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Explore the anatomical, clinical, etiological, and pathological classifications of uveitis, its pathophysiologic mechanisms, clinical features, and grading systems for aqueous flare and opacification. Learn about uveitis associated with joint diseases like Juvenile Rheumatoid Arthritis and HLA-B27 disorders.
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Uvea & Sclera SINA A. SHOOL MD www.drsinashool.blogspot.com
1) Anatomic classification • Anterior Uveitis: iris & Anterior part of the ciliary body (pars plicata • Intermediate Uveitis: posterior part of the ciliary body (pars plana) • Posterior Uveitis • Panuveitis
Anatomical - Anterior Uveitis • Juvenile Rheumatoid Arthritis • Fuchs’ heterochromic Iridocyclitis • Ankylosing Spondylitis • Reiter’s Syndrome
Anatomical - Intermediate Uveitis • Pars Planitis • Sarcoidosis • Multiple Sclerosis • Lyme Disease
Anatomical - Posterior Uveitis • Toxoplasmosis • Histoplasmosis • Toxocariasis • Serpiginous chorioretinitis • Birdshot retinochoroiopathy
Anatomical - Diffuse Uveitis • Sympathetic Ophthalmia • VKH syndrome • Behcet’s disease
(2) Clinical Classification • Acute Uveitis: sudden, symptomatic onset; persists for <6 weeks • Chronic Uveitis: insidious onset, may be asymptomatic; persists for months
(3) Etiological Classification • Exogenous Uveitis: due to external injury to the uvea or invasion of micro-organisms from the outside • Endogenous Uveitis: caused by micro-organisms or other agents from inside the patient www.drsinashool.blogspot.com
(4) Pathological Classification • Granulomatous Uveitis - epithelioid and giant cells, lymphocytes • Non-granulomatous Uveitis - lymphocytes and plasma cells
(5) Pathophysiologic Mechanisms (Opremcak) • Traumatic • Surgical vs. non-surgical • Infectious • Bacterial, Viral, Fungal, Protozoal, Helminthic, Insect, Animal • Immunologic • Masquerade Diseases
Immunologic • Type I hypersensitivity disorders • Ig E- mediated • Mostly ocular surface disease hay fever, vernal & atopic keratoconjunctivitis • Type II • Antibody dependent cellular cytotoxicity (autoantibodies) • Ocular Cicatricial Pemphigoid; Lens-induced Uveitis
Immunologic • Type III • Immune Complex formation & deposition • Ankylosing Spondylitis, SLE, JRA, Behcet’s disease, PAN, Wegener’s granulomatosis, inflammatory bowel disease, other collagen vascular diseases • Type IV • Cell mediated immune response • Interleukins • Contact dermatitis, Phylectnulosis, , Fuchs HI, Sympathetic Ophthalmia, VKH, Birdshot retinochoroidopathy, Pars Planitis, Leber’s Idiopathic Stellate Neuroretinitis, Sarcoidosis
Masquerade Syndrome • Vascular tumors • Infectious • Congenital anomalies • Metabolic/Degenerations (amyloidosis, retinitis pigmentosa) • Neoplastic Disease (lymphoma, leukemia)
Aqueous Flare 0 Optically empty 1+ Faint 2+ Moderate- clear iris & lens 3+ Marked- hazy iris & lens 4+ Intense- fibrin with no motion of cells Aqueous cell (1x3 mm beam @ 5-10 °) 0 0 Rare Rare Occasional 1-2 cells Trace 2-4 cells 1+ 5-15 2+ 16-25 3+ 26-60 4+ > 60 cells Grading System for Anterior Chamber Flare & Cell
Flare (Vitreous Opacification) 0 Good View of NFL 1+ Clear ON & vessels but hazy NFL 2+ ON & vessels hazy 3+ ON only 4+ No ON Cell (1x3 mm beam in anterior vitreous) Trace 0-10 cells 1+ 10-20 2+ 20-30 3+ 30-100 4+ > 100 cells Grading System for Vitreous Cell and Opacification
Keratouveitis: with corneal involvement • Sclerouveitis: with scleral involvement • Keratosclerouveitis
Anterior Uveitis • Juvenile Rheumatoid Arthritis • Fuchs’ heterochromic Iridocyclitis • Ankylosing Spondylitis • Reiter’s Syndrome
Anterior Uveitis 1) Uveitis Associated with Joint Disease a) Juvenile Rheumatoid Arthritis (JRA) - chronic, bilateral, non-granulomatous - arthritis of at least 3 mos’ duration in a child less than 16 years - females - insidious onset - uveitis may precede the onset of arthritis
JRA (cont) • types of arthritis: pauciarticular onset <4 joints polyarticular onset > 5 joints systemic onset
Anterior Uveitis 2) Uveitis Associated with Joint Disease(cont) b) HLA-B27-associated - Ankylosing spondylitis - Reiter’s syndrome - Psoriatic arthritis - Inflammatory Bowel Disease (IBD)
HLA-B27-associated Ankylosing Spondylitis • inflammatory arthritis affecting axial skeleton • Fixed flexion deformity due to fusion of spine and sclerosis of sacroiliac joints • Klebsiella & Chlamydial infections
HLA-B27-associated Reiter’s Disease • Triad: urethritis, conjunctivitis, arthritis • Extraarticular features: Mouth ulcers, skin lesions (keratoderma blenorrhagica), penile lesions (circinate balanitis), nail changes, cardiovascular changes, genitourinary lesions
HLA-B27-associated Psoriatic Arthritis - Psoriasis: skin disease due to epidermal hyperproliferation
HLA-B27-associated Inflammatory bowel disease • Crohn’s disease and ulcerative colitis • Gastrointestinal and articular manifestations • Systemic manifestations include skin lesions, oral ulcerations, hepatobiliary disorders, urogenital involvement
Anterior Uveitis 3) Fuch’s Heterochromic Iridocyclitis • unilateral, insidious, third or fourth decade • No symptoms; low grade uveitis • Early cataract • Fine, stellate, evenly distributed deposits on the posterior corneal surface; iris atrophy; telangiectatic vessels in the angle • Synechiae is rare