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Sarcoidotic
Uveitis
Bipin Bista
Resident
Ophthalmology
National medical College
& Teaching Hospital
• Multisystem granulomatous disease which affects every
organ.
• Lungs, thoracic LN & Skin – commonest
• Adrenal glands are spared.
• Initially, termed as skin disease – Hutchinson (1869)
• Boeck termed it as sarcoid – biopsy.
• Association with uveitis in 1900’s
• Sarcoid uveitis associated with Facial Nerve palsy &
uveoparotid fever : Heerfordt’s syndrome.
• Epidemiology : Black > White, Common : Female.
• Etiology : Unknown.
ETIOLOGY
Etiology
• Macrophage :::: inflammatory response.
• Release cytokines (TNF –α & IL-2) infiltration of T-
helper lymphocytes release cytokines & recruit other
inflammatory cells to site of inflammation.
• In response, granulomas are formed &
immunoglobulin produced in large quantities by
stimulated B lymphocytes are sent into circulation.
• Sensitization & resultant immune response to
endogenous lung antigen or inhaled agents.
• Environmental factors : molds & insecticides.
Clinical features
• Asymptomatic.
• Respiratory symptoms, fever, fatigue or weight loss.
• Abnormal chest X-ray or laboratory tests.
• Biopsy : Non-caseating,granulomatous,non-infectious
inflammatory process.
• 2/3rd of case – remission with in a decade.
• 1/3rd of case - recurrent, progressive – significant organ
damage.
• Ocular involvement is about 26 % in chronic cases.
• B/L but may be U/L as well as markedly asymmetric.
Clinical Manifestation
Ocular examination
• Proper evaluation of lacrimal apparatus & conjunctiva.
• Conjunctiva ( 7-17%)
• Lacrimal gland (7-26%)
Anterior Uveitis
• Most common
• 2/3rd ( 50-60%) will have
chronic granulomatous uveitis.
• Most have Mutton fat KPs,
large & dense, Iris nodules.
• Inflammatory vs Malignancy.
• Chronic form : 4th-6th decade &
causes cataract (8-17%) &
glaucoma (11-23%) with
increase in duration
• 15-45 % associated with acute
iridocyclitis .
• Mild vitritis ( occasional)
Posterior Segment
• Less common . 6-33%
• Clump of cells & proteinacous debris –
snowballs
• ‘Candle wax dripping’ (en taches de
bongie)
• Perivenous sheathing rarely
periarterioral.
• Deep yellow choroidal lesions
consistent with D-F nodules &
mottling of pigment epithelium (36%)
• Serous RD – large granuloma
• Chronic Cystoid Macular Oedema.
• Neovascularization of Optic Disc
(15%)
• Retinal & subretinal neovascularization
(R > SR)
• Peripapillary or macular subretinal
neovascular membrane.
Systemic Involvement
• Liver (10% Liver
enzyme)
• Hypercalciuria
• Child & infants.
• Blau syndrome
• Skin lesions are common.
• Erythema nodusom &
granulomatous sarcoid
nodules
• Colitis
• Behςet’s Disease
• Arthritis (Non-
granulomatous synovitis)
• Neurological involvement
(25%), facial nerve.
• Hilar adenopathy
• Lymphadenopathy
• Splenomegaly
PATHOLOGY
• Sarcoid granuloma : whorls of epitheloid cells
surrounding multinucleated giant cells, mononuclear
cells, fibroblasts & lymphocytes.
• Under polarised light : Asteroid Bodies, Schaumann’s
body & crystalline inclusions.
• Th1 driven inflammatory process , CD4+ T
lymphocytes
Diagnosis
• Requires biopsy
• Asymptomatic
• Abnormal chest x-ray.
• 60% granuloma on transbronchial lung biopsy even with
Normal radiography (Stage 0 disease)
• 90% parenchymal involvement.
• CT–Scan more useful as it’s more sensitive.
• 27-55 %-Conjunctival biopsy
• 22%-lacrimal biopsy
LABORATORY TESTS
• ACE is pre-dominantly pulmonary macrophage & vascular
endothelium ( elevated in 60-90%)
• May be normal in quiescent disease.
• ACE : TB, leprosy, silicosis, cirrhosis, asbestosis &
histoplasmosis.
• Present in aqueous humor & even in CSF.
• Serum lysosymes
• Hypercalciuria & hypercalcemia.
• Lymphopenia & elevated liver enzyme.
• KVEIN TEST : Spleen antigen : 6 weeks : 80%
• Gallium scan
• PFT
• Limited diffusing capacity.
Treatment
• Asymptomatic with hilar adenopathy (Doesn’t require)
• 2 main indications :
―Symptomatic pulmonary disease
―Uveitis
• Acute anterior inflammation by corticosteroids but
frequently peri-ocular steroid injection is required.
• Chronic uveitis requires PULSE Therapy.
• Immunosuppresants
• Methotrexate : low dose effective & safe
• Hydroxychloroquine
• TNF-α Inhibitor : Infliximab
• Pars plana lensectomy
• ECCE
• Laser photocoagulation : Subretinal neovascularization
GOALS
• Control inflammatory condition
• Prevent permanently visually impairing changes such as
photoreceptor damage caused by Cystoid Macular
Oedema.
