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Interesting case

Chiombon. Chua. Corpuz. Cua. David. De Vera. Detera. Diaz. Din. Dizon. Eugenio. Evangelista. Interesting case. General Data. CT 29/Female DOA: 03 August 2010. Chief Complaint. Blurring of Vision. History of Present Illness. History of Present Illness. History of Present Illness.

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Interesting case

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

  2. General Data • CT • 29/Female • DOA: 03 August 2010

  3. Chief Complaint • Blurring of Vision

  4. History of Present Illness

  5. History of Present Illness

  6. History of Present Illness

  7. History of Present Illness

  8. Past Medical History • Allergies: none • No previous illnesses, hospitalizations and blood transfusions • TB exposure: (-) • Injuries: Burn 2nd and 3rd degree burns (1992) • Medications: none

  9. 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

  10. Family History • Diabetes Mellitus- Grandmother • Glaucoma- (-) • Hypertension – (-) • Cancer – (-)

  11. 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

  12. 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

  13. 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

  14. 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

  15. Eye Examination Amsler Grid (+) Scotoma on OU (+) Metamorphopsia

  16. 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

  17. 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

  18. 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

  19. Initial Assessment • Incomplete Vogt-Koyanagi-Harada Disease, Acute uveitic stage • r/o TB Uveitis

  20. 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).

  21. DIFFERENTIAL DIAGNOSIS

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

  23. DIAGNOSTICS

  24. Laboratories • CBC • Chest X-ray • AFB smear • PPD

  25. CBC

  26. Chest X-ray • Lung fields are clear • The heart is not enlarged • Both hemidiaphragm and costophrenic sulci are intact • Impression: No significant chest findings

  27. AFB Stain • No Acid Fast Bacilli seen in 300 oil immersion fields on both routine and concentration methods

  28. 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

  29. 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

  30. 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.

  31. 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

  32. Course in the wards • On the 6th hospital day • Patient received last dose of Methylprednisolone 1mg/kg/IV to run for 1 hour.

  33. Course in the wards • 7th hospital day • Prednisone 20mg/tab 1 tab OD was started

  34. Course in the wards • 8th hospital day • Indirect Fundoscopy: Decrease in macular edema, decrease in vitreous cell and optic nerve hyperemia • Patient was discharged

  35. DISCUSSION VOGT-KOYANAGI-HARADA DISEASE

  36. 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):413-20

  37. Epidemiology • Predilection for more darkly pigmented races: Asians, Hispanics, American Indians • 6.8-9.2% of all Uveitis referrals in Japan

  38. Vogt-Koyanagi-Harada diseaseClassification • International Nomenclature Committee Revised Diagnostic Criteria • Classification: • Complete VKH disease • Incomplete VKH disease • Probable VKH disease New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  39. Applicability of the 2001 revised diagnostic criteria in Brazilian Vogt-Koyanagi-Harada disease patients Arq Bras Oftalmol. 2008;71(1):67-70

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

  41. Stages

  42. Pathophysiology Vogt-Koyonagi-Harada Disease

  43. 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 New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  44. 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 New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  45. 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 New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  46. Pathophysiology

  47. Clinical findings in acute phase of VKH Figure 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” New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  48. Clinical findings in chronic phase of VKH Figure 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 New insights into Vogt-Koyanagi-Harada disease. Arq Bras Oftalmol. 2009;72(3):413-20

  49. Treatment- Corticosteroids For 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

  50. 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

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