S16 Indian Journal of Rheumatology 2010 November; Vol. 5, No.
3 (Suppl)
Conclusion: 1) Endothelial dysfunction is present in newly diagnosed SLE patients even in
the absence of traditional cardiovascular risk factors. 2) FMD using vascular ultrasonogra-
phy on brachial artery represents a non-invasive, reproducible and cost effective tool for
the assessment of endothelial dysfunction. 3) Flow mediated dilatation of brachial artery
may be an early physiological feature of endothelial dysfunction and may precede the
anatomical increase in carotid intimal thickness. 4) FMD of brachial artery has significant
negative correlation with disease activity and antidsDNA antibody.
P9
Male Lupus: A retrospective data review
Vishad Viswanath, Yogesh Preet Singh, Able Lawrence, Vikas Agarwal,
Ramnath Misra, Amita Aggarwal
Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences,
Lucknow
Introduction: SLE is a disease with female predominance; only 8–22% of affected are males.
Male SLE presents with distinct clinical and epidemiological features. Studies from the west-
ern countries have reported a higher prevalence of nephritis and thrombocytopenia.
However data from India is sparse. This study attempts to characterize the clincoepidemology
of male Lupus in Indian patients.
Methods: This is a retrospective review of male patients with SLE (1990 ACR criteria) seen
between 1992–2010 in our centre, and of their follow up during subsequent years. Data was
collected from the clinic files and electronic records.
Results: Of the total of 650 SLE patients under follow up, 45 were males (1:14). Mean age at
disease onset was 21.8 years (6–56 yrs). 1/3rd of patients had Juvenile Onset Lupus. Average
delay from onset of first clinical feature to diagnosis was of 1.1 year (0–11.2 yrs).
Most patients had active disease at onset with median SLEDAI 14 (0–28). Presenting fea-
tures included fever (84%), joint symptoms (71%), hematological involvement (71%), renal
disease (56%) and NPSLE (36%).
ANA was positive in 40 (89%) patients. DsDNA was elevated in 26 (72%); 31 patients had
low complements at onset (70%). Among the patients in whom it was tested, 9/25 and 5/22
patients had positive IgG and IgM anti phospholipid antibodies respectively. A diagnosis of
APS syndrome was made in 4 of them.
Majority of the patients received Hydroxy choloroquine (43) and steroids (41). 24 of
patients received Cyclophosphamide; all except one received it for lupus nephritis. 8 received
Mycophenlate.
Mean duration of follow up was 4 years. 16 major and 4 minor flares occurred during fol-
low up. All major flares involved kidneys, 2 had CNS and 1 had Serositis alongside. Mean dura-
tion to flare was 52.8 months (range 11–108 months).
Short term complications included 19 major infections in 12 patients, GI bleed in 2, severe
esophagitis, toxic megacolon, CRVO and recurrent venous thrombosis in 1 patient each.
During follow up, 10 patients developed SLICC score more than 0. Long term morbidities
included CKD in 3, CAD in 1, valvular heart disease in 2 and avascular necrosis in 1.4 patients
died; 1 due to CAD with stent thromboses and 3 of infections.
Conclusions: Male SLE as compared to females is still rarer in Indian population. SLE in
males has an early onset and present with highly active disease. It is associated with signifi-
cant mortality and morbidity.
P10
Clinical and immunological profile of patients of SLE presenting to
a rheumatology OPD in the western part of India
C Sapan Pandya, Rakesh Solanki, Jignesh Patel, Rheumatic disease clinic
Vedanta Institute of medical sciences, Ahmedabad
Background: There is scarce data on the profile of SLE patients in the subcontinent. The ear-
lier reports are also low in numbers. We analysed from our datasheet the clinical and
immunological profile of SLE patients from in and around the state of Gujarat in the
Western part of India reporting to our OPD.
Aims & Objectives: To study the clinical and immunological profile of SLE patients present-
ing to our OPD (state of Gujarat).
Material & Methods: Data was retrieved from records for a 6 and half year period from Jan
2004 to July 2010. Clinical and immunological features were analysed using descriptive statis-
tics. The data was compared with that from the Western and other Indian series.
Results: Between Jan 2004 and July 2010, 394 patients of SLE presented to the OPD. Mean age
of these patients was 29.33 + 10.71 with a median of 26.5 and range from 6 years to 70 years.
There were 359 females and 35 males (ratio 10.2:1). The mean duration of illness was
2.15 + 16.2 years. On presentation, 252 (63.9%) had fever, 276 (70%) had arthralgias/arthritis,
86 (21.8%) myalgias, 306 (77.6%) complained of fatigue, 64 (16.2%) had significant weight loss
and 223 (56.5%) had pallor. Of mucocutaneous symptoms, 212 patients (53.8%) had oral
ulcers, 248 (62.9%) had significant hair fall and 263 (66.7%) had some form of rash, 29 had
discoid lupus while 234 (59.3%) complained of photosensitivity. Only 50 patients (12.6%)
complained of Raynaud’s phenomenon. 13 patients had serositis (pericardial/pleural effusion),
total of 31 patients had GI symptoms, 54 patients (13.7%) had pedal edema while 7 patients
presented with deep vein thrombosis. 30 patients had autoimmune hemolytic anemia. 41
patients (10.4%) presented with some form of neuropsychiatric complaints while 12 had
peripheral neuropathy. On examination, 209 (53%) had malar rash, 2 had gangrene, 26 had
lymphadenopathy, 10 splenomegaly and 4 had hepatomegaly. 6 patients had dry eyes and 5
dry mouth while 29 had significant proximal muscle weakness.