Reference:
- Nussenblatt and whitecup 4th
edition
- Myron yanoff 4th edition

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Sarcoidotic uveitis

  • 2. • Multisystem granulomatous disease which affects every organ. • Lungs, thoracic LN & Skin – commonest • Adrenal glands are spared. • Initially, termed as skin disease – Hutchinson (1869) • Boeck termed it as sarcoid – biopsy. • Association with uveitis in 1900’s • Sarcoid uveitis associated with Facial Nerve palsy & uveoparotid fever : Heerfordt’s syndrome. • Epidemiology : Black > White, Common : Female. • Etiology : Unknown.
  • 4. Etiology • Macrophage :::: inflammatory response. • Release cytokines (TNF –α & IL-2) infiltration of T- helper lymphocytes release cytokines & recruit other inflammatory cells to site of inflammation. • In response, granulomas are formed & immunoglobulin produced in large quantities by stimulated B lymphocytes are sent into circulation. • Sensitization & resultant immune response to endogenous lung antigen or inhaled agents. • Environmental factors : molds & insecticides.
  • 5. Clinical features • Asymptomatic. • Respiratory symptoms, fever, fatigue or weight loss. • Abnormal chest X-ray or laboratory tests. • Biopsy : Non-caseating,granulomatous,non-infectious inflammatory process. • 2/3rd of case – remission with in a decade. • 1/3rd of case - recurrent, progressive – significant organ damage. • Ocular involvement is about 26 % in chronic cases. • B/L but may be U/L as well as markedly asymmetric.
  • 7. Ocular examination • Proper evaluation of lacrimal apparatus & conjunctiva. • Conjunctiva ( 7-17%) • Lacrimal gland (7-26%)
  • 8. Anterior Uveitis • Most common • 2/3rd ( 50-60%) will have chronic granulomatous uveitis. • Most have Mutton fat KPs, large & dense, Iris nodules. • Inflammatory vs Malignancy. • Chronic form : 4th-6th decade & causes cataract (8-17%) & glaucoma (11-23%) with increase in duration • 15-45 % associated with acute iridocyclitis . • Mild vitritis ( occasional)
  • 9. Posterior Segment • Less common . 6-33% • Clump of cells & proteinacous debris – snowballs • ‘Candle wax dripping’ (en taches de bongie) • Perivenous sheathing rarely periarterioral. • Deep yellow choroidal lesions consistent with D-F nodules & mottling of pigment epithelium (36%) • Serous RD – large granuloma • Chronic Cystoid Macular Oedema. • Neovascularization of Optic Disc (15%) • Retinal & subretinal neovascularization (R > SR) • Peripapillary or macular subretinal neovascular membrane.
  • 10. Systemic Involvement • Liver (10% Liver enzyme) • Hypercalciuria • Child & infants. • Blau syndrome • Skin lesions are common. • Erythema nodusom & granulomatous sarcoid nodules • Colitis • Behςet’s Disease • Arthritis (Non- granulomatous synovitis) • Neurological involvement (25%), facial nerve. • Hilar adenopathy • Lymphadenopathy • Splenomegaly
  • 11. PATHOLOGY • Sarcoid granuloma : whorls of epitheloid cells surrounding multinucleated giant cells, mononuclear cells, fibroblasts & lymphocytes. • Under polarised light : Asteroid Bodies, Schaumann’s body & crystalline inclusions. • Th1 driven inflammatory process , CD4+ T lymphocytes
  • 12. Diagnosis • Requires biopsy • Asymptomatic • Abnormal chest x-ray. • 60% granuloma on transbronchial lung biopsy even with Normal radiography (Stage 0 disease) • 90% parenchymal involvement. • CT–Scan more useful as it’s more sensitive. • 27-55 %-Conjunctival biopsy • 22%-lacrimal biopsy
  • 13. LABORATORY TESTS • ACE is pre-dominantly pulmonary macrophage & vascular endothelium ( elevated in 60-90%) • May be normal in quiescent disease. • ACE : TB, leprosy, silicosis, cirrhosis, asbestosis & histoplasmosis. • Present in aqueous humor & even in CSF. • Serum lysosymes • Hypercalciuria & hypercalcemia. • Lymphopenia & elevated liver enzyme. • KVEIN TEST : Spleen antigen : 6 weeks : 80% • Gallium scan • PFT • Limited diffusing capacity.
  • 14. Treatment • Asymptomatic with hilar adenopathy (Doesn’t require) • 2 main indications : ―Symptomatic pulmonary disease ―Uveitis
  • 15. • Acute anterior inflammation by corticosteroids but frequently peri-ocular steroid injection is required. • Chronic uveitis requires PULSE Therapy. • Immunosuppresants • Methotrexate : low dose effective & safe • Hydroxychloroquine • TNF-α Inhibitor : Infliximab • Pars plana lensectomy • ECCE • Laser photocoagulation : Subretinal neovascularization
  • 16. GOALS • Control inflammatory condition • Prevent permanently visually impairing changes such as photoreceptor damage caused by Cystoid Macular Oedema.
  • 17. Reference: - Nussenblatt and whitecup 4th edition - Myron yanoff 4th edition