Pachymeningitis as the presenting manifestation of Systemic Lupus Erythematosus (SLE)

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Sixteen pregnancies in 11 patients out of 37 married patients were studied. Three maternal complications occurred: Preeclampsia(APA-ve),postpartum hemorrhage and maternal mortality due to disseminated tuberculosis at 8 months of gestation. Flares were seen in 7 patients(nephritis-3), of 7 patients,3 were APAþve. Out of 16 pregnancies,9 live births occurred:7 normal vaginal delivery and 2 planned LSCS;5 preterm(2 LowBirthWeight) and 4 fullterm. Seven pregnancies weren't successful:2 IUFD,3 first trimester spontaneous abortion,1 MTP and one maternal mortality. Mothers were treated with steroids,hydroxychloroquine,aspirin (if APAþve),azathioprine (if required for LN).

Successful Pregnancy(9)

Unsuccessful pregnancy(7)

Low SLEDAI at conception No.of patients with flare APA+ve

6 2(1 LN) 1

Anti Ro/La Average steroid dose(mg/day)

0 10

2 5(2 LN) 4(2 abortion, 1 IUFD) 1(IUFD) 30

Conclusions: Amenorrhea in young was due to high disease activity which reversed on treating irrespective of cyclophosphamide dose/regimen. In older patients treated with cyclophosphamide amenorrhea was irreversible. Successful pregnancy is possible if planned when disease is controlled. Antiphospholipid antibodies and flare(esp. nephritic) have negative impact on pregnancy outcome.

P83. Complement Receptor 1 (CR1) gene polymorphisms in Systemic Lupus Erythematosus (SLE) patients and their association with clinical expression of the disease Vandana Pradhana, Sangeeta Paulc, Vinod Umarea, Manisha Patwardhana, Milind Nadkarb, Anjali Rajadhyakshab, Alok Dhawanc, Kanjaksha Ghosha; aDepartment of Clinical and Experimental Immunology, National Institute of Immunohaematology, Indian Council of Medical Research, Mumbai, India; bDepartment of Medicine, King Edward Memorial Hospital, Mumbai, India; cInstitute of Life Sciences, Ahmadabad University, Gujarat, India Background: Complement Receptor 1 (CR1) is a monomeric singlepass type 1 membrane glycoprotein mediating the binding and transport of immune complexes (ICs) to phagocytes. A number of studies have reported the association of the CR1 polymorphisms with Systemic Lupus Erythematosus (SLE). The aim of this study was to explore whether the single nucleotide polymorphisms (SNPs) of CR1 gene in exon 22 (1208A/G) and exon 33 (1827C/G) are associated with susceptibility of SLE. Methods: SLE patients were clinically examined by ACR criteria and the disease activity was assessed by using SLEDAI after obtaining requisite ethical committee permission. Genomic DNA of 50 SLE patients and 50 age and sex matched normal healthy individuals were genotyped by PCR-RFLP method. Genotyping results were compared with phenotype expression of the disease and autoantibodies. Results: CR1 analysis showed the significant prevalence of variant genotype, 1208GG (OR: 4.947, 95% Confidence Interval [CI]: 1.30118.808, p: 0.018) and the allele, 1208G (OR: 2.241, 95% CI: 1.2484.004, p: 0.006) among patients compared to controls. Further analysis showed the combined occurrence of 1208GG and AG

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genotype was associated with the arthritis manifestation of SLE. CR1 1827C/G showed no significant association with SLE. Conclusion: Nevertheless, CR1 (1208A/G) polymorphism may constitute as a risk factor for the susceptibility of the disease. However, the study needs to be extended to investigate more number of patients.

