interesting case

Chiombon. Chua. Corpuz. Cua. David. De Vera. Detera. Diaz. Din. Dizon. Eugenio. Evangelista.

1

General Data

CT 29/Female DOA: 03 August 2010

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Chief Complaint

Blurring of Vision

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History of Present Illness

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Past Medical History

Allergies: none No previous illnesses,

hospitalizations and blood transfusions

TB exposure: (-) Injuries: Burn 2nd and 3rd degree

burns (1992) Medications: none

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Personal and Social History Diet: Mixed Smoking: 3.5 pack years Alcohol: 2 bottles of beer/day from

23-27 years Substance abuse: Denies illicit drug

use

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Family History

Diabetes Mellitus- Grandmother Glaucoma- (-) Hypertension – (-) Cancer – (-)

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Review of Systems

(-) Weight change , (-) fever & chills (-) rashes; (-)pruritus;(-) bruising (-) dizziness; syncope (-) blurring of vision, eye discharge (-) hearing changes; pain; discharge; vertigo; (-) epistaxis; obstruction; nasal discharge, gum bleeding; oral

ulcers (-) cough, (-) dyspnea, (-) night sweats (-) chest pain; (-)dyspnea on exertion; (-)PND; (-)palpitations (-) abdominal pain; (-)dysphagia, (-)nausea, (-)vomiting,(-)

Dyspepsia (-)diarrhea, (-) constipation (-) indigestion, (- flatulence (-) frequency; urgency; dysuria; nocturia; dribbling (-) arthralgia/arthritis (-) trauma; (-)back pain

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Physical Examination • Conscious, Coherent, Ambulatory, not in Cardiorespiratory distress

• Vital signs: 110/80, PR 74 Regular, RR: 20, Temp 36.6 C

• Warm, moist skin, no active dermatoses (+) Scars, both upper extremeties

• Pink palpebral conjunctiva, anicteric sclerae, pupils L= 3-4mm, RTL, R= 2-3 mm RTL anisocoric, slightly hyperemic conjunctiva

• Septum midline, turbinates not congested, no nasoaural discharge, impacted cerumen

• Moist buccal mucosa, no oral and palatal lesions, nonhyperemic posterior pharyngeal wall, tonsils not enlarged

• No limitation of neck movement, no palpable cervical lymphadenopathy

• (-) thyroid gland enlargement

• No breast mass palpated, no discharge

• Thorax: no deformities, no intercostal and subcostal retractions, symmetrical chest expansion, equal tactile fremiti, resonant, clear and equal breath sounds

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Physical Examination •Adynamic precordium, AB at the 5th LICS MCL, no LV heave, thrills, no lifts, s1 louder than s2 at the apex, s2 louder than s1 at the base, no murmurs

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Physical Examination

Globular abdomen, Normoactive bowel sounds, (-) bruits, (-) tenderness, guarding, masses, (-) Murphy’s sign, Nonpalpable gallbladder, Traube’s space not obliterated

Pulses full and equal, (-) cyanosis, edema, clubbing

14

Eye Examination

Visual Acuity Right Eye Left Eye

Without Correction 20/50, J12 20/200, J12

Pin hole 20/30 20/50

Amsler Grid

(+) Scotoma on OU(+) Metamorphopsia

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Eye Examination

External eye examination: Eyelids: non tender Lashes: not matted Conjunctiva: Hyperemic Sclera: anicteric Cornea: Clear Anterior Chamber: deep Lens: Clear Pupils: Anisocoric L= 3-4mm, RTL, R= 2-3 mm

RTL Iris: Pigmented

16

Eye Examination

EOM: Full and equal Fundoscopy:

(+) ROR

(+) Blurred disc Margins , OU (+) Serous Retinal Detachment, OU (+) Hyperemia of choroid

Tonometry: 18mmHg OU Fluorescein angiography:

hyperfluoresence of optic disc

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NeuroExam

Conscious, coherent, oriented to 3 spheres, able to follow commands, GCS 15 E4V5M6

(-) Anosmia, anisocoric pupils L= 3-4mm, RTL, R= 2-3 mm RTL; Can smile, frown, raise eyebrows, uvula midline, can shrug shoulders, turn head from side to side against resistance tongue deviated to right. Motor exam: RUE: 5/5, RLE:5/5, LUE and LLE 5/5.

Reflexes: ++ Cerebellar- can do APST, FTNT with ease, No tremors No sensory deficit (-) Babinski, Nuchal rigidity, Brudzinski, Kernig’s sign

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Initial Assessment

Incomplete Vogt-Koyanagi-Harada Disease, Acute uveitic stage

r/o TB Uveitis

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Uveitis

is inflammation of the Uvea Tract, middle section of the eye.

Uvea Tract has three parts: the Iris (the colored part of the eye) Ciliary body (behind the iris,

accomodation, aqueous humor) Choroid (the vascular lining tissue

underneath the Retina).

