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Sarcoidosis

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Sarcoidosis

  1. 1. - Dr.Akif A.B
  2. 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. 3. Etiology  Unknown  Few infective and non infective causes has been known  MC Infection - Propionibacterium acne > Mycobacterium
  4. 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. 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. 6. 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
  7. 7. Skin in Sarcoidosis  Erythema nodosum  Lupus pernio - 2nd most commonly invoved - Skin (25%)
  8. 8. 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)
  9. 9. Causes of B/L Hilar adenopathy  TB  Malignancy  Silicosis or beryliosis  Extrinsic allergic alveolitis  Sarcoidosis
  10. 10. 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
  11. 11. 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
  12. 12. 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
  13. 13. Prognosis  Many patients resolves within 2-5yr (>95%)  Patients requiring steroids in first 6months of presentation are more prone to have chronicity

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