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![• Br J Ophthalmol. 2019 Feb 23. pii: bjophthalmol-2018-313356. doi:
10.1136/bjophthalmol-2018-313356. [Epub ahead of print]
• Revised criteria of International Workshop on Ocular Sarcoidosis (IWOS)
for the diagnosis of ocular sarcoidosis
Results The survey and subsequent workshop reached
consensus agreements of the revised criteria for the
diagnosis of OS as follows:
(1) other causes of granulomatous uveitis must be ruled
out;
(2) seven intraocular clinical signs suggestive of OS;
(3) eight results of systemic investigations in suspected
OS and
(4) three categories of diagnostic criteria depending on
biopsy results and combination of intraocular signs and
results of systemic investigations.](https://image.slidesharecdn.com/sarcoidosis-190903151724/85/Sarcoidosis-52-320.jpg)
More Related Content
Sarcoidosis
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- 3. • Sarcoidosis-associated uveitiswas first described in the early 1900s and in 1936 • Heerfordt – Danish Ophthalmologist – Heerfordt syndrome – Uveoparotid fever syndrome
- 4. Definition Sarcoidosis is achronic multisystem inflamatory disorder characterized histologically by the accumulation of non caseating epitheloid granulomas in affected tissues. Main organs involved are lungs, lymphnodes, eyes, skin, heart,brain, liver, kidney. Ocular involvement - 40% of patients with sarcoidosis, most commonly uveitis Usually bilateral – asymmetric
- 5. ETIOLOGY AND PATHOGENESIS •Etiology – unknown Proposed mechanisms • Triggering of immune responses by an inifectious or non-infectious agent in the environment in a genetically susceptible host. • HLA B8, HLA DRB1 – sarcoidosis • HLA-DRB1*0401 allele - ocular involvement (ACCESS)
- 6. • Sarcoidal granulomas areorganized, structured masses composed of macrophages and their derivatives, epithelioid cells, giant cells, and T cells. • TNFa – induction and maintainence of granuloma • IL 2 – recruit more number of CD 4 cells in granuloma and alters CD4/CD8 ratio
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- 12. EYELID • Small milletshaped to large nodules on eyelids • Lesions are non tender and rarely ulcerate • eye irritation and eyelid deformities with mucocutaneous notching. • Extensive scarring of the posterior lamella causing entropion and trichiasis
- 13. CONJUNCTIVA • 25% ptswith sarcoid uveitis have conj changes • Most common lesion is granuloma • Seen as small round yellow nodules • Usually present in infero palpebral conj and fornix • Exxtensive – resolve with symblepharon formation
- 14. SCLERITIS • In sarcoidosispresents as nodular, anterior diffuse, posterior • Not associated with severe pain • Non necrotizing • Responds to steroids
- 15. CORNEA • Band shapedkeratopathy – chronic inflammation, calcium deposition in bowman membrane • Interstial keratitis and nummular keratitis can also occur • Peripheral ulcerative keratitis • SPK – after KCS
- 16. UVEITIS • Uveitis associatedwith sarcoidosis can present as anterior, intermediate, posterior or panuveitis • Sarcoidosis is well known as a cause of granulomatous uveitis • “Granulomatous” uveitis is used when at least one of the following clinical signs are observed: 1) large mutton-fat keratic precipitates (KPs) (accumulation of inflammatory leukocytes that deposit on the corneal endothelium), 2) iris or trabecular meshwork nodules, or 3) choroidal granuloma
- 17. ANTERIOR UVEITIS M.c manifestation •Seen on 2/3rd cases • Typical chronic B/L Non caseating granulomatous uveitis • Acute or chronic ACUTE ANTERIOR UVETIS • Abrupt onset asso with pain redness and photophobia • Blurred vision • examination reveals cells,flare and fine KPs • Likely to occur at the onset of systemic disease • Well responds to treatment
