Poster Session: Session 2: Sjogren's syndrome

Introduction:

Sjogren Syndrome is frequent autoimmune disorder (prevalence 0,5% - 2%) characterized by lymphocyte infiltration of the salivary and lacrimal glands lead to dry eyes and mouth. Cutaneus skin manifestations in Sjogren Syndrome are often underestimated, whilst 50% of Sjogren syndrome have cutaneus finding. This condition made patients with Sjogren are wrongly diagnose nor had appropriate treatment earlier.

Case Presentation:

A 44 years old woman comes to our hospital with redness in her cheek since three years ago. She also felt pain in her skin and getting worse every time she got exposure from the sun. Patient also complained for headache and myalgia. From her physical examination we found malar rash and dry skin. The laboratory test showed positive Anti Nuclear Antibody, dsDNA was 13,8 , SS A +++, and Anti RO S2 ++. We also checked for her schirmer test and the result was 5 mm for right eye and 10 mm for left eye. Patients was treated with Hidroxychloroquine , Methylprednisolone 0,5 mg/Kg body weight, Vit D3, and Folic Acid. After one month therapy, patient showed improvement in her skin manifestation and she no longer feel pain in her skin.

Conclusion:

We treated a 44 years old women with cutaneus manifestation as a symptom of Sjogren syndrome. Patients was showed improvement while treated with Hidroxychloroquine , Methylprednisolone 0,5 mg/Kg body weight, Vit D3, and Folic Acid.
P2-018 PRIMARY SJOGREN’S SYNDROME PRESENTING WITH PERICARDIAL AND PLEURAL EFFUSION
V. Selvan¹, S. Xu<sup>1


1</sup>Medicine, Khoo Teck Puat Hospital, Singapore, Singapore

Introduction

SjogrenÕs syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. However, they are rarely accompanied

by serositis such as pleuritis or pericarditis. We present a case report of a primary SjogrenÕs syndrome presenting with pericardial and pleural effusion.

Case Report

The patient is a 50 year old Chinese female who was previously well. She presented with complaints of pleuritic chest pain, cough, fever and shortness of breath for 7 days. Physical examination revealed decreased breaths sounds in left lung base. She had a papulo-squamous lesion on her arm and leg. Chest xray showed bilateral pleural effusions and & cardiomegaly. ECG showed small voltages with non-specific T wave changes. CT scan of thorax and abdomen confirmed the presence of pericardial & pleural effusions. In addition there were mediastinal, axillary & supraclavicular lymphadenopathy.

Blood investigations showed anemia of 9.8gm/dL, ESR 59 mm/1^st hr, CRP 66 mg/L. Pleural fluid analysis showed exudative effusion which was negative for malignancy and infection. T-spot was negative. On direct questioning, the patient admitted to a long history of dry eyes and dry mouth. Opthalmological review confirmed presence of keratoconjunctivitis sicca. Tests for ANA (+), Anti Ro (+), Anti La (+), ds DNA (-), Rheumatoid Factor (+). Tests for other ENA (-). She declined labial gland biopsy.

Primary SjogrenÕs syndrome was diagnosed based on the sicca symptoms, positive Ro and La but negative dsDNA.The patient was treated with steroids and Hydroxychloroquine with improvement.

SjogrenÕs Syndrome should be considered as one of the connective tissue diseases potentially presenting with pleural and pericardial effusion. This has been reported by other authors.
P2-019 THE IMPROVEMENT OF THE SURGICAL DENTAL CARE FOR PATIENTS WITH SJOEGREN'S DIS EASE
E. Selifanova¹, E. Selifanova<sup>1


1</sup>Stomatology, Mental Health Research Center of RAMS, Moscow, Russia

Abstract:

Background and aims:

Background: Cytokines are the important mediators of immunity and disequilibrium in cytokine network may paramount for autoimmune disease susceptibility and severity. Vitiligo is an acquired depigmenting disorder resulting in the destruction of melanocytes in the epidermis affecting 1-4% worldwide with no prediction for sex or race. The incidence of vitiligo is found to be 0.5 to 2.5% in India. The cause of vitiligo remains obscure, although the autoimmune hypothesis has been now recognized as the most influential pathogenic mechanism. The present study was conducted to determine the equilibrium between two important cytokines IFN-γ and IL-10 in nondermatomal vitiligo and healthy individuals from South India.

Aim: To estimate the serum levels of IFN-γ and IL-10 in nondermatomal vitiligo patients and assess the ratio of these proinflammatory and anti-inflammatory cytokines with respect to disease duration.

Methods: Estimation of IFN-γ and IL-10 levels in human serum from each of 60 nondermatomal vitiligo patients and healthy controls using ELISA. Data analysis was performed using One-way ANOVA and t-test.

