Doctor, I Can't See

27th May 2016

Dr Lai Ee Ling

Hospital Sultan Ismail Johor Bahru

First presentation - 12/2015

•  Madam A •  33 years old •  6 months post-partum •  Onset of symptoms - 3 months post partum •  Presented with

–  Constitutional sx •  Prolonged intermittent fever

– Mucocutaneous sx •  Malar rash, photosensitivity, oral ulcer & alopecia

–  Arthalgia

Eye Symptoms

•  Bilateral blurring of vision for 1 month •  Painless •  Gradual Onset •  Progressively worsening

•  Obstetric history: – Para 3, delivered full term – No h/o miscarriage – No h/o PIH

•  FH : No h/o CTD •  Drug history: No h/o traditional medicine or

other drug history

Physical Examination

•  General examination: Alert, GCS 15/15 •  BP : 101/64 mmHg •  HR : 134 bpm •  T : 39.3 •  Lung : Clear •  CVS : S1S2 •  P/A : Soft, no hepatosplenomegaly

First presentation -12/2015

Opthalmologist review

•  Bilateral pupils round and reactive •  Bilateral eye EOM full •  RAPD negative •  Visual Acuity

- R finger counting - L hand movement

Fundoscope: Right Eye

Fundoscope: Left Eye

Purtscher's Like Retinopathy - Rare disease

Progress in Ward

•  Developed acute confusion & psychosis in ward –  auditory hallucination –  delusion –  disorientated to time

•  CECT brain : Normal •  LP : Not suggestive of intra-cranial infection •  Metabolic cause excluded (except the

hypothyroid)

Blood Investigation

30/12/2015

WCC 3.2Hb 9.4Plt 86Urea 2.3Crea4nine 48AST 95ALT 30T.Bilirubin 7FT4 6.7TSH 8.9

•  PBF : No evidence of hemolysis •  UFEME: Protein 2+, Blood negative •  24 Hour urine protein : 0.47g/day •  Serum albumin : 19 g/L •  Serum amylase : 31 u/L (normal) •  Hep B & C : negative •  HIV : Negative

Immunology Investigations

•  Positive Result

•  ANA 1:1280 (speckled) •  Anti-Sm >600u/mL •  Anti U1RNP 125u/mL •  Anti SSA > 600u/mL •  C3 : 0.59 (↓) •  C4 : 0.224 (↓) •  Anti-thyroglobulin Ig :

Positive (169 )

•  Negative Result

•  Anti SSB Negative •  Anti Scl 70 Negative •  Anti Centromere

Negative •  LA: Negative •  ACA : Rejected

CSF result

•  Opening pressure: 12cmH2O •  Clear and colorless •  No cell seen •  Indian Ink : negative •  Latx agglutinan : negative •  AFB direct smear & PCR : Negative •  C&S : negative

(1) SLE with mucocutananeous, joint, renal, hematological & CNS lupus (2) Purtscher's Like Retinopathy secondary to SLE (3) Autoimmune hypothyroidism

SLEDAI score

Descriptor ScoreOrganicbrainsyndrome 8Psychosis 8

Visual 8Vasculi4s 8Newmalarrash 4Oralulcer 4Alopecia 4Lowcomplement 4Thrombocytopenia 4TOTAL 60

Treatment

•  IV methylprednisolone 500mg OD for 3 days

•  Tapering dose of prednisolone •  Antibiotic for sepsis •  Refuse IV Cyclophosphamide

Clinic Review

5/2016 - Clinic Follow up 5 months later

Visual Progress

VisualAcuityatPresenta:on

VisualAcuityAt5months

RightEye FingerCoun4ng Fingercoun4ng

LePEye Handmovement 6/60

Right Eye Fundoscope After 5 month

Left eye fundoscope

Clinic Review

•  On tapering prednisolone dose •  Still refuse Cyclophosphamide •  Started on azathioprine in view of active

disease and Purtscher retinopathy – Developed azthioprine induced hepatitis

Investigation during follow up

30/12/2015 15/3/2016 17/5/2016WCC 3.2 6.0 5.4Hb 9.4 12.7 13.6Plt 86 237 246Urea 2.3 2.7 2.1Crea4nine 48 58 59AST 95 23 282ALT 30 32 426T.Bilirubin 7 5 102(DIrect

predominant)FT4 6.7 15.9TSH 8.9 1.06

Started AZA

Purtscher's Retinopathy

Purtscher-like Retinopathy

Purtscher's Retinopathy

•  First described by Otmar Purtscher in

1910 in a patient with head trauma •  Similar retinal appearance also descibe in

other conditions, eg pancreatitis

Epidemiology

•  Incidence: - 0.24 person per million per year (Including Purtscher and Purtscher's like retinopathy)

•  Prevalance in SLE :

