uvea and joints ophthalmologists

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  • 1. The Uvea and Joints Syed Atiqul Haq Professor of Rheumatology BSMMU

2. RDs and Uveitis Spondyloarthropathies (SpAs) Juvenile idiopathic arthritis (JIAs) Sarcoidosis Behcets disease Sjgren syndrome SLE Systemic vasculitis Relapsing polychondritis 3. Spondyloarthropathies Ankylosing spondylitis (AS) Reactive arthritis (ReA) Psoriatic arthritis (PsoA) Enteropathic arthritis Undifferentiated SpA 4. Features of Spondyloarthropathies Young male Axial pain Family history Pauciarticular Big joints Asymmetric Enthesopathies Rheumatoid factor negative Radiological sacroiliitis HLA-B27 5. Reactive Arthritis Preceding diarrhea/dysentery/ST-urethritis Conjunctivitis Acute onset Full remissions & relapses 6. Ankylosing Spondylitis Features of reactive, psoriatic, enteropathic - absent Bilateral sacroiliitis 7. Uveitis in ReA & AS Acute, recurrent, may recur in the other eye Symptoms occur 1-2 days before clinical signs M:F= 2:1 Resolves within 3 months Prognosis excellent 8. Enteropathic Arthritis Chronic diarrhea, recurrent abdominal pain Colonoscopic/imaging features of Ulcerative colitis Crohns disease 9. Psoriatic Arthritis FH of Psoriasis Psoriatic plaques - hidden Nail lesions Hand joint involvement, particularly DIPs 10. Uveitis in PsoA & Enteropathic Arthritis Chronic Insidious onset Bilateral Posterior to lens F>M 11. Definition Arthritis in One or more joints Persisting 6 weeks or more Begins before 16th birthday Has no other known cause 12. Classification Oligo-articular JIA: commonest Persistent Extended Enthesitis-related JIA (ERA) Polyarthritis: RF negative RF positive Psoriatic arthritis Systemic onset (Stills) Undifferentiated arthritis 13. Uveitis in JIA Common (20-75%) in oligo-articular & ERA Occurs in 6% of polyarticular Other risk factors: Female gender ANA positive Eye & joint disease evolve independently 14. Characteristics of Uveitis in JIA Anterior uveitis, rarely posterior Bilateral in 2/3rd Insidious onset, acute in ERA Chronic: lasts >2 yrs, often >15 yrs Remitting & relapsing 60% Persistent 20% Single episode 20% 15. Uveitis in Oligo-articluar JIA Often asymptomatic Visual disturbance, photophobia, headache: rare Pain and redness in 25% Acutely painful in ERA Prognosis poor, potentially blinding Periodic slit lamp surveillance for 1st 4 years 3 mnthly 2 yrs, 6 mnthly 2 yrs, then yrly up to puberty 16. Complications of Uveitis in JIA (May be Diagnostic) Band keratopthy Posterior synechie Cataracts Glaucoma Blindness 17. Cardinal Features Usually a subacute illness: HO a few weeks Symptoms: Fever Cough Arthritis: mono- or oligo-articular, often ankle Signs: Bilateral parotid enlargement Erythema nodosum Uveitis CXR-PA view: Bilateral hilar lymphadenopathy 18. Sarcoidosis: Ophthalmic Features 20-40% develop eye disease Ophthalmic features: Uveitis: commonest Dry eyes Optic neuritis Blepharitis Orbital disease Asymptomatic conjunctival or scleral nodules: diagnostic Retinal vasculitis: periphlebitis, focal cuffing of veins, new vessesls on disc or periphery 19. Sarcoidosis: Characteristics of Uveitis Anterior in black, posterior in 25%, white and elderly female Acute or chronic, relapsing Granulomatous mutton fat keratic precipitates 20. Mutton Fat Keratic Precipitates 21. Behcets: Cardinal Features > 3 episodes oral aphthii/yr + > 2 of following: Recurrent genital ulceration Uveitis or retinal vasculitis Erythema nodosum, pseudofolliculitis, papulopustular lesions, acneform nodules Positive pathergy test 22. Behcets: Ocular Involvement Eyes involved in 70-80% pts 25% become blind Eye