- 18. CHRONIC ANTERIOR UVEITIS •Mutton fat KPs • Iris nodules • Trabecular meshwork nodules on gonio • Tent shaped PAS on gonio • May lead to glaucoma and cataract • More difficult to treat and has higher incidence of complications and worst prognosis
- 20. INTERMEDIATE UVEITIS • Inflammationof vitreous,pars plana and peripheral retina • Symptoms:Floaters, defective vision, pain. • Pars plana exudates and accumulation of white blood cells and vitreous debris on the retinal surface may be characterized as snow banks and snow balls • Cluster – snow ball • Linear array or strands – string of pearls
- 21. POSTERIOR UVEITIS • Inflammationof the retina, and choroid is less common but more visually disabling than inflammation in the anterior segment • occurs in 6–33% of patients with sarcoidosis. CLINICAL FEATURES Candle wax dripping ( periphlebitis) Multifocal chorioretinal lesion Retinal granuloma Cystoid macular edema Dalen fuchs nodules
- 22. PERIPHLEBITIS • Perivascular inflammation •appears as yellowish or grey-white perivenous sheathing. • Midperipheral periphlebitis is characteristic. • Candle wax drippings or taches de bougie Periphlebitis associated with segmental cuffing, extensive sheathing and perivenous infiltrates. • Occulusive periphlebitis
- 23. CHORIORETINAL LESION • Choroidallesions are uncommon & vary in appearance: • multiple small pale-yellow infiltrates, • sometimes with a punched-out appearance most numerous inferiorly. • Multiple large confluent infiltrates • Multifocal choroiditis – secondary CNVM poor visual prognosis • Retinal granuloma
- 25. CYSTOID MACULAR EDEMA •CME is a common cause of vision loss in patients with sarcoid uveitis. • It may accompany severe active inflammation or retinal vasculitis in the posterior pole. • It generally responds to corticosteroid therapy. • Epiretinal membrane formation can also occur.
- 26. CHOROIDAL GRANULOMA • Choroidalgranuloma may be unifocal or multifocal, and may vary in size from small (Dalen-Fuchs-like nodules or sarcoid spots) to large . • Peripheral granulomas are unlikely to cause visual disturbance, but central lesions may lead to severe visual impairment. • Choroidal neovascularization can occur.
- 27. • Extensive chorioretinalatrophy and scar secondary to longstanding involvement from ocular sarcoidosis • Exudative retinal detachment can rarely be seen in patients with ocular sarcoidosis, particularly those with large chorioretinal granulomas • Intraretinal hemorrhages and retinal or optic disc neovascularization with subsequent vitreous hemorrhage may complicate retinal vasculitis secondary to sarcoidosis
- 28. OPTIC NERVE Rare ,may lead to rapid vn loss • Four types of optic nerve disease described 1.Papilloedema sec to severe raised ICP and CNS involvement 2.Infiltration of optic nerve by non caeseating granulomas 3.Retrobulbar neuritis 4.Glaucomatous optic atrophy secondary to raised IOP FOR long periods
- 29. LACRIMAL GLAND • Dacryoadenopathy •Asymptomatic swelling over the lateral aspect of orbit • Loss of functionality may lead to keratoconjunctivitis sicca
- 30. CATARACT • prevalence ofcataracts in chronic sarcoid uveitis is 8–17% • Complicated cataract occurs due to inflammation • Steroid
- 31. GLAUCOMA • prevalence ofglaucoma varies from 11% to 23%. • pupillary block - due to posterior synechiae. • trabecular meshwork damage or occlusion by inflammatory cells can cause glaucoma. • Severe or chronic anterior chamber inflammation may cause angle closure glaucoma secondary to peripheral anterior synechiae formation
- 32. CHILDHOOD SARCOIDOSIS • lesscommon in the pediatric population • Under 5 yrs • typically characterized by a granulomatous anterior uveitis, polyarticular arthritis, rash, and lymphadenopathy. • Pulmonary involvement 1/3rd • Vision loss – amblyopia • Aggressive treatment • D/D – Blau syndrome.