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DIFFERENTIAL DIAGNOSIS

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Our Patient Sympathetic Ophthalmia

Tuberculosis

(-) History of Trauma (-) Previous TB exposure

Previous History of Trauma , perforating eye injury

Previous Infection of Tuberculosis

Sees “Flashes of light” RednessBlurred Vision

PhotophobiaRedness Blurred visionFloaters

Unilateral Cases of ocular involvement

(+) ROR (+) Blurred disc Margins , OU (+) Serous Retinal Detachment, OU(+) Hyperemia of choroid(+) Hypreflouresence of optic disc

Soft yellow white exudates in the deep layer of the Retina

Granulomatous keratic precipitates or choroidal granulomas are present

Incomplete Vogt-Koyanagi-Harada Disease, Acute uveitic stager/o TB uveitis

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DIAGNOSTICS

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Laboratories

CBC Chest X-ray AFB smear PPD

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CBC

HGB 126

RBC 4.03

HCT 0.38

MCV 94

MCH 31.3

MCHC 33.3

RDW 12.30

MPV 7.40

PLT 255

WBC 7.90

Neut 0.52

Seg 0.52

Lymph 0.44

Eosin 0.04

ESR 8.0

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Chest X-ray

Lung fields are clear The heart is not enlarged Both hemidiaphragm and

costophrenic sulci are intact Impression: No significant chest

findings

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AFB Stain

No Acid Fast Bacilli seen in 300 oil immersion fields on both routine and concentration methods

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Therapeutics

Methylprednisolone 1mg/kg/day per IV for 3 days

Ranitidine 150mg/tab, 1 tab OD Tropicamide eyedrops 1gtt BID CaC03 + Vit D 1 tab OD

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Course in the wards

On admission: CBC with Platelet, PPD, Chest X-ray, and

ESR were requested. Tropicamide eyedrops OU was also

started. On the 2nd hospital day

Referred to Rheumatology Plans for induction of high-dose steroids

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Course in the Wards

On the 3rd Hospital day: PPD test was started

On the 4th Hospital day Methylprednisolone 1g/kg to run for 1

hour for 3 days.

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Without Correction 20/50, J12 20/200, J12

Course in the wards

On the 5th hospital day, visual acuity of the patient improved:

(-) Hyperemia of the Conjunctiva (-) PPD test Patient was given 2nd dosage of

Methylprednisolone 1mg/kg/IV to run for 1 hour

CaC03 + Vit D 1 tab OD was started


Without Correction

20/40 20/70

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Course in the wards

On the 6th hospital day

Patient received last dose of Methylprednisolone 1mg/kg/IV to run for 1 hour.


Without Correction

20/40 20/40

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Course in the wards

7th hospital day Prednisone 20mg/tab 1 tab OD was

started


Without Correction 20/40 20/40

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Course in the wards

8th hospital day

Indirect Fundoscopy: Decrease in macular edema, decrease in vitreous cell and optic nerve hyperemia

Patient was discharged


Without Correction 20/40 20/40

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VOGT-KOYANAGI-HARADA DISEASE

DISCUSSION

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Vogt-Koyanagi-Harada disease Inflammatory condition of autoimmune

nature in which cytotoxic T cell target melanocytes (eyes, inner ears, skin)

Described by Persian Physician (Ali-ibn-Isa 940-1010 AD) –Poliosis + eye inflammation

1932- Combined disorders described by Vogt, Koyanagi and Harada manifestations were under the same disease process

New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-2036

Epidemiology

Predilection for more darkly pigmented races: Asians, Hispanics, American Indians

6.8-9.2% of all Uveitis referrals in Japan

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Vogt-Koyanagi-Harada diseaseClassification International Nomenclature

Committee Revised Diagnostic Criteria

Classification: Complete VKH disease Incomplete VKH disease Probable VKH disease


Applicability of the 2001 revised diagnostic criteria in Brazilian Vogt-Koyanagi-Harada disease patients Arq Bras Oftalmol. 2008;71(1):67-7039

Vogt-Koyanagi-Harada diseaseStages Prodromal stage Acute uveitic stage Convalescent stage Chronic recurrent stage

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Stages Prodromal Acute Uveitic Stage Convalsecent

stage Chronic Recurrent stage

Mimics viral Infection

Bilateral blurring of vision

Ocular pain secondary to Ciliary spasm

VitiligoAlopeciaPoliosis

43% in 1st three months52% in 1st six months

Fever

Neurological Symptoms

Multifocal Choroidtis Multifocal detachment of the sensory retina Exudative retinal detachment

Uveal depigmentationSunset glow

Foci of hyperpigmentation of RPE

GlaucomaCataractSubretinal Fibrosis

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Vogt-Koyonagi-Harada Disease

Pathophysiology

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Vogt-Koyanagi-Harada diseaseAutoimmunity Against Melanocytes

Clinical features of choroidal and skin depigmentation

Transmission electron microscopy (early stage): close contact between melanocytes and lymphocytes in the uvea

Histopathology (end stage): disappearance of choroidal melanocytes, and

Immunohistochemistry (end stage): T and B lymphocytes in the choroid


Vogt-Koyanagi-Harada diseaseAutoimmunity Against Melanocytes Role of CD4+ T cells

Cytotoxic leukocytes against melanoma cells in peripheral blood and CSF

Cytotoxic CD4 and CD8 T lymphocytes against human melanocytes are present in the peripheral blood.

