Sarcoidosis
General
·
Widespread formation of non-caseating granulomas
·
3:1 female:male and 14:1 black:white predominance
·
ACE (angiotensin converting enzyme) elevated in 70%
·
Kveim skin test
o
Positive in (70%) but rarely used today
·
Lofgren Syndrome:
o
Acute bilateral hilar adenopathy, fever,
erythema nodosum and arthralgia
·
Intrathoracic disease (90%)
Stage I
·
Adenopathy alone (43%)-Stage 1
·
Intrathoracic adenopathy in 80%
·
Location
o
Bilateral hilar and (R) paratracheal
o
Most common (75-90%)
o
“1-2-3 sign”, “Pawn-broker’s sign”, “Garland sign”
◊
Lymph
nodes which enlarge in sarcoid are broncho-pulmonary nodes which are more
peripherally placed in the lung than the nodes which enlarge in lymphoma
which are true hilar nodes
◊
Therefore, there is frequently a
separation between nodes and heart in sarcoid which is not seen in
lymphoma
·
Unilateral hilar nodes rare (3-8%)
·
Egg-shell calcification hilar nodes in long-standing sarcoid
o
Rare
o
DDX: Silicosis
Frontal view of the chest
shows bilateral hilar adenopathy with "separation"
of nodes from heart since broncho-pulmonary nodes in sarcoid
are more peripherally placed than are true hilar nodes that enlarge in
lymphoma
Stage II
·
Adenopathy and parenchymal disease (41%)-Stage 2
·
Adenopathy usually decreases as parenchymal disease
increases
·
About 1/3 of patients with adenopathy develop parenchymal
disease
Stage III
·
Parenchymal disease alone (30%)
·
Adenopathy does not develop subsequent to parenchymal
disease
·
If adenopathy develops, think of lymphoma or TB
Stage IV
·
End-stage lung disease-Stage 4
o
Diffuse fibrosis
o
Bronchiectasis-honeycomb lung
o
Multiple cysts
Patterns of lung disease
·
Reticulonodular (46%)
·
Acinar pattern (20%)
·
Larger nodules
o
“Alveolar sarcoid” (2%)
o
Coalescence of numerous interstitial
granulomas
o
Air bronchograms present
·
DDX
o
Alveolar cell ca
o
Alveolar proteinosis
o
Lymphoma
Uncommon manifestations
·
Associated with TB (13%)
·
Pleural effusion (2%)
o
Usually exudate with lymphocytic predominance
·
Cavitation of nodules (<1%)
·
Fungus ball formation in chronic sarcoid cavities (usually
TB)
·
Focal pleural thickening
·
Bronchostenosis with lobar atelectasis
·
Pulmonary arterial hypertension
o
Cor pulmonale
·
Pneumothorax 2° chronic lung disease
Extrathoracic disease
·
Peripheral adenopathy (30%)
·
Liver
o
Hepatomegaly
·
Spleen
o
Splenomegaly
·
Bone-especially hands
·
Skin
o
Erythema nodosum
o
Lupus pernio (raised purplish nodules)
·
Muscle
o
Myopathy
·
Eyes
o
Uveitis
o
Uveoparotid fever
·
CNS
o
Granulomatous meningitis
o
Facial nerve palsy
·
Myocardium
o
Arrhythmias
o
Heart block
o
Cardiomyopathy
·
Salivary gland
o
Parotid enlargement
Prognosis
·
3/4 show complete resolution of hilar adenopathy
·
1/3 show complete resolution of parenchymal disease
·
20% have irreversible pulmonary fibrosis
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