P84. An old parameter in a new avatar!!!! e QT interval in SLE S. Sham, N. Thilagavathi, T.N. Tamilselvam, S. Rajeswari; Department of Rheumatology, Madras Medical College, Chennai, India Introduction: SLE needs periodic assessment of disease activity with various markers like complements, ds DNA which are quite costly in a country like ours. So the aim of this study was to find a surrogate marker for the same which would be readily available and cheaper. Aim: 1. To study any correlation between QT interval parameters (QTc interval & QT dispersion) on Electrocardiogram (ECG) and disease activity (SLEDAI) in patients with SLE. 2. To study QT interval parameters during episodes of flare. Methods: The study was done on 100 newly detected SLE patients and 100 age matched controls between January 2012 e December 2013. Results: In our study, 84% had high disease activity (SLEDAI> 10). 51 among cases & 6 among controls had QTc> 440 msec. QTd was prolonged among 6 cases and 6 controls. The mean QTc interval was 436.30 msec (S.D of 27.43) among cases and 397.24 msec (S.D of 31.85) among controls which was statistically significant (p<0.001; Levene's Test for Equality of Variances). The mean QTd among cases was 44.40msec (S.D of 20.61) and 39.2 (S.D of 17.7) among controls which was not statistically significant (p<0.057). Difference of QTc values during severe flare from baseline QTc values was statistically significant (r¼0.863; Pearson's correlation coefficient). Conclusions: This study emphasizes the increased prevalence of QTc prolongation in SLE patients with high disease activity. So, QTc interval may be used as a surrogate marker for assessing disease activity in SLE.

P85. Pachymeningitis as the presenting manifestation of Systemic Lupus Erythematosus (SLE) Hegde Arun, Shanmuganandan Krishnan, D.S. Bhakuni, Kumar Abhishek, Atal Amar Tej; Department of Rheumatology, Army Hospital Research and Referral, Delhi Cantt, India Introduction: Pachymeningitis is characterized by focal or diffuse thickening of the dura mater and is uncommonly associated with systemic connective tissue disorder. Herein we report the case of a young girl with pachymeningitis as the presenting clinical manifestation for her underlying SLE. Methods: A 12 yr old girl with no known previous illness presented with severe headache for two weeks. Baseline clinical evaluation was normal. There were no clinical signs of raised intracranial tension. Magnetic resonance imaging (MRI) of the brain done for persistent headache showed mild pachymeningeal thickening and contiguous post contrast enhancement over bilateral cerebral hemispheres suggestive of pachymeningitis. Cerebrospinal fluid (CSF) examination revealed high opening pressure, normal biochemistry, negative bacterial, fungal and mycobacterial cultures, negative PCR for viral etiology and negative cytology for malignant cells.

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Blood test revealed normal hematological and biochemical parameters. Acute phase reactants were elevated. Immunological evaluation revealed positive Antinuclear antibodies by Indirect Immunofluorescence method (homogeneous 4þ at 1: 80 titer), positive dsDNA and SmD1, on Line immunoassay and reduced complement levels. Although there were no other clinical manifestations, the immunological profile was highly suggestive of SLE. A diagnosis of pachymeningitis with evolving SLE was thus made and the patient was managed with pulse methylprednisolone followed by high dose prednisolone and hydroxychloroquine with which there was clinical resolution. Conclusion: Pachymeningitis as the presenting manifestation of SLE has rarely been reported in literature and to the best of our knowledge, none from this country.

P86. Mean platelet volume as a surrogate marker of disease activity in systemic lupus erythematosus M.M. Kavithaa, M. Madheshwarana, T.N. Tamilselvam.a, J. Euphrasia Lathab, S. Rajeswaria; aDepartments of Rheumatology and bImmunology, Madras Medical College, Chennai, India Aim: To assess the levels of mean platelet volume in patients with lupus and to study its correlation with disease activity, complement levels and erythrocyte sedimentation rate. It is aimed at studying its usefulness as a surrogate marker of disease activity. Methods: Patients with lupus, irrespective of age, sex and disease activity were included in the study. SLEDAI was used as an indicator of disease activity. Any change of SLEDAI >3, new organ involvement were included in active lupus(n¼ 50) and patients with no disease activity were included in the inactive group(n¼150). 100 age and sex matched healthy controls were included in the study. Mean platelet volume was measured using Sysmex XP -100 fully automated blood cell counter. Results: Mean platelet volume of the control group was 8.6þ0.98 fL, a value of 9.48fL was taken as cut off point. In our study, 43(out of 50) in the active lupus group(mean¼10.54fL) and 15(out of 150) in the inactive group had a mean platelet volume higher than 9.48fL. The mean platelet volume was increased in patients with active lupus, which was also statistically significant(P value¼0.0). Mean platelet volume positively correlated with SLEDAI(p value 0.03) and ESR(0.01) and negatively correlated with Complement levels(r¼0.34, p value<0.05). Conclusion: We conclude that mean platelet volume is a good indicator of disease activity in lupus and can be used as a surrogate marker in active lupus.