Poster presentations
The mean hemoglobin was 9.78gm% with 48 patients having Hb < 8 gm%, 76 (19.2%) had
leucopenia, 40 (10.6%) had thrombocytopenia. The mean ESR was 74.3. Of available data on
the ANA pattern, 113 (28.6%) had a speckled pattern, 103 (26%) homogenous, 3 patients had
a rim pattern, 2 nucleolar, 3 atypical pattern, 2 cytoplasmic and in 47 patients, it was
reported positive by ELISA. 82% had anti ds DNA positivity whenever done, 31 had anti Sm,
27 anti RNP, 39 Anti Ro and 26 Anti La (not done in all). 3 patients were IgM ACLA positive
and 4 IgG ACLA positive. More than 70% had hypocomplementemia when complements were
checked. 29.1% had active urinary sediments. Of kidney biopsies done (82), 28 (34%) had
class 4 nephritis, 22 (26.8%) had class 3, 13 patients had class 2 and 2 class 6 nephritis.
6 patients had changes of class 4 and 5 while 2 had class 2 + class 5 nephritis. 14 patients
had pulmonary hypertension by 2D echocardiography.
182/216 patients at first follow up (after 2 to 3 months of baseline visit) received hydroxy-
chloroquine (84.2%). 24 needed cyclophosphamide boluses, 18 were on azathioprine and
11 on mycophenolate mofetil.
Follow up data showed 46% dropping from follow up after the 1st visit itself. Only 216/394
(54%) presented at 2 to 3 months of baseline visit. By last follow up at 6 years data was avail-
able only on 33 patients (15.6%) which reveal a high dropout rate. There were 7 confirmed
deaths (1 due to hemorrhagic infarct in the brain due to disease itself), 1 of coronary artery dis-
ease and the rest due to infections. One patient is on dialysis.
When compared with the series reported elsewhere (from the West), significantly more
patients presented with oral ulcers and Raynauds phenomenon was significantly lesser in
our patients. When compared to the other Indian series, significantly more patients pre-
sented with fever to our OPD.
Conclusions: Most of the clinical and immunological features of our patients matched with
those reported elsewhere from India and the West. Raynaud’s phenomenon was signifi-
cantly lesser in our patients than the West. Most of the deaths were due to infections which
still remain a major concern in these patients. Follow up has been very poor at least at our
center due to various reasons. More data from tertiary level clinics is needed to confirm
these findings so that follow up with rheumatologists can be improved.
P11
Role of HLA DR-DQ in susceptibility to systemic lupus erythematosus:
report from Tamil Nadu, South India
VS Negi1, M Christina Mary1, R Tamouza2
1
Medicine, Division of Clinical Immunology, JIPMER, Puducherry, India, 2UMRS940,
Laboratory of Immunology and Histocompatibility, Hospital Saint-Louis, Paris, France
Background: Polymorphisms within the human leukocyte antigen (HLA) region are impor-
tant in determining the susceptibility to SLE and its clinical heterogeneity. HLA DRB1*02,
HLA DRB1*15 (allelic variant of DRB*02) and DRB1*03 are reported to be associated with SLE
in Blacks, Asians and Caucasians respectively. HLA DR*04 has been reported to be associated
with SLE in north and DR*03 in western Indian population. Present study was conducted to
determine the association of Class II antigens with SLE in South Indian Tamil ethnic population.
Method: A total of 228 healthy controls and 131 SLE patients (125 Females and 6 males) from
Tamil Nadu, South India were typed for polymorphism in the HLA DRB1 and DQB1 genes by PCR
SSP method. Haplotypes were constructed using PHASE software for haplotype reconstruction.
Result: In our study, none of the HLA DR and DQ alleles were found to be associated with SLE.
Haplotypes of the three susceptibility gene loci (HLA, MICA and TNFα-857-308-238) for
patients with SLE were constructed. The haplotype DR*15-DQ-*06-TNFαGGC-MICAval con-
ferred susceptibility to SLE while haplotypes, DR*07-DQ*03-TNFαGGT-MICA met and DR*14-
DQ*05-TNFαGGC-MICAmet conferred protection. The effect of HLA DR was dominant over
the effect of other genes.
Conclusion: HLA DR-DQ was not associated with susceptibility to SLE although the haplotype
containing DR*15 appeared to confer susceptibility to SLE.
P12
Cytokine production, serum levels and disease activity in paediatric systemic
lupus erythematosus in North India cohort
Anita Rana1, Ranjana W Minz1, Ritu Aggarwal1, Neelam Pasrija1, S Anand1, Surjit Singh2
1
Department of Immunopathology, 2Department of Pediatrics, Advanced Pediatric Centre,
Allergy and Immunology Unit, Post Graduate Institute of Medical Education and Research
(PGIMER), Chandigarh
Background: Systemic lupus erythematosus (SLE) is a multi-organ complex autoimmune dis-
ease characterized by abnormal production of autoantibodies and various clinical manifes-
tations. T cell abnormalities, B cell hyperactivity and abnormal cytokine production are
pathogenic importance in SLE. Imbalance in cytokine production between T helper type 1
(Th1) and Th2 T cells (predominance of Th2 cytokine) and Th17 type in SLE has been
reported earlier. The increased knowledge of the pathogenetic mechanisms will open the
possibility for more specific immunological treatments, targeting, for example, cytokines in
SLE. With this background present study aims to analyze role of Th1 and Th2 cytokines pro-
file in children with SLE and their correlation disease activity.
Objective: 1) To analyze Th1, Th2, IL-17 and IL-23 cytokines profile by ELISA and Flow
cytometry in paediatric SLE (pSLE) patients 2) To study their correlation with disease activity
and organ manifestations in pSLE.
Methods: 40 paediatric SLE patients (age 5–16 yrs) and 20 healthy age matched controls
were investigated. All patients fulfilled at least four American College of Rheumatology
(ACR) criteria. Disease activity was evaluated according to SLE Disease Index (SLEDAI).