CME: Grand Round Presentations Disc Swelling Revisited

1. CME: Grand Round PresentationsDisc Swelling Revisited Dr Alex Lau, Medical Officer Dr Chin Chee Fang, Medical Officer Dr Johnson Tan, Medical Officer Chairman: Dr Goh Kong Yong, Senior Consultant

2. Case 1 Dr Alex Lau Medical Officer Tan Tock Seng Hospital

3. Ms TSY • 37/Chinese/housewife • No PMH • presented in Nov 05 • 1/52 Hx of bilateral BOV • Lt>Rt redness and chemosis

4. Presentation • Denies headache/nausea/vomiting • No pain/rash/recent URTI • Had episode of non-specific GI symptoms 3 wks ago a/w mild fever • S/B GP, but no significant improvement. • BOV occurred 2 wks later • Symptoms felt slightly better than 1 week ago

5. Examination

6. Examination

7. What does it show? Bilateral optic disc swelling with macular star • What are the differential diagnoses?

8. Differential diagnosis • Compressive neuropathy • Malignant hypertension • Posterior scleritis • Optic disc edema with macular star (ODEMS) – ‘Neuroretinitis’

9. Differential diagnosis ODEMS

10. What to do next? Investigations • Physical parameters • Blood • Neuroimaging

11. Investigations BP 144/90mmHg B-scan Normal CXR Normal MTT 10x9mm Neuro-imaging CT brain Normal MRI brain Scleral thickening

12. Investigations Blood tests FBC Hb 10.8, WBC/plt normal ESR 73, CRP 1.0 VDRL/TPHA negative Bartonella IgG/IgM negative Toxoplasma IgG/IgM negative

13. Investigations ANA ≥1/640 Anti-ds DNA >800 Rheumatoid factor negative

14. What is wrong with the patient? Impression: ODEMS 2o to ? Connective Tissue Disorder • Further investigations?

15. Further investigations APTT Elevated (x2 repeat) 60.4, 60.3 sec (range 28-39) Lupus anticoagulant present ACA IgG/IgM negative

16. Final impression • ODEMS 2o to Systemic Lupus Erythematosus (SLE) • with ? 2o Antiphospholipid syndrome (APS)

17. Further management • Oral prednisolone 1mg/kg • Referral to RAI • Final Diagnosis: SLE complicated by proteinuria & autoimmune haemolytic anaemia (AIHA) • Not APS (because does not satisfy clinical criteria - no previous thrombotic event(s) or miscarriages) • Currently on immunosuppression without anticoagulation

18. Follow up visit

19. Systemic Lupus Erythematosus • Autoimmune, non-organ specific connective tissue disorder • 20% have ocular involvement

20. Diagnostic Criteria • ≥4 of below: • Malar rash • Discoid rash • Photosensitivity • Oral or nasopharyngeal ulcers • Nonerosive arthritis • Serositis • Renal disorder • Neurological disorder • Haematological disorder • Immunological disorder • ANA +ve • Ocular manifestation notpart of criterion • Hence, high index of suspicion required to prevent systemic & ocular morbidity from delayed diagnosis & treatment

21. Systemic Lupus Erythematosus • 100 cases per 100,000/year (Asia) vs 1.8~20 cases (Western) • 90% of patients are women • HLA-DR2, -DR3 • Trigger factors?: microbes, drugs, chemicals, sunlight • Dysfunction in immune regulation • Hyperreactivity of B-cells with expression of autoantibodies • Abnormal regulation of T-cells • Deposition of immune-complexes with tissue injury

22. Ocular Manifestations of SLE • Most common: KCS (25%) • Anterior segment • Severity of episcleritis and scleritis may closely mirror the activity of systemic disease. • Necrotizing scleritis rare • 2nd most common: Retinal involvement • Classic: CWS vasculopathy (avascular zones) • Infiltration of vessel walls with fibrillar material (i.e. not ‘true’ vasculitis) • Widespread vascular constrictions and thrombus • Vessel walls typically free of inflammatory cells. • Deposition of IgG with C1q and C3 • 88% of patients with lupus retinopathy have active systemic disease and a significantly decreased survival rate. (Stafford-Brady et al. Lupus retinopathy: patterns, associations & prognosis. Arthritis Rheum 1988;31(9):1105-10) • Uveitis may occur in the absence of retinal involvement. • Choroidopathy less common. • Multifocal RPE and serous retinal detachments • Choroidal changes appear to be subclinical. • Neuroophthalmic manifestations

23. Common Posterior Segment Manifestations in SLE • Retinal haemorrhages • Cotton wool spots • Hard exudates • Disc swelling • Arteriolar narrowing • Venous engorgement • 2o retinal vein / artery occlusion

24. Antiphospholipid Syndrome • Primary: occurs in isolation Secondary: a/w CTDs esp SLE, sarcoidosis • 35% SLE have ↑ antiphospholipid antibodies • Diagnostic criteria: Defined as the presence of antiphospholipid antibodies, arterial or venous thrombosis (systemic & ocular), recurrent spontaneous abortions, and thrombocytopenia • Clinical – Episode of vascular thrombosis or pregnancy morbidity / foetal loss • Laboratory - ACA, LAC positive

25. Ocular Manifestations in APS • Multisymptomatic, potentially sight-threatening • 90% of patients with 1o APS have ocular involvement • 30% of them can be asymptomatic • VA is severely impaired in 15% of the eyes. • ACA seen in 85% of patients with SLE with retinal vasculitis(Durrani. Surv Ophthalmol 2002;47(3):215-38) • ACA IgG is a highly specific marker for AION a/w GCA • ACA IgA found in 29% of the patients with ARN • Esp in those with aqueous HSV PCR –ve.

26. Castanon et al. Ocular vasoocclusive disease in primary APS. Ophthalmology 1995; 102(2):256-62 Bolling JP et al. The APS. Curr Opin Ophthalmology 2000;11(3):211-3. Lima Cabrita FV et al. ACA and ocular disease. Ocul Immunol Inflamm 2005;13(4):267-70.

27. Thank you A presentation by The Eye Institute @ Tan Tock Seng Hospital