- 0.14% Miguel et al 2013 Chan Wu et al 2014

Pathogenesis

•  Vaso-oclussive retinopathy •  Pre-capillary occlusion

–  Emboli (Fat, air, amniotic fluid) –  Vasculitis –  Leukoaggregation by complement activation –  Vascular endothelial dysregulation, follow by

endothelin induced vasculopathy

•  Microvascular infarct of retinal nerve fiber layer

Causes of Purtscher's Retinopathy

•  TRAUMATIC –  Head Trauma –  Chest compression –  Long bone #/Crush

injury –  Weight lifting –  Battered baby

syndrome –  Barotrauma –  Orthopaedic surgery

•  NON TRAUMATIC –  Acute pancreatitis –  Connective Tissue

Disease (SLE, SSc, Dermatomyositis)

–  Cryoglobulinemia –  Chronic renal failure –  TTP/HUS –  Pre-eclampsia –  HELLP –  Embolism (fat, air,

amniotic fluid)

Symptoms

•  Loss of visual acuity •  Loss of visual field

– Central – Paracentral – Arcuate scotoma – Peripheral field usually preserved

•  Combination loss of visual acuity AND visual field

Bilateral Vs Unilateral Eye

•  Bilateral(60%)> Unilateral •  Bilateral > common in cases precipitated

by systemic illness •  Unilateral > common in cases of chest

compression and trauma

•  No different in the extent of retina involvement

Diagnosis

•  Made on clinical ground – Sudden visual loss after the causal pathology – Fundoscopic signs

•  Typically in posterior pole of eye •  Cotton wool spots •  Intra-retinal haemorrhage •  Purtscher flecken (pathognomic)

Diagnosis

•  Supported by Intravenous flourescein angiography – Arteriolar obstruction – Leakage of dye from retinal arteriole, capillary

and venules

Flourescein Angiography

Arteriolar occlusion in right eye (arrow) Area of capillary leakage in the macule of left eye

Diagnosis Criteria

•  Migual et al •  At least 3 of 5 criteria

– Purstcher flecken – Retinal haemorrhage in low to moderate

number – Cotton wool spots (confine to posterior pole) – Probable explanatory etiologies – Complementary investigation compatible with

diagnosis

Acute Retinal Signs

•  Cotton wool spot (93%) •  Retinal haemorrhage (65%) •  Purtscher flecken (63%) •  Pseudo cherry red spot (26%) •  Macula edema (22%) •  Optic disc swelling (16%)

AIM Minguel et al 2013

Purtscher Flecken

•  Polygonal patches of retinal whitening •  Between affected retinal arterioles and

venules •  Characteristic clear zone •  50um unaffected on either side of retinal

arteries and pre-capillary arterioles

Purstcher Flecken

Clear zone between flecken and arterial wall

Chronic Retinal Signs

•  Optic atrophy (64%) •  Normalization of retinal appearnce (40%) •  Mottling of retinal pigment epithelium

(23%) •  Narrowing of retinal arterioles (4%)

AIM Miguel et al 2013

Chronic Retinal Changes

Pale optic disc Retinal pigment epithelial changes in macular

Treatment

•  No proven beneficial therapy •  Spontaneous visual improvement (variable

degree) even without therapy •  Steroid thereotically beneficial in systemic

vasculitis disease

Prognosis

•  Visual prognosis is variable

Purtscher's and Purtsher Like Retinopathy

Prognostic Factors

•  Visual Acuity Improvement – Male gender – Etiology : Trauma and acute pancreatitis – Absence of macular edema – Absence of pseudo cherry red spot

– No statistically significant difference in VA improvement between those reveived corticosteroid Vs NO corticosteroid

Purtscher-like Retinopathy in SLE

Conclusion

•  In SLE patient with purtscher's retinopathy – CNS lupus more common – SLEDAI on initial presentation is significant

higher (>20) – No different in prevalance of lupus nephritis – No different in prevalance of APS

Conclusion

•  POOR visual prognsis despite aggressive treatment (High dose steroid + CYC/MTX) – At 6/12 of treatment, 67% had optic atrophy – Pseudo-cherry spot at presentation had the

worse VA at 6/12 – Retinal neovascularization, require laser

therapy

Summary

•  Our patient had features that consistent with report by Chan wu et al – CNS lupus – High SLEDAI score – Poor visual prognosis

Reference

(1) Chan Wu, Rongping D, Fangtian D, Qian W. Purtscher-like Retinopathy in Systemic Lupus Erythematosus. American Journal of Ophthalmology 2014;158(6): 1335-1341.

(2) Agrawal A, McKibbin M. Purtscher's retinopathy:

epidemiology, cinical features and outcome. Br J Ophthalmol2007; 91(11):1456-1459

(3) Miguel AI, Henriques F, Azevedo LFR, Loureiro AJR,

Maber;ey DAL. Systemic review of Purtscher's and Purtscher like retinopathies. Eye (I.orul) 2013;19(6):512-518

Thank You