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ABSTRACTS
IRACON 2016: Poster Presentations

October 2016, 11(5):20-108
  842 104 -
REVIEW ARTICLE
Emerging evidence-based therapies for systemic sclerosis
Jasmin Raja, Christopher P Denton
September 2016, 11(3):153-163
DOI:10.4103/0973-3698.187423  
Systemic sclerosis (SSc) (scleroderma) is an uncommon multisystem connective tissue disease with high unmet need and mortality. There has been an improvement in overall outcome and survival over the past three decades, but it still has the highest mortality among any of the autoimmune rheumatic diseases. Progress in its management has come through more organized assessment and treatment together with the emergence of therapies that can target specific complications of the disease such as renal crisis and pulmonary arterial hypertension. In addition, there is a growing understanding of pathogenesis that allows more targeted approaches to therapy to be explored in clinical trials. In this review, several aspects of SSc management including the more targeted therapies including strategies to block specific pathways or mediators have been discussed.
  724 130 -
EDITORIALS
Checklist prior to biologics: Indian perspective
Rajkiran Dudam, Narsimulu Gumdal
September 2016, 11(3):126-128
DOI:10.4103/0973-3698.187419  
  566 156 -
CASE-BASED REVIEW
Carcinomatous polyarthritis as a presenting manifestation of papillary carcinoma of thyroid gland
Himanshu Pathak, Ray Lonsdale, Ketan Dhatariya, Chetan Mukhtyar
September 2016, 11(3):164-166
DOI:10.4103/0973-3698.187411  
A 61-year-old female presented with 6 months of polyarthralgia associated with constitutional symptoms. These included weight loss, night sweats, lethargy and worsening mobility and activities of daily living. There was no significant medical history. On examination, she had synovitis of multiple joints. Investigations for rheumatoid factor and anti-cyclic citrullinated peptide antibody were negative. There was an acute phase response in the form of raised erythrocyte sedimentation rate and C-reactive protein. Contrast-enhanced computed tomography showed pancreatic and right ovarian cystic lesions, which turned out to be clinically insignificant. Positron emission tomography-computed tomography demonstrated fluorodeoxyglucose avid lesion in the right hemi-thyroid. Ultrasound of thyroid gland showed a 13 mm hyporeflective, irregular, subcapsular nodule in the upper lobe with some microcalcification. Fine needle aspiration cytology was diagnostic of papillary carcinoma, confirmed on total thyroidectomy. Arthritis completely resolved within 8 weeks postoperatively. We report the first case of paraneoplastic carcinomatous polyarthritis in association with a papillary thyroid carcinoma as evidenced by a resolution of joint manifestations and laboratory markers of inflammation posttotal thyroidectomy.
  643 49 -
REVIEW ARTICLES
Antiphospholipid syndrome in pregnancy
Anisur Rahman
November 2016, 11(6):117-121
DOI:10.4103/0973-3698.194543  
Antiphospholipid syndrome (APS) is an autoimmune condition, in which antiphospholipid antibodies (aPL) cause clinical features including thrombosis, fetal loss, and preterm delivery. Studies in large numbers of patients with APS show that they suffer both early and late fetal loss as well as complications of pregnancy such as preeclampsia. The fetal loss in patients with APS is not caused primarily by thrombosis, but by a number of biological effects of aPL that affect implantation of the embryo. These factors are not yet understood fully but include effects on trophoblast cell viability and migration, inflammation at the fetal-maternal interface, and activation of complement. The established management of pregnancy in patients with known obstetric APS is to give daily low-dose oral aspirin plus daily subcutaneous heparin. This gives a live birth rate of over 70%. The trials that led to this form of management being adopted were small but overall do support the use of the heparin/aspirin combination over aspirin alone. There is no definite evidence supporting the use of heparin plus aspirin in patients who are aPL-positive, but who have never suffered any problems in pregnancy. However, patients taking long-term warfarin for thrombotic APS should have this changed to heparin during pregnancy.
