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Uvea & Sclera. SINA A. SHOOL MD www.drsinashool.blogspot.com. Uveitis – “inflammation of the uveal tract”. 1) Anatomic classification. Anterior Uveitis: iris & Anterior part of the ciliary body (pars plicata
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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