conditions: Uveitis Retinal vasculitis Glaucoma Cataract 23. Uveitis in Behcets Recurrent, 2/3rd cases bilateral Panuveitis commonest in both sexes Minority female may have isolated anterior uveitis Posterior frequent in male Often severe Resolves spontaneously Resolution is incomplete Blindness more often due to concomitant retinal vasculitis 24. Steps of Evaluation History Examination Laboratory investigation Referral 25. Demographics, Joint Pain Age 16-young adults: SpAs Sex Male: AS, ReA, ERA Female: PsoA, Oligoarticular JIA Recurrent, short episodes, Diarrhea, dysentery, ureth discharge prior to onset ReA Limb predominance LL: ReA, AS, UL: PsoA Large vs. small Large: ReA, AS, Small: PsoA Axial SpAs 26. Other Systems, Past & Family History Cough, breathlessness Sarcoidosis Abdominal pain, bloody diarrhea Enteropathic Oral and genital ulcers Behcets Past HO joint pain, IBP, PRE, pso SpAs FH of psoriasis PsoA FH of RDs, painful red eye SpAs 27. Steps of Examination General examination Systemic examination (screening) Examination of MSK system Examination of the eye 28. Bilateral Parotid Enlargement Sarcoidosis 29. Aphthous ulcer Behcets disease 30. Psoriatic Plaques Psoriatic arthritis 31. Psoriatic Nail Changes Psoriatic arthritis 32. Erythema Nodosum Sarcoidosis Behcets Reactive arthritis Post-yersinial 33. Keratoderma Blenorrhagicum Reactive arthritis 34. Systemic Examination Findings Suggested diagnosis Bilateral pulm. creps Sarcoidosis Regurgitant murmurs in precordium AS, ReA Lump in RIF CD with enteropathic arthritis Neuropthies (mononeuritis multiplex) Sarcoidosis, Behcets 35. Examination of MSK System Gait Tenderness in hand joints Restriction of movement of spine SI joint tenderness Tenderness at foot joints Enthesitis 36. Routine Urinalysis Hb%, ESR, CBC 37. Selective Investigations CXR-PA view X-ray of SI joints ANA HLA-B27 CT scan of chest 38. Indications for CXR-PA Fever Cough, breathlessness Parotid enlargement Erythema nodosum 39. X-Ray SI Joints Views: X-ray pelvis AP view including both SI joints X-ray both SI joints oblique view Suitable for younger pts Indications: Adults with suspected SpAs Children with suspected ERA, when clinically not obvious 40. ANA Oligo-articular JIA: assessing need for periodic slit lamp Features of SLE Young woman Fever Butterfly rash Hair loss Proteinuria 41. HLA-B27 In adults, suspected SpA Clinically not obvious SI joint X-ray normal/equivocal In children, confusion between oligoarticular JIA and ERA 42. CT Scan of Chest Eye features of sarcoidosis in the absence of clinical or CXR evidence of systemic disease: Granulomatous mutton fat keratic precipitates Nodules on iris and choroid Associated retinal vasculitis, periphlebitis, focal cuffing of veins, new vessels on disc or periphery 43. Treatment Outline Initial treatment Topical glucocorticoids Disease resistant to initial treatment Oral glucocorticoids Glucocorticoid Sparing Agents (GSA) 44. Indications for Oral steroid Refractory to local therapies, peri-ocular triamcinolone Refractory bilateral disease interfering with ADL Behcets disease 45. Protocols of Oral Steroid Short (+intermittent), 40 to 60 mg for 2 weeks: Acute uveitis associated with AS, ReA and ERA Long term: Refractory chronic, 4 phases: 40 to 60 mg till suppression of inflammation Gradual tapering Maintenance with minimum dose: 6-12 months Gradual withdrawal 46. Glucocorticoid Sparing Agents Azathiprine Methotrexate Mycophenolate mofetil Cyclosporin Tacrolimus Cyclophosphamide Thalidomide Infliximab 47. Indications For GSAs Severe refractory uveitis failed to respond to 40- 60 mg/d prednisolone Remission not maintained with pred @