Sarcoidosis
Idiopathic. Granulomatous disease (non-caseating).
Systemic disease involving the lymph nodes, lungs, liver, heart etc.
Lung is the most commonly involved organ.
Idiopathic. Granulomatous disease (non-caseating).
Systemic disease involving the lymph nodes, lungs, liver, heart etc.
Lung is the most commonly involved organ.
Clinical features:
- 3rd or 4th decades
- More in African american
- 70% have increased serum ACE - An indicator of the granuloma burden on the body
Nuclear Medicine:
- Gallium (Ga 67 scintigraphy) avid during active phase. No uptake when in remission.
Degree of uptake corresponds to degree of severity
Confirms diagnosis of sarcoidosis
Differentiate active alveolitis from inactive fibrosis
Signs:
- Lambda sign: Hilar and Right paratracheal nodal uptake
- Diffuse pulmonary uptake
- Panda sign: Lacrimal and salivary glands inflammation
- PET scan: FDG avid during active phase. SUV can be >2.5
Acute Sarcoidosis
aka Lӧfgren syndromeClinical features:
- Fever, malaise, arthralgia +/- parotitis / uveitis
- Erythema nodosum
- Serum ACE level often increased
- Erythema nodosum
Has bilateral hilar lymphadenopathy, but absence of lung changes
Lungs:
Lambda sign on Gallium scan
- Bilateral hilar lymphadenopathy
- Right paratracheal lymphadenopathy
CXR:
-
Reticulonodular opacities, bilateral upper lobe dominant
-
Symmetric bilateral hilar, paratracheal,
aortopulmonary window lymphadenopathy
HRCT:
-
Lymphadenopathy
- 1-5mm nodules in centrilobular and subpleural distribution
- Upper lobe and peribronchovascular distribution
- Irregular interlobular septal thickening
- Architectural distortion
- 1-5mm nodules in centrilobular and subpleural distribution
- Upper lobe and peribronchovascular distribution
- Irregular interlobular septal thickening
- Architectural distortion
-
Advanced: Interstitial fibrosis- Peripheral honeycombing, Bullae
- Some cases (rare): Alveolar sarcoidosis = Peripheral consolidation + Mediastinal and Bilat.hilar lymphadenopathy
- Some cases (rare): Alveolar sarcoidosis = Peripheral consolidation + Mediastinal and Bilat.hilar lymphadenopathy
Radiographic staging:
- Stage 0: Normal CXR
- Stage 0: Normal CXR
-
Stage I: disease limited to mediastinal nodes
-
Stage II: involvement of lung and lymph nodes
-
Stage III: involvement of lung only
-
Stage IV: lung fibrosisDDx:
- When advanced, can mimic progressive massive fibrosis (PMF)
Cardiac
Result in:- Arrhythmia
- Ventricular aneurysms
- Sudden death
Head & Neck:
Involvement of glandular tissues, muscles, nerves.1. Optic nerve and EOM hypertrophy and enhancement - uveitis
2. Parotid glands and lacrimal glands exhibits heterogeneous bilateral symmetrical enlargement - chronic dacroadenitis, parotitis
Gallium scan: "Panda sign" where there is increased uptake of the parotid and lacrimal glands (Looks like the whole panda sitting on the person's neck / replacing the head)
Neurological system:
Neurosarcoidosis, 5% of sarcoidosis patientUsually lungs would have been involved already.
Granulomatous infiltration of the perineural tissue
Involvement of nerves, optic chiasm, hypothalamus, infundibulum, IAM
Presenting with cranial nerve deficits
Imaging:
Exhibit contrast smooth / nodular enhancements along the involved nerves, thickened leptomeninges, pachymeninges
Also enhancement in the perivascular space, ependyma
5% have solitary extra-axial mass
Complication: Hydrocephalus
Gastrointestinal:
Spleen:Splenomegaly
Present as splenic granulomas, +/- calcification
If present = advanced stage of Sarcoidosis.
Spontaneous splenic rupture (rare)
Lymph nodes:
Mesenteric nodes
Musculoskeletal:
Affect small bones of hands and feetReticulated trabecular pattern (lace-like)
Cyst-like lesions
Destruction of terminal phalanges
Diffuse sclerosis of the vertebral bodies
Osteolysis in skull
No comments:
Post a Comment