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ABBREVIATIONS
ACR : The American College of Rheumatology ALT : alanine aminotransferase ANA : antinuclear antibodies Anti ds-DNA: anti double stranded-deoxyribonucleic acid antibodies Anti-RNP : anti- ribonucleoprotein APA : anti-phospholipid antibodies AST : aspartate aminotransferase BW : body weight DLE : discoid lupus erythematosus GP : general practitioner HLA : human leukocyte antigen IU/L : international units per litre KCS : keratoconjunctivitis sicca LE : lupus erythematosus mEq : mili equivalent MSCT : multi slice computed tomogtraphy PUK : peripheral ulcerative keratitis SLE : systemic lupus erythematosus USG : ultrasonography
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INTRODUCTION
Lupus erythematosus is a basic term of diseases combined:
clinical manifestations characteristic pattern of polyclonal B cell autoimmunity
INTRODUCTION
INTRODUCTION
INTRODUCTION
INTRODUCTION
INTRODUCTION
Sivaraj RR, at al (2007); Rosenbaum J, et al (2007); Fox R, et al (2002); Ushiyama O, et al (2000); Akib AA, et al (2010)
INTRODUCTION
The purpose of this paper present a rare case of lupus retinopathy in a child with SLE
CASE REPORT
A, female, 8 y.o, 19 kgs
came to Pediatric Outpatient Clinic on May 25th, 2011 chief complaint of fever referred by a GP in Tuban diagnosed as suspicious of SLE
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CASE REPORT
History:
fever 5 months before accompanied w/ non-productive cough, headache, fatigue, rashes on back & face, hospitalized w/ dx: typhoid fever, for 7 days discharged A few days later fever again + fatigue, malaise, anemic, loss of appetite, hair fall, & joints pain
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CASE REPORT
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CASE REPORT
blurred vision since 3 mo. ago
getting worse not accompanied with pain and trauma no ocular pain even with eye movement dryness and redness of the eye was not found.
headache (+), chest pain, abdominal pain, nausea and vomiting, cough, dispnea, edema, seizure, pareses, behavioral disturbance (-) defecation and urination: normal
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CASE REPORT
px was the only child of family mother diabetic (-), hypertension (-), nor taking any drugs and traditional herbs during pregnancy delivered spontaneously per vaginam, aterm, by midwife, BW 2500 g immunizations up to date. growth and development normal
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CASE REPORT
Physical examination
weak, alert girl BW: 19 kgs, BH: 119 cms BP: 100/60 mmHg, pulse: 126 tpm, RR: 20 tpm, tax : 38.1oC head & neck:
anemia (+), jaundice (-), cyanotic (-), dispnea (-), lymph node enlargement (-) malar rash (+), discoid rash (+), visual acuity
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CASE REPORT
Figure 1. The photographs of the patient show: (a) malar rash ;(b) oral ulcer; (c) and discoid rash
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CASE REPORT
Figure 2. Rashes on the patients upper and lower extremities and ears; discoid lesions on back
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CASE REPORT
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CASE REPORT
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CASE REPORT
CASE REPORT
CASE REPORT
CASE REPORT
Initial therapy:
methylprednisolone pulse 500 mg in 100 mL i.v ampicillin-sulbactam 150 mg q.i.d. i.v azathioprine 30 mg b.i.d p.o acetaminophen as needed sun-block cream during the day, and hidrocortison 1% cream during bedtime diet of 1500 kcal/day
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CASE REPORT
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CASE REPORT
On 2nd day of admission, The temporary conclusion pale optic discs DD atrophic optic nerve Suggestion further examinations at ophthalmology outpatient clinic
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CASE REPORT
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CASE REPORT
The abdominal USG: non-specific hepatomegaly no specific treatment was given due to these results
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CASE REPORT
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CASE REPORT
Conclusions: bilateral optic atrophy and bilateral retinal vasculitis due to systemic lupus erythematosus
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CASE REPORT
CASE REPORT
CASE REPORT
CASE REPORT
DISCUSSION
SLE
interaction : genetic factors, acquired factors, and environmental factors
Blodi BA (2002); Klein-Gitelman, et al (2007); Tutuncu ZN, et al (2007), Akib AA, et al (2010) 36
DISCUSSION
some target organs musculoskeletal: arthritis skin: malar rash renal: glomerulonephritis cardiovascular: pericarditis neurologic: seizure pulmonary: pleuritic pain hematologic: anemia digestive system: oral ulcer, dysphagia, anorexia, ascites, perotinitis eye: keratitis, retinopathy, optic neuropathy
Klein-Gitelman, et al (2007); Tutuncu ZN, et al (2007); Fox R, et al (2002) 37
DISCUSSION The diagnosis of SLE is based on clinical and laboratory criterions proposed by the American College of Rheumatology (ACR). In our case, 7 of 11 creterions were fullfilled
the diagnose of systemic lupus erythematosus could be established
DISCUSSION
Table 1. Our case compared with ACR criterions
Criterionn
1. Malar Rash
2. Discoid rash
Our case
Criterionn
7. Renal Disorder 8. Neurologic Disorder 9. Hematologic Disorder 10. Immunologic Disorder 11. Positive Antinuclear Antibody
Our case
-
+
+
3. Photosensitivity
4. Oral ulcers 5. Nonerosive Arthritis 6. Pleuritis or Pericarditis
+
+
-
+
+ +
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Posterior segments
Might be sight threatening
DISCUSSION
Table 2. Manifestations of SLE on anterior segments of eye (Peponis, 2006)
Anterior Segments
External ocular
Clinical manifestation
Keratoconjunctivitis sicca (KCS) Secondary Sjgrens syndrome
Adnexal involvement
Corneal involvement
Superficial punctate keratopathy Recurrent epithelial erosions Peripheral ulcerative keratitis (PUK) Interstitial stromal keratitis Corneal endothelitis (keratoendothelitis)
