This document discusses sarcoidosis, a disease characterized by non-caseating granulomas in multiple organs. Key points: - It most commonly involves the lungs and is more prevalent in women, especially in the 3rd and 6th decades of life. - The cause is unknown but it involves an abnormal immune response mediated by T-cells and macrophages forming granulomas. Lung involvement is seen in over 90% of cases. - Diagnosis involves clinical features, imaging showing hilar lymphadenopathy, elevated ACE levels in 60% of acute cases, and biopsy demonstrating non-caseating granulomas. Most patients see resolution within 2-5 years.

1. - Dr.Akif A.B

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.

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

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

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

18. Prognosis
 Many patients resolves within 2-5yr (>95%)
 Patients requiring steroids in first 6months of presentation
are more prone to have chronicity