  476 152 -
ORIGINAL ARTICLES
Experience of biological agents usage in patients with rheumatoid arthritis from a Western Indian center
Anuj Singhal, Darshan Bhakuni, Vishal Marwaha, Vivek Hande, Garvit Bagga
September 2016, 11(3):144-148
DOI:10.4103/0973-3698.187412  
Background: In this study the clinical outcomes of different biologics agents in patients with rheumatoid arthritis (RA) has been appraised. Methods: Nineteen RA patients with DMARD failure were administered etanercept (n = 7) or infliximab (n = 12), and 17 RA patients with TNFi failure were administered rituximab (n = 13), abatacept (n = 2), or tocilizumab (n = 2) as per 2013 EULAR guidelines. Baseline demographic details, disease duration, rheumatoid factor, and anticyclic citrullinated peptide antibody were obtained. To monitor disease activity, disease activity score 28-erythrocyte sedimentation rate (DAS28-ESR) score was obtained at baseline, and after 3 months and 6 months of therapy initiation. Results: All the groups were comparable in the baseline. Over 6 months of treatment, the reduction in disease activity, as evidenced by reduction in the mean DAS28-ESR scores was statistically significant for all patients when considered together, as well as when individual biologics were considered separately (P < 0.05 in all cases). However, there was no statistically significant difference in the magnitude of reduction in the mean DAS28-ESR scores between patients who received etanercept and infliximab in DMARD failure RA patients (P = 0.877), or between patients who received rituximab, abatacept, and tocilizumab in TNFi failure patients (P = 0.455). Conclusions: Different biologic agents showed similar efficacy in patients with RA.
  502 94 -
REVIEW ARTICLES
Fertility and pregnancy in systemic lupus erythematosus
Alexis Jones, Ian Giles
November 2016, 11(6):128-134
DOI:10.4103/0973-3698.194546  
Systemic Lupus Erythematosus (SLE) is a chronic multisystem autoimmune disease with a heterogeneous pattern of clinical and serological manifestations. The predilection for women, particularly of childbearing age, combined with improved survival has led to increasing numbers of women with lupus considering pregnancy. Management of pregnancy in SLE however, requires careful planning and close medical and obstetric monitoring to ensure optimal outcomes. This review, discusses possible causes of subfertility, issues regarding contraception and family planning as well as management of lupus during pregnancy and outcomes in pregnant women with SLE.
  482 108 -
ORIGINAL ARTICLES
Treatment outcomes from a multiethnic lupus cohort with proliferative nephritis
Angela Pakozdi, Ravindra Rajakariar, Michael Sheaff, Debasish Pyne
September 2016, 11(3):136-143
DOI:10.4103/0973-3698.187417  
Objective: To assess treatment responses and long-term outcomes in a multiethnic lupus cohort with proliferative lupus nephritis (LN) from a single United Kingdom (UK) center. Methods: 86 lupus patients were diagnosed with active proliferative LN between 1995 and 2015 at Barts Health, a large inner city hospital in London, UK. They were grouped by ethnicity into South Asians (Bangladeshi, Indian, Pakistani, and Sri Lankan), blacks (African Blacks and Afro-Caribbeans), and Caucasians. Remission rates were analyzed at 6 and 24 months after induction treatment with cyclophosphamide (CYC) or mycophenolate mofetil (MMF). Prognostic factors for the treatment response were identified by regression analysis. Kaplan-Meier method was applied to assess long-term renal survival and Cox proportional hazards model for risk factors for developing end-stage renal disease. Results: MMF achieved a higher remission rate in blacks compared to CYC (70% vs. 16.7%, P = 0.005) at 6 months, showed a trend in Asians (77.8% vs. 38.9%, P = 0.057), and comparable response in Caucasians (42.9% vs. 55.6%, P = 0.614). Low baseline serum creatinine was the strongest predictor for favorable treatment response (odds ratio 0.98, 95% confidence interval [95% CI]: 0.97-0.99, P = 0.045). Renal survival glomerular filtration rate (GFR >15 ml/min/1.73 m 2 ) was 79.8% and 75.6% at 5 and 10 years, lowest in blacks (60.5%) followed by Asians (86.7%) then Caucasians (88.9%) (P = 0.030). Low GFR (GFR <30 ml/min/1.73 m 2 ) on presentation was an independent risk factor for poor 10 years renal survival (hazard ratio 32.55, 95% CI: 3.70-286.64, P = 0.002). Conclusions: MMF appears to be at least as effective as CYC as an induction agent in this multiethnic cohort but there were important differences in long-term renal outcomes based on ethnic group and baseline GFR and creatinine.
