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IMPORTANT SYSTEMIC ASSOCIATIONS OF UVEITIS

4. Beh ç et disease. 5. Vogt-Koyanagi-Harada syndrome. 7. Tubulointerstitial nephritis and uveitis. 5. 5. IMPORTANT SYSTEMIC ASSOCIATIONS OF UVEITIS. 1. Spondylarthropathies. 2. Juvenile idiopathic arthritis. 3. Sarcoidosis. Systemic features. Ocular features.

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IMPORTANT SYSTEMIC ASSOCIATIONS OF UVEITIS

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  1. 4. Behçet disease 5. Vogt-Koyanagi-Harada syndrome 7. Tubulointerstitial nephritis and uveitis 5 5 IMPORTANT SYSTEMIC ASSOCIATIONS OF UVEITIS 1. Spondylarthropathies 2. Juvenile idiopathic arthritis 3. Sarcoidosis • Systemic features • Ocular features • Systemic features • Ocular features 6. Inflammatory bowel disease • Ulcerative colitis • Crohn disease

  2. Spondylarthropathies Gender HLA-B27 Acute iritis 70% males 95% 30% Ankylosing spondylitis 90% males 60% 20% Reiter syndrome equal 30% 10% Psoriatic arthritis

  3. Spondylarthropathies Sacroiliitis Peripheral arthritis Bowel inflammation Ankylosing spondylitis 100% 20% Common Reiter syndrome 60% 100% Uncommon Psoriatic arthritis 30% 100% Occasional

  4. Clinical features of Reiter syndrome Plantar fasciitis Painless oral ulceration Conjunctivitis Urethritis and circinate balanitis Keratoderma blenorrhagica Nail dystrophy

  5. Juvenile idiopathic arthritis Pauciarticular Polyarticular Systemic (60%) (20%) (20%) < 5 > 4 Variable Joints no. < 6 years Variable Variable Onset Absent Mild or absent Severe Systemic features 75% 40% 10% Positive ANA Iridocyclitis 20% 5% Absent

  6. High risk factors for uveitis • Girls • Early onset • Pauciarticular onset • ANA • HLA-DR5

  7. Complications of uveitis Posterior synechiae - 30% Cataract -20% Glaucoma due to PAS - 15% Band keratopathy - 10%

  8. Systemic Features of Sarcoidosis 1. Idiopathic, multisystem non-caseating granuloma 2. More common in blacks than whites 3. Presentation • Acute - third decade • Insidious - fifth decade 4. Organ involvement • Lungs - 95% • Thoracic lymph nodes - 50% • Skin - 30% • Eyes - 30%

  9. Acute sarcoidosis Erythema nodosum Parotid enlargement Facial palsy Hilar lymphadenopathy

  10. Classification of sarcoid lung lesions Stage 2 Hilar Lymphadenopathy and parenchymal infiltrates Stage 1 Hilar Lymphadenopathy Stage 3 Parenchymal Infiltrates alone Stage 4 Fibrosis and Bronchiectasis

  11. Sarcoid skin lesions Granulomata Lupus pernio Indurated, purple-blue lesions On face, buttocks and extremities

  12. Anterior segment lesions in sarcoidosis Conjunctival granuloma Lacrimal gland involvement and dry eyes

  13. Iridocyclitis in sarcoidosis Acute non-granulomatous Chronic granulomatous In young patients with acute sarcoid In older patients with chronic sarcoid

  14. Posterior segment lesions in sarcoidosis Candlewax drippings Subtle periphlebitis Peripheral neovascularization Vitritis and snowballs

  15. Fundus granulomata in sarcoidosis Retinal and preretinal Choroidal Optic nerve head

  16. Behçet Disease 5 1. Idiopathic multisystem disease 2. Presentation - third to fourth decade 3. Most prevalent in Mediterranean region and Japan 4.Associated with HLA-B5 in Turkey and Japan 5. Major diagnostic criteria • Oral aphthous ulceration (100%) • Genital ulceration (90%) • Skin lesions (80%) • Uveitis (70%)

  17. Mucocutaneous ulceration in Behçet disease Oral aphthous ulceration - painful, recurrent Genital ulceration

  18. Skin lesions in Behçet disease Acneiform Erythema nodosum Pustule after scratching skin (pathergy test) Lines after stroking skin (dermatographism)

  19. Vascular lesions in Behçet disease Migratory thrombophlebitis of extremities Obliterative thrombophlebitis of major internal veins

  20. Uveitis in Behçet disease Acute iritis Retinitis Occlusive periphlebitis Diffuse leakage

  21. Signs of Vogt-Koyanagi syndrome Alopecia Granulomatous iridocyclitis Poliosis Vitiligo

  22. Harada syndrome Progression of Harada disease Multifocal choroiditis Exudative retinal detachment Multifocal sensory retinal detachments

  23. Inflammatory bowel disease Ulcerative colitis Crohn disease • Stricture and ‘rose thorn’ • ulceration • Large bowel ulceration Acute iritis - uncommon • Acute iritis - uncommon

  24. Tubulointerstitial nephritis and uveitis (TINU) Renal histology Urine • Most frequently affects women and children • Proteinuria and renal • failure • Hypersensitivity reaction to drugs • Good response to systemic • steroids • Bilateral, recurrent anterior uveitis

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