Activated CD4+ T cells in depigmented skin and melanin-laden macrophages d in CSF


Vogt-Koyanagi-Harada diseaseAutoimmunity Against Melanocytes

Immunogenetics HLA-DR4/DR53 secondary association with HLA-DR1

involving a shared sequence linked to susceptibility to rheumatoid arthritis.

HLA-DRB1*0405


Pathophysiology

46

Clinical findings in acute phase of VKHFigure 1 - A & B: Fundus pictures of both eyes show disc hyperemia, white-yellowish choroidal lesions,

and localized exudative retinal detachments; C & D: Fluorescein angiographies of both eyes show pin-point hyperfluorescence and dye pooling corresponding to areas of retinal detachments; E & F: Indocyanine green angiographies show areas of diffuse hyperfluorescence, dark spot, and “hot-spots”


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Clinical findings in chronic phase of VKHFigure 2 – A & B: Fundus pictures of both eyes show diffuse retinal depigmentation and peripapillary fibrosis;

C & D: Fluorescein angiographies of both eyes show diffuse window retinal pigment epithelium defects; E & F: Indocyanine green angiographies

show dark spots and diffuse late hyperfluorescence suggestive of disease activity


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Treatment- CorticosteroidsFor most patients with bilateral serous detachments and

severe visual loss, begin therapy with systemic prednisone

Severe Cases use intravenous methylprednisolone (up to 1 g/d) for

several days before beginning oral prednisone (1 mg/kg/d)

Corticosteroids anti-inflammatory properties and modify the body's

immune response to diverse stimuli the length of treatment and subsequent taper must be

individualized for each patient

Treatment- Systemic Corticosteroids

Prednisone• Decrease inflammation

– reversing increased capillary permeability and suppressing PMN activity

• DOSE– 1-1.5 mg/kg/d PO qd initially– Severe cases with profound loss of vision and

bilateral serous detachments may require up to 2 mg/kg/d

– length of treatment and tapering individualized for each patient • not be less than 3 mo to avoid recurrence

• CI: hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Treatment- Topical Corticosteroids

Prednisone Acetate

• For the treatment of associated anterior uveitis

• Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability

DOSE:

• Instill1 gtt into conjunctival sac

• dosing frequency is based upon severity of inflammation – Severe inflammation may require dosing every hour– moderate-to-mild anterior uveitis, dosing at 4-6 times daily may be

sufficient– taper over an appropriate period to avoid rebound inflammation

Precaution: hypertension, cataract formation with long-term use, decrease frequency to avoid adrenal insufficiency

CI: Documented hypersensitivity; viral, fungal, or tubercular infections; cataract and glaucoma

Treatment- CycloplegicsTropicamide parasympatholytic that produces short acting mydriasis and

cycloplegia Instillation of a long-acting cycloplegic agent can relax any ciliary

muscle spasm that can cause a deep aching pain and photophobia used to treat anterior uveitis, decreasing risk of posterior

synechiae and decreasing inflammation in the anterior chamber of the eye

Side Effects transient stinging and a slight and transient rise in intraocular

pressure cause redness or conjunctivitis (inflammation) and also blurs vision

for a short while after instillation

Tropicamide is preferred over Atropine Atropine has a longer half-life, causing prolonged dilation and blurry vision for

up to a week

Treatment- Homatropine

Homatropine• Blocks responses of sphincter muscle of iris and

muscle of ciliary body to cholinergic stimulation, inducing mydriasis in 10-30 min and cycloplegia in 30-90 min

• last up to 48 h• Individuals with heavily pigmented irides may

require larger dosesDOSE: 1-2 gtt of 2% or 1 gtt of 5% solution up to qid

to induce cycloplegia and relieve ciliary spasmCI: Documented hypersensitivity; narrow-angle

glaucomaPrecaution: elderly persons w/ increased intraocular

pressure; toxic anticholinergic systemic adverse effects can occur but are rare when used sparingly

Treatment- ImmunosuppressivesFor those patients who fail to respond to high-

dose systemic corticosteroids or develop intolerable adverse effects, immunodulatory therapy

Cyclosporine Mycophenolate mofetil Azathioprine Tacrolimus Cyclophosphamide

interesting case

Documents

eye examination eom

history of present illness

rtl anisocoric

physical examination

rtl iris

discharge thorax

nasoaural discharge

social history diet