- 33. SYSTEMIC MANIFESTATION OFSARCOIDOSIS
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- 37. Blau syndrome • AD •Children <12yr • Granulomatous arthritis, uveitis, and dermatitis • Lack of visceral involvement, vasculitis • Camptodactyly • No pulmonary disease, no hypercalcemia • DD – childhood sarcoidosis
- 38. DIFFERENTIAL DIAGONOSIS GRANULOMATOUS UVEITISTb, Syphilis, leprosy, vkh, toxoplasmosis, herpetic uveitis ISOLATED IRIS NODULES Iris neoplasms, metastatic carcinomas, leukemic infiltrates, seeding from retinoblastoma INTERMEDIATE UVEITIS multiplesclerosis,pars planitis CHOROIDAL LESIONS tb, birdshot retinopathy, metastatic tumours, large cell lymphomas, vkh, serpigrnous choroidopathy, behcets disease , CMV retinitis, Sympathetic ophthalmia, histoplasmosis
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- 40. INVESTIGATION • Serum ACELevels :- – May be elevated , SENSITIVITY – 60%, SPECIFICITY – 70% – Increased in other granulomatous disorders not in malignancy • Serum lysozyme level • Hypercalcemia • Elevated ALP levels • Hypercalciuria • MANTOUX – Negative response • KVEIM SILTZBACH’s TEST • BIOPSY of the involved organ • Increased CD4/ CD8 ratio > 2.5 in BAL fluids, vitreous humour • Transbronchial biopsy
- 41. ANGIOTENSIN CONVERTING ENZYME •Normal value = 8 – 52 U/L SENSITIVITY – 60%, SPECIFICITY – 70% • ACE is predominantly produced in pulmonary macrophages and vascular endothelium. • In addition ACE is produced by epithelioid cells present in granulomas • elevated in approximately 60% of patients • Conversely, a normal serum ACE does not exclude diagnosis – ACE inhibitor pts, small lesion.
- 42. KVEIM SILTZBACH TEST •Kveim antigen was prepared from the spleens of patients with proven sarcoidosis • Injected intradermally into a patient with suspected sarcoidosis. • The presence of granulomatous inflammation in a skin biopsy performed 6 weeks after the injection was considered diagnostic of sarcoidosis. positive in ~80% of patients with sarcoidosis.
- 43. TISSUE BIOPSY • Thegold standard for the diagnosis of sarcoidosis is a tissue biopsy • The most common biopsy samples are retrieved from the lungs, lymph nodes, skin, conjunctivae, lacrimal glands or orbital tissues.
- 44. RADIOLOGICAL INVESTIGATION CHEST XRAY BILATERAL HILAR LYMPHADENOPATHY
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- 47. GALLIUM 67 SCAN:- • LAMBDASIGN • PANDA SIGN
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- 49. DIAGNOSIS OF OCULARSARCOIDOSIS • In 2009, the first International Workshop on Ocular Sarcoidosis (IWOS) published the international criteria for the diagnosis of ocular sarcoidosis • The members of the IWOS identified - 7 clinical signs suggestive of ocular sarcoidosis, - 5 laboratory investigations in suspected ocular sarcoidosis, and - 4 diagnostic terms for ocular sarcoidosis
- 51. DIAGNOSIS OF OCULARSARCOIDOSIS
- 52. • Br JOphthalmol. 2019 Feb 23. pii: bjophthalmol-2018-313356. doi: 10.1136/bjophthalmol-2018-313356. [Epub ahead of print] • Revised criteria of International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis Results The survey and subsequent workshop reached consensus agreements of the revised criteria for the diagnosis of OS as follows: (1) other causes of granulomatous uveitis must be ruled out; (2) seven intraocular clinical signs suggestive of OS; (3) eight results of systemic investigations in suspected OS and (4) three categories of diagnostic criteria depending on biopsy results and combination of intraocular signs and results of systemic investigations.