  491 74 -
EDITORIALS
Racial differences in presentation and treatment outcome of lupus nephritis
Manish Rathi, Aman Sharma
September 2016, 11(3):124-125
DOI:10.4103/0973-3698.187421  
  479 83 -
ORIGINAL ARTICLES
Role of CD134 and FAS and FAS ligand genes polymorphism as biomarkers for disease activity in lupus nephritis: A preliminary egyptian study
Hala I El Gendy, Mona N Abdel Gawad, Aml S Nasr, Elham A Ghoneim
September 2016, 11(3):129-135
DOI:10.4103/0973-3698.187414  
Objective: To illustrate the role of CD134 and FAS and FAS ligand genes polymorphism as biomarkers for disease activity in Egyptian patients with Lupus Nephritis. Materials and Methods: Twenty-five patients with biopsy-proven LN, 25 patients with SLE with no evidence of nephritis, and fifty patients matched apparently healthy volunteers. Levels of CD134 were measured using flow cytometry. FAS and FASL gene polymorphisms were detected using polymerase chain reaction-restriction fragment length polymorphism. Furthermore, carotid artery intima-media thickness (IMT) measurements were done. Results: LN group had highest level of CD134 compared to other two groups, and also higher among SLE compared to controls with highly significant differences in between. Frequency of AA genotype of FASA-670G polymorphism was significantly higher in LN and SLE patients than in controls. The frequency of A allele was statistically higher in LN and in SLE group than in controls. Furthermore, the frequency of CC genotype of C-844T polymorphism of FASL gene was significantly higher in LN and SLE patients than in healthy controls. The frequency of C allele was statistically higher in LN and in SLE group than in controls. Conclusion: Co-stimulatory molecules on CD4+ T-cells together with FAS, and FASL polymorphisms are associated with disease activity in this preliminary study.
  475 66 -
REVIEW ARTICLES
Lupus pregnancies: An Indian perspective
Mithun C Mohan, Vinod Ravindran
November 2016, 11(6):135-138
DOI:10.4103/0973-3698.194547  
There are several challenges to a successful pregnancy outcome in patients with lupus in India including unplanned pregnancies, myths and false beliefs related to lupus pregnancies and poor access to dedicated care. Pregnant lupus patients are best managed by a multidisciplinary team consisting of rheumatologist, obstetrician, and other relevant specialists. In this narrative review, we have appraised available literature on the outcomes of pregnancy among lupus patients in India, highlight the lacunae in the care of such patients, and also present perspective of this issue based on our own experience.
  420 99 -
PREFACE
Fertility and pregnancy in autoimmune rheumatic diseases
Caroline Gordon, Molly Thabah
November 2016, 11(6):115-116
DOI:10.4103/0973-3698.194539  
  356 118 -
ORIGINAL ARTICLES
Test–retest reliability and correlates of 6-minute walk test in patients with primary osteoarthritis of knees
Mahamed Ateef, Sivachidambaram Kulandaivelan, Shaziya Tahseen
December 2016, 11(4):192-196
DOI:10.4103/0973-3698.192668  
Background: In the assessment of primary osteoarthritis (OA) of knees several clinical tool including 6 minutes walk test (6 MWT) are used. The objective of this study was to to analyze the test-retest reliability of 6 MWT and its correlation with various parameters. Methods: Eighty patients (age, 56-79 years) with OA of knees met the inclusion criteria. Demographic and clinical characteristics including radiological severity of OA (by X-ray K/L grading) were recorded. Patients filled in the knee injury and osteoarthritis outcome score (KOOS) questionnaire. All patients performed 6 MWT twice with at least 48 h gap in-between. Results: Test–retest reliability of 6 MWT in primary OA knee patients was excellent with ICC 0.991 (95% confidence interval was 0.986–0.994). 6 MWT had a weak correlation with KOOS-symptom and KOOS-activities of daily living (rho = 0.397 and 0.364 respectively), a strong correlation with KOOS-pain and KOOS-sports (rho = 0.605 and 0.521 respectively), and a very strong correlation with KOOS-quality of life (rho = 0.758). It had a weak correlation with age and height (r = 0.497 and 0.302), a strong correlation with VAS, weight, and BMI (rho = −0.655, r = −0.510, and − 0.691, respectively), and a very strong correlation with disease severity (rho = −0. 849). Conclusion: 6 MWT was a reliable test and positively correlated with all KOOS subscales and negatively correlated with other parameters except height in primary OA knee.
  421 44 -
REVIEW ARTICLES
Inflammatory rheumatic diseases in the elderly
Vikramraj K Jain, Vir Singh Negi
December 2016, 11(4):207-215
DOI:10.4103/0973-3698.192684  
Rapid aging of world's population will translate into more elderly patients in the near future. Diagnosis of inflammatory rheumatic diseases in this age group is complicated by atypical clinical features compared to the younger onset group, nonspecific positivity of serological parameters, and confounding radiological signs. Management of these diseases also presents unique challenges in lieu of altered physiology of elderly, cognitive decline, presence of comorbidities, and altered immune system (inflammaging). Hence, this review attempts to synthesize the existing knowledge of the clinical, diagnostic, and therapeutic idiosyncrasies of inflammatory rheumatic diseases in this subgroup of population.