P87. ESR, uric acid and its correlation with disease activity in SLE e Is it a myth or reality!!!! S. Sham, M. Hema, T.N. Tamilselvam, S. Rajeswari; Department of Rheumatology, Madras Medical College, Chennai, India Introduction: SLE needs periodic assessment of disease activity with various markers like complements, ds DNA which are quite costly in a country like ours. So the aim of this study was to find a surrogate marker for the same which would be readily available and cheaper. Aim: (1). To study any correlation between ESR & disease activity (SLEDAI) in patients with SLE. (2). To study any correlation between Serum Uric acid & disease activity (SLEDAI) in patients with SLE. Methods: The study was done on 100 newly detected SLE patients, admitted as inpatients in Department of Rheumatology. ESR was

calculated with Westergren's method [Correction for anemia: Corrected Hb ¼ (ESR X Hematocrit/45)] & serum uric acid (determined by Trinder's method) with automated analyser. Duration of study: January 2012 e December 2013. Design of the study: Prospective analytical study. Results: In our study, 84% had high disease activity (SLEDAI>10). 4% of patients had ESR values less than 25mm/hr, 41% had between 26-50mm/hr, 41% had between 50e75 mm/hr and 14% had values between 75e100 mm/hr.The relationship between the SLEDAI and ESR was positive and statistically significant (‘r’¼ 0.329; Pearson's correlation; p ¼ 0.001). 42% had serum uric acid > 6 mg/dl and average serum uric acid was 6.13. The relationship between the SLEDAI and serum uric acid was positive and statistically significant (‘r’¼ 0.339; Pearson's correlation; p ¼ 0.001). Conclusions: This study emphasizes the positive correlation between ESR, serum uric acid and disease activity (SLEDAI) in SLE and hence may be used as surrogate markers.

P88. Systemic lupus Erythematosus and kikuchi fujimoto disease e A rare association A. Dua, Jain N., Duggal L.; Department of Rheumatology & Clinical Immunology, Sir Gangaram Hospital, New Delhi, India Introduction: Kikuchi Fujimoto's disease (KFD) is a rare, benign, self-limiting; immune-mediated, disease. It has been found to be associated with a number of infectious and autoimmune diseases, including its rare association with systemic lupus erythematosus (SLE). KFD may precede, follow or coincide with the diagnosis of SLE. Methods: Two female patients were admitted in our hospital with prolonged pyrexia and lymphadenopathy. They were found to have SLE as per SLICC criterion. They also fulfill ACR criterion for SLE. Both patients had lymphadenopathy, so to rule out other possible cause of fever with lymphadenopathy, lymph node biopsy was done which clinches us to the final diagnosis. Results: Lymph node biopsy showed the presence of necrotizing lymphadenitis with no granuloma with paucity of polymorph and absence of vacuities, suggestive of KFD. Hence the final diagnosis of SLE associated with KFD was made. Conclusions: We believe these cases support the recommendation that a possible association between KFD and SLE should be always considered.

P89. Dancing Lupus K. Chanakya, Thomas Mathew, Vineeta Shobha; Department of Rheumatology, St Jones Medical College, Bangalore, India Introduction: Central nervous system lupus is a serious but potentially treatable condition. Chorea is a relatively uncommon neurological manifestation of SLE. Here, we present 2 cases where chorea was the initial presentation antedating establishment of Lupus diagnosis. Case 1: 15 years old girl presented with involuntary rhythmic movements of all the extremities for 3 days suggestive of chorea. She also developed double hemiparesis while in hospital. Retrospectively, history of low grade fever for 2 months with fleeting large joint pains was revealed. Investigations showed pancytopenia. MRI Brain showed features suggestive of vasculitis. Workup for rheumatic fever turned to be negative; connective tissue disorder workup disclosed a strongly positive ANA, dsDNA and reduced complements. So, diagnosis of SLE was established and