CME: Grand Round Presentations Disc Swelling Revisited

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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

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Case 1

• Dr Alex Lau
• Medical Officer
• Tan Tock Seng Hospital

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Ms TSY

• 37/Chinese/housewife
• No PMH
• presented in Nov 05
• 1/52 Hx of bilateral BOV
• LtgtRt redness and chemosis

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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

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Examination
Right Left
VA 6/24?6/18 6/24?NI
Ishihara 10/15 8/15
Confrontation VF HM in infero-nasal quadrant Normal
Pupils No RAPD No RAPD
Ant segment Normal Normal
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Examination
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• What does it show?
• Bilateral optic disc swelling with macular star
• What are the differential diagnoses?

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Differential diagnosis

1. Compressive neuropathy
2. Malignant hypertension
3. Posterior scleritis
4. Optic disc edema with macular star (ODEMS)
   Neuroretinitis

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Differential diagnosis

• ODEMS

Infective Inflammatory
Tuberculosis Sarcoidosis
Syphilis CTDs
Cat-scratch disease (Bartonellosis)
Toxoplasmosis
Viral
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• What to do next?
• Investigations
• Physical parameters
• Blood
• Neuroimaging

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Investigations

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

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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

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Investigations

• ANA 1/640
• Anti-ds DNA gt800
• Rheumatoid factor negative

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

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Further investigations

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

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Final impression

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

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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

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Follow up visit
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Systemic Lupus Erythematosus

• Autoimmune, non-organ specific connective tissue
  disorder
• 20 have ocular involvement

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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 not part of criterion
• Hence, high index of suspicion required to
  prevent systemic ocular morbidity from delayed
  diagnosis treatment

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Systemic Lupus Erythematosus

• 100 cases per 100,000/year (Asia) vs 1.820 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

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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 198831(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

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Common Posterior Segment Manifestations in SLE

1. Retinal haemorrhages
2. Cotton wool spots
3. Hard exudates
4. Disc swelling
5. Arteriolar narrowing
6. Venous engorgement
7. 2o retinal vein / artery occlusion

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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

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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
  200247(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.

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Symptoms BOV, transient diplopia, transient field loss, amaurosis fugax, photopsia, asymptomatic
Conjunctiva telangiectasia, aneurysm, episcleritis
Cornea KP, limbal keratitis
Anterior chamber Uveitis ( hypopyon)
Vitreous VH, vitritis
Optic nerve Disc oedema, AION
Retina RAO /or RVO, venous tortuosity, aneurysm, CWS, vasculitis, macular serous detachment, ARN
Castanon et al. Ocular vasoocclusive disease in
primary APS. Ophthalmology 1995
102(2)256-62 Bolling JP et al. The APS. Curr
Opin Ophthalmology 200011(3)211-3. Lima Cabrita
FV et al. ACA and ocular disease. Ocul Immunol
Inflamm 200513(4)267-70.
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Thank you
A presentation by The Eye Institute _at_ Tan Tock
Seng Hospital