Sarcoidosis

Sung Chul Hwang, M.D.

Dept. of Pulmonary and Critical Care Medicine

Ajou University School of Medicine

definition
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
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
laboratory features
Laboratory features
  • Lymphopenia
  • Hyperglobulinemia
  • Hypercalcemia
  • Hypercalciuria
  • Increased ESR
  • Abnormal LFT, ALP increase
  • Immunologic abnormality
  • PPD anergy
  • Circulating immune complex present
slide11
PFT
  • Typical DILD pattern
  • Restrictive pulmonary insufficiency
  • FVC decrease
  • RV decrease
  • DLCO decrease
  • Decreased lung compliance “ stiff Lung”
x ray findings
X-ray Findings
  • Bilateral Hilar or Mediastinal Lymphadenopathy
  • Interstitial pulmonary infiltrates
  • Fibrosis
  • Nodular changes
  • Gallium Scan : “ Panda-Eye Sign”
slide13
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

Organ Involvement
differential diagnosis
Lymphoma

Tuberculosis

Eosinophilic Granuloma

Gout

Rheumatoid Arthritis

Primary hyper PTH

Berylliosis

Differential Diagnosis
treatment and prognosis
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
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
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 ”
sarcoid uveitis
Sarcoid Uveitis

Hypopion

Posterior Synechiae: Iris deformity

Cataract

sarcoid skin lesions
Sarcoid Skin Lesions

Papule

or Plaque