Conjunctival involvement
Scleral involvement
Uveal involvement
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DISCUSSION
Table 3. Manifestations of SLE on posterior segments of eye (Peponis, 2006)
Posterior Segments
Retinal involvement
Clinical manifestation
Cotton-wool spots Retinal edema, ischemia, hard exudates, hemorrhages Retinal vasculitis Central retinal artery occlusion, cilioretinal artery occlusion Retinal vein occlusion (central or branch) Proliferative retinopathy, retinal neovascularization Pseudoretinitis pigmentosa-like retinopathy
Choroidal involvement
Multifocal serous detachments of the retinal pigment epithelium (RPE) and neural retina Choroidal neovascularization Central serous chorioretinopathy (CSR) Ciliochoroidal effusions
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DISCUSSION
Table 3. Manifestations of SLE on anterior segments of eye (continued) Posterior Segments Clinical manifestation
Neuro-ophthalmological involvement Optic nerve involvement (papillitis, ischemic optic neuropathy, retrobulbar optic neuritis) Ocular motor nerve palsies Visual disturbances (amaurosis fugax, cortical blindness, visual field defects) Pupillary abnormalities (light-near dissociation, Horners syndrome) Gaze abnormalities (internuclear ophthalmoplegia, one-and-ahalf syndrome) Nystagmus, ocular flutter Pseudotumor cerebri MillerFisher syndrome
DISCUSSION
In our case, blurred vision (+) ocular pain (-) red of eyes (-) dryness of eyes (-) visual acuity about 1/60 on both eyes fundus examination: bilateral pallor optic discs optic atrophy, soft exudates cotton-wool spots, decreasing macular reflexes, and sheathing on retinal vascular retinal & neuro-opht implication was the prominent manifestation in this case
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DISCUSSION Painless and gradually loss of vision on both eyes ischemic optic neuropathy
may due to occlusion of the small vessels of the optic nerves demyelination or axonal necrosis.
DISCUSSION Choroidopathies should be considered in this patient The best examination to identify is fluorescein fundus angiography (FFA). In our case, it was not performed
due to financial problem lack of experience to do this procedure on a child in Dr. Soetomo Hospital
Sun exposure should be minimized and include use of a sunscreen Corticosteroids control symptoms and autoantibody production in lupus
started on 1-2 mg/kg/24hr of oral prednisone in divided daily doses severe patient methylprednisolone 25-30 mg/kg/24hr intravenously for three consecutive days followed by prednisone orally
Klein-Gitelman, et al (2007); Akib AA, et al (2010); Bartels CM, et al (2011) 47
DISCUSSION Azathioprine has been used as immunosuppressant and corticosteroid sparing agent Treatment of microangiopathic changes on retinopathy chosen in the context of the systemic disease.
DISCUSSION Higher daily dosages of up to 2 mg/kg/day of oral prednisone have been used in patients with retinal vasculitis. In some cases, especially of asymmetrical ocular involvement, systemic corticosteroid may be supplemented with regional corticosteroid injection (triamcinolone acetonide, 40 mg/mL, in sub-Tenon or peribulbar injections)
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DISCUSSION
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DISCUSSION
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DISCUSSION SLE is characterized by remission and exacerbation (flare) Exacerbation is development of any new signs and/or symptom that is in necessary for change of the treatment, caused by: UV exposure, infection, some drugs such as: aromatic-forming antibiotics (penicillin, sulfa, tetracycline), salt of aurum, phenothiazine, and anticonvulsants, pregnancy
Akib aa, et al (2010); Mosca M, et al (2006) 54
DISCUSSION Measurement of the disease activity is based on scoring system many disease activity indices SLE Disease Activity Index (SLEDAI), and revised versions as SLEDAI-2K and Safety of Estrogen in Lupus Erythematosus National Assessment (SELENA)-SLEDAI the most common adapted indices in more countries, includes Indonesia
Akib aa, et al (2010); Mosca M, et al (2006); American College of Rheumatology (2011) 55
SUMMARY
A rare case of lupus retinopathy and optic atrophy in a child with SLE has been presented. Chief complaints of fever, rash, and loss of vision. Based on the history, physical examination, laboratory data diagnosis of systemic lupus erythematosus was established.
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SUMMARY
By ophthalmologic examination the ocular seemed to be the most involved target organ on the patient The manifestations were retinal vasculitis and optic atrophy. The treatment of intravenous, followed by oral corticosteroid improved her general status and laboratory indices, but not for her sight or retinal impairment.
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SUMMARY
Activity of the disease or flare can be assessed by several scoring system. Visual prognosis is bad due to optic atrophy and severe retinal vasculitis, so need to be prepared for visual rehabilitation.
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THANK YOU
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Chest X-ray
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Echocardiography
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Abdominal ultrasonography
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Head MSCT
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Deng, Y. & Tsao, B. P. (2010) Genetic susceptibility to systemic lupus erythematosus in the genomic era Nat. Rev. Rheumatol. doi:10.1038/nrrheum.2010.176
SLEDAI
71
SLEDAI
72
SLEDAI
73
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76
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Polyclonal
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LE cell
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Complement system
80
Normal fundus
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Nutritional status