4. Definition
Multisystem chronic inflammation characterized by non caseating granulomas.
Caseating granulomas
TB
Non caseating granulomas
Sarcoidosis
5. Epidemiology
It occurs mainly in 3rd or 7th decade of life
F>M
Affects siblings of first- or second- degree relatives in
15% of patients with sarcoidosis.
10. Morphology
classic well formed noncaseating granuloma, each
composed of an aggregates of tightly clustered
epithelioid cell
langhans or foreign body giant cell
central necrosis is unusual
with chronicity the granulomas may become
enclosed within fibrous rims or may be eventually be
replaced by hyaline fibrous scars.
11. Morphology
Laminated concentration composed of calcium and
proteins known as schaumann bodies and stellate
inclusion as asteroid bodies enclosed with in giant
cells are formed in approximately 60% of granulomas.
Characteristic
12. LANGHANS' GIANT CELL
Langhans' giant cell in center of granuloma is surrounded
by epithelioid cells
Epitheliod cells produce collagenase, elastase, lysozyme and ACE
23. + Clinical Presentation
Most patients have the pulmonary manifestations, most commonly
presenting with incidental findings on CXR.
Interstitial disease
Symptoms include dry cough, dyspnea, and chest discomfort
Family history
Non smoker
Exposure to beryllium
History of tuberculosis
58. +Pleural disease
Rare < 1%
Pleural effusion.
Hemothorax.
Chylothorax (due to involvement of the mediastinal LN or
thoracic duct).
59. +
Air way involvement
May occur at any level
Bronchial wall thickening
Luminal abnormalities
Obstruction of lobular or
segmental bronchi
Mosaic perfusion
Air-trapping on expiration
65. +
Sarcoidosis of Lymph nodes
Lymphadenopathy
nodes are characteristically enlarged discrete
and sometimes calcified
Tonsil may affected in about quarter to one
third of the cases
66. + Lymphadenopathy
Typical
1.Bilateral hilar & right paratracheal LN,
2.Middle mediastinal LN occur in 50% of cases.
3.Left paratracheal, aorto-pulmonary & subcarinal
LN.
1-2-3 sign present in 95% of cases. This is called
Garland triad
76. + Skin
Lupus pernio- indurated blue purple swollen
shiny lesions on nose, cheeks, lips, ears and
fingers.
Papules, nodules, and plaques
Psoriatic like lesions
Lesions in scars and tattoos
92. + Liver
33% have hepatomegaly or biochemical evidence
of disease
Symptoms usually absent
Cholestasis, fibrosis, cirrhosis, portal
hypertension, and the Budd-Chiari syndrome
have been seen
93. +SPLEEN & LIVER GRANULOMAS
The small low attenuation lesions in the liver and
spleen in sarcoidosis.
102. + Nervous System
Cranial nerves, and peripheral nerves can be involved
7th nerve facial palsy is most common
Acute, transient, and can be unilateral or bilateral
HEREFORDT'S SYNDROME; facial palsy accompanied by
fever, uveitis, and enlargement of the parotid gland
103. +
T1-W POST GADOLINIUM MR IMAGE
Post contrast image of high signal intensity temporal lobe sarcoid lesion
106. + Kidney
Granulomatous interstitial nephritis produces renal failure
Develops over a period of weeks to months
Rapid response to steroid therapy
Kidney stones (nephrolithiasis) and nephrocalcinosis are
very unusual secondary to hypercalcemia and
hypercalciuria
108. + Kidney
Increased calcium absorption in the gut
Related to high levels of circulating 1,25-dihydroxy
vitamin D produced by mononuclear phagocytes in
granulomas
116. DIAGNOSIS
Identify noncaseating granulomas
Variety of infections
Transbronchial biopsies positive in 65-95%, even if no
lung parenchymal abnormalities imaged.
Tissue from mediastinoscopy
Scalene node biopsy
117. DIAGNOSIS
• Difficult to differentiate from chronic infections, fungal
diseases, T.B. and lymphoma.
• Based on combined clinical, radiologic and histologic
findings.
• Laboratory tests seldom important
118. THE KVEIM-SILTZBACH TEST
• If positive, is considered to be diagnostic of sarcoidosis
• Test material
• Positive test
• No corticosteroids
• Why does this reaction occur?
