2. Introduction
Non – caseating granulomatous inflammatory disease
Most common involvement - Lungs
2 or more organs should be involved for specific diagnosis
Womens > Mens
Bimodal : 3rd decade and 6th decade
5% have a family history of sarcoidosi.
3. Etiology
Unknown
Few infective and non infective causes has been known
MC Infection - Propionibacterium acne >
Mycobacterium
4. Inclusion body seen in Giant cell in
sarcoidosis
S - Schaumann bodies
A - Asteroid bodies
R - Residual bodies
C
O
I
D
O
S
I
S
5. Pathophysiology
T-Cells are the most important in pathogenesis . (T-Helper – CD4)
HLA-DRB1 is involved in 95% patient - Good prognosis.
Macrophages are also involved.
Antigen presenting cell (APC) binds toT-cell and activates it.
T-cell releases IL-2 and macrophage releasesTNF and IFN-¥.
Thus HIV patients with low CD4 count rarely develops Sarcoidosis.
Granulomas resolves in most of the patient on its own. But in 20% patient
chronicity is seen. IL-8 is responsible for chronic changes.
6.
7. Lung in sarcoidosis
MC involved >90%
Infiltrates primarily involves upper lobe.
50% patients presents with obstructive symptoms due to
external compression.
Rest presents as ILD Staging
Stage 1 Hilar adenopathy alone
Stage 2 Hilar adenopathy with pulmonary infiltrates
Stage 3 Pulmonary infiltrates
Stage 4 fibrosis
8. Skin in Sarcoidosis
Erythema nodosum
Lupus pernio
- 2nd most commonly invoved - Skin (25%)
9. Other organ involved
Eye - Anterior Uveitis
Liver - Elevated Alkaline phophatase (Cholestatic pattern)
Hematologial - Lymphopenia (due to sequestration at inflammation site)
Cardiac - Arrhythmias and CHF
Hypercalcaemia - Due to increased production of 1,25(OH)Vit.D
Renal nephropathy is due to hypercalcaemia.
Myalgias and arthralgias
Breast lesion
Cranial nerve - 7th (Bell’s [Palsy)
10. Causes of B/L Hilar adenopathy
TB
Malignancy
Silicosis or beryliosis
Extrinsic allergic alveolitis
Sarcoidosis
11. Investigations
Lymphopenia
Chest Xray
Gallium scan : PET has replaced it now : Increase activity in parotid and
Lacrimal gland (PANDA sign) or right paratracheal or Left Hilar area
(LAMBDA Sign)
Hilar lymphadenopathy >2cm in short axis is more in favour of Sarcoidosis.
PET Scan : to look for other granulomatous areas
BAL : Lymphocytosis or CD4/CD8 >3.5
PANDA Sign
12.
13. Serum ACE
Elevated in 60% of acute patients
20% of chronic patients
Patients on ACE Inhibitors will have falsely low values
False positive :
Gaucher’s disease
Hyperthyroidism
MiliaryTB
Leprosy
Silicosis,Asbestosis, Beryliosis
TB
Diabetes : But rise is <50% of normal
14.
15. Kveim Siltzbar Procedure
Specific diagnostic test
Tissue of the spleen of sarcoid patient is taken
Injected intradermally in patient with suspect of Sarcoidosis
Skin Biopsied after 4-6weeks
If shows non caseating granuloma – confirm sarcoidosis
Test is obsolete now due to lack of tissue
16.
17.
18. Prognosis
Many patients resolves within 2-5yr (>95%)
Patients requiring steroids in first 6months of presentation
are more prone to have chronicity