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- 54. TOPICAL • Prednisolone acetate1% (8 times /day) • Prednisolone sodium phosphate 0.5% • Difluprednate 0.05% ( 4times/day) • Dexamethasone 0.1% • Loteprednol 0.2% and 0.5% • Fluorometholone 0.1% and 0.25% • Betamethasone 1% PERIOCULAR:- • Triamcinolone acetonide (20–40 mg) • Transconjunctival route • Can be repeated after 4-6wks INTRAOCULAR IMPLANTS • Dexamethasone implant (0.7 mg) • Triamcinolone acetonide (1–4 mg) • Fluocinolone acetonide (0.19 mg and 0.59 mg)
- 55. SYSTEMIC CORTICOSTEROIDS • Life threatening •Vision threatening • Systemic conditions • Bilateral involvement • Prednisolone 1- 1.5 mg/kg body wt • Taper to lowest effective doses SYSTEMIC IMMUNOSUPPRESIVE AGENTS • When steroids are required for longer durations
- 56. Immunosuppressive METHOTREXATE • 7.5-25mg/week • Folicacid 5mg 6times/week • RFT, LFT, blood count monitoring AZATHIOPRINE • 1-3mg/kg /day • Max 3mg/kg/day • LFT, blood count monitoring
- 57. Immunosuppressive CYCLOPHOSPHAMIDE • For induction –500mg iv – slow infusion • Maintainence – 1-2mg/kg/day – Max 3mg/kg/day • Oral MESNA for hemorrhagic cystitis OTHERS • CYCLOSPORINE – 2-5mg/kg/day – Max 10mg/kg/day • LEFLUNOMIDE – Alternative to MTX – Less toxicity – Chronic sarcoidosis • TACROLIMUS – Lichenoid type of skin sarcoidosis
- 58. BIOLOGICALS :- ANTI TNFαINHIBITORS • Infliximab (3–5 mg/kg loading, then 3–10 mg/kg every 4–8 weeks IV ) • Adalimumab (loading dose 80 mg, then 40 mg every 2 weeks SC) • Golimumab (50 mg SC monthly) • Certolizumab 50 mg S.C monthly INTERLEUKIN INHIBITORS • IL-6 receptor antagonists:- - Toclizumab (4 mg/kg IV every 4 weeks) • IL- 1 receptor antagonist:- - ANAKINRA 100mg/day sc • IL- 2 receptor anatagonists:- - DACLIZUMAB ( 1-2 mg/kg everfy 4 weeks) CHIMERAL MONOCLONAL AB AGAINST CD 20 Rituximab (1 g every 2 weeks IV)
- 59. TREATMENT • Scleritis &external eye disease – NSAIDS • cutaneous lesions on the eyelids may be treated with intralesional triamcinolone injection or oral chloroquine • Conjunctival lesion & KCS – cyclosporine eye drops • Orbital lesion – oral steroid/ immunosuppresive drugs/ surgery
- 60. SURGICAL TREATMENT CATARACT Eyequiet for 3 months Pre op steroids 1 week Cataract surgery GLAUCOMA trabeculectomy with or without antimetabolites tube shunt procedures SUBRETINAL NEOVASCULARISATION Laser photocoagulation CME persists without active inflammation intravitreal injections of bevacizumab or ranibizumab However, treatment of associated inflammation and/or edema should be maximized prior to considering surgery to avoid postoperative inflammation
- 61. PROGNOSIS; Visual prognosis ofocular sarcoidosis may vary depending upon severity and chronicity of eye inflammation, a delay in presentation to a specialist, and ocular complications secondary to uveitis. OUTCOME • Spontaneous regression • Improvement with treatment • Persistence of lesions • Relapse, recurrence • Deteriortion
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