Results: Vitiligo patients showed increased levels of IFN- γ (11.7±5.2 vs 9.9±4.1 pg/ml) and decreased levels of IL-10 (9.4±5 vs 11.5±5.0 pg/ml) compared to controls. Ratio of IFN-γ to IL-10 was also significantly different in patients (1.5±0.7 pg/ml) and controls (1.0±0.6 pg/ml), indicating association of proinflammation with nondermatomal vitiligo. Further, significant correlation (p<0.05) was observed with disease duration.

Conclusion: Enhanced production of IFN-γ in patients may play a vital role in disease development suggesting Th1 mediated immune response in the pathogenesis of vitiligo.

Background and aims: Psoriasis patients are at increased risk of cardiovascular diseases (CVD), including atherosclerosis. Carotid intima-media thickness (CIMT) has been recognized as a diagnostic tool to identify premature atherosclerosis. Studies have shown that apolipoprotein measurement can help in prediction of CVD, however association between apolipoprotein and CIMT need to be studied. The aim of this study was to measure CIMT and estimate its relationship with apoB/apoA-I ratio in patients of psoriasis.

Methods: Serum lipid profile and apolipoprotein (apoA-I and apoB) were measured, in all hundred psoriasis patients and age-sex matched healthy controls recruited for the study. Intima-media thickness and carotid plaques were simultaneously measured by carotid sonography.

Results: The apoB/apoA-I ratio ( P < 0.001) and serum apoB ( P < 0.05) significantly correlated with raised CIMT in patients. Conventional lipid profile and apoA-I levels were not correlated to IM thickness. By regression analysis we found that apoB/apoA-I ratio was associated with CIMT.

Conclusions: The study shows that apoB/apoA-I ratio is associated with raised intima-media thickness and can be considered as a predictor of sub-clinical atherosclerosis in patients of psoriasis.
P2-024 NEVIRAPINE INDUCED STEVENS JOHNSON SYNDROME
D. Madi¹, C. Mahabala¹, M.R. Pavan<sup>1


1</sup>Medicine, Manipal university, mangalore, India

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare immune mediated cutaneous adverse reactions. The basic difference between SJS and TEN is the percentage of body surface area (BSA) involved: <10% in SJS; >30% in TEN; 10 to 30% in SJS-TEN overlap. Drugs and infections commonly cause SJS. Nevirapine a non-nucleoside reverse transcriptase inhibitor (NNRTI), is widely prescribed in India as a part of the combination therapy to treat human immunodeficiency virus (HIV) infection. Nevirapine which is used to treat HIV can also cause SJS. Patients infected with human immunodeficiency virus are at increased risk of developing mucocutaneous drug reactions. We report a case of Nevirapine induced SJS.

BACKGROUND AND AIMS

Autoimmune blistering diseases ( AIBD ) in children are uncommon and are due to circulating antibodies to either epidermal adhesion protein desmoglein or basement membrane zone components causing epidermal and subepidermal blisters respectively.Amongst AIBD,most common is linear IgA disease (LAD ).Pemphigus vulgaris(PV) pemphigus foliaceus( PF ),dermatitis herpetiformis ( DH )and bullous pemphigoid ( BP )rarely occur.The aim was to study incidence and clinical profile of AIBD in children over five years period from 2008 to 2012.

METHODS:

RESULTS:

CONCLUSION:

Direct immunofluorescence is the gold standard in the diagnosis of AIBD.Presence of oral lesions may be a marker of childhood BP.
P2-026 TREATMENT OF ALOPECIA AREATA UNIVERSALIS WITH DIALYZABLE LEUKOCYTE EXTRACTS, A REPORT OF 2 CASES.
T.A. Homberg¹, M.C. Andaluz¹, O. Pineda¹, E. Cervantes¹, M.A. Alonso¹, M.C. Jimenez², S.M. Pérez-Tapia<sup>1


1</sup>Immunology, Instituto Politécnico Nacional, Mexico City, Mexico
²Research Unit, I.A.P. Fundación Conde de Valenciana, Mexico City, Mexico

Alopecia areata is a T-lymphocyte mediated autoimmune disease of the hair follicle. Alopecia universalis is a severe form that affects hair in the entire body. Initial presentation is often related to physical or emotional stress. No specific treatment has proved successful on the long term. Although alopecia areata often presents spontaneous remission over a few months, persistent cases cause patients to seek experimental and alternative treatment. We present two cases of persistent alopecia areata universalis treated with dialyzable leucocyte extracts (DLE). Case 1 is a 4 year old girl with alopecia universalis since age 2, not responding to previous topical treatment or corticosteroids. After 3 weeks of treatment with DLE there is growth of eyebrows, eyelashes and scalp hair, continuing for 5 more months. Case 2 is a 38 year old female with alopecia universalis since age 34, with concomitant autoimmune hypothyroidism, not responding to different treatments including immunosupressants. She presents 70% improvement after 4 months of treatment with DLE.