  398 66 -
BRIEF REPORT
Clinical profile and outcome of kawasaki disease in children in himalayan region of North India
Vijay Yadav, Parveen Bhardwaj, Minoo Sharma
September 2016, 11(3):149-152
DOI:10.4103/0973-3698.187418  
Background: Kawasaki disease (KD) is an important cause of acquired heart disease with significant morbidity if not diagnosed and managed appropriately. It is usually under-diagnosed due to lack of knowledge regarding this entity among pediatricians and dermatologists. In this retrospective, tertiary care teaching institute based study clinical profile and outcome of KD in children from Himalayan region of north India was appraised. Methods: Clinical details of all the cases diagnosed as KD over 3 years from January 2011 to December 2013 were collected from inpatient files and outpatient follow-up clinical records. Results: Twelve children were diagnosed to have KD out of which seven had classical KD and five had incomplete KD. Mean age at presentation was 2.45 years and male to female ratio was 1.4:1. All children were treated with intravenous immunoglobulin and aspirin. Two out of 12 children had residual coronary artery disease at 6 weeks, 3 and 6 months of follow-up echocardiography. Conclusions: KD remains an important differential diagnosis for fever, rash, and lymphadenopathy in children <5 years.
  396 49 -
REVIEW ARTICLES
Nonbiologic disease-modifying antirheumatic drugs in pregnancy
Durga Prasanna Misra, Jignesh Babulal Usdadiya, Vir Singh Negi
November 2016, 11(6):156-162
DOI:10.4103/0973-3698.194551  
Rheumatic disease often affects young females and males in the reproductive age group. Management of these diseases with immunosuppressive disease-modifying antirheumatic drugs (DMARDs) is fraught with potential risks for the developing fetus as well as adverse pregnancy outcomes. In developing countries like India, most patients are treated with conventional DMARDs. We reviewed recent literature on safety during pregnancy for conventional disease-modifying agents, in view of the recently published landmark guidelines of the British Society for Rheumatology and the European League against Rheumatism. Exposure to leflunomide and cyclophosphamide is contraindicated in mothers during pregnancy and preconception phase, and fathers during conception due to risks for the developing fetus. Maternal exposure to methotrexate (MTX) during pregnancy or preconception confers higher risk of adverse pregnancy and fetal outcomes although paternal exposure may not be harmful. Sulfasalazine is safe during pregnancy; however, paternal exposure may reduce fertility, but this is reversible with cessation of the drug. Hydroxychloroquine, azathioprine, cyclosporine, and tacrolimus are compatible with maternal and paternal exposures without evidence for increased risk to the developing fetus. Insufficient data exist regarding safety profile of tofacitinib and apremilast during pregnancy. Rheumatologists should be cautious while starting these drugs in women and men of reproductive age group, and counsel patients accordingly to minimize the risk of adverse fetomaternal outcomes.
  362 77 -
FROM THE EDITORS DESK
From the Editor's Desk
Vinod Ravindran
September 2016, 11(3):123-123
DOI:10.4103/0973-3698.187422  
  332 88 -
REVIEW ARTICLES
Modified-release prednisone in rheumatoid arthritis: Rationale for chronotherapy, mechanistic considerations, and clinical implications
Vinod Ravindran, Ernest H Choy
December 2016, 11(4):216-221
DOI:10.4103/0973-3698.192680  
Pain, stiffness of joints, and functional disability of rheumatoid arthritis (RA) are maximum in the morning. Cytokines, especially interleukin (IL)-6, demonstrate a circadian variation in patients with RA and contribute to the severity of aforementioned symptoms. Perturbed cortisol response in patients with RA is not effectively able to negate the effects of interleukin 6 rise in the early morning. Modified-release (MR) prednisone is a polymer-based drug delivery system which releases the drug 4 h after the ingestion of tablet closely simulating the cortisol rise and peak in patients with RA. In this review, we focus on the rationale for chronotherapy, mechanistic considerations, and clinical implications of MR prednisone.
  351 63 -
IMAGES IN RHEUMATOLOGY
Skeletal fluorosis mimicking seronegative arthritis
Anuj Shukla
September 2016, 11(3):171-173
DOI:10.4103/0973-3698.187413  
  347 64 -
REVIEW ARTICLES
Pregnancy and systemic vasculitis
Himanshu Pathak, Chetan Mukhtyar
November 2016, 11(6):145-149
DOI:10.4103/0973-3698.194549  
The systemic vasculitides are rare diseases of vessel wall inflammation. They are classified according to the vessel wall size. Systemic vasculitis is usually diagnosed after the fifth decade of life, except Takayasu arteritis and Bechet's disease which can manifest earlier. The increase in understanding of etiopathogenesis and effective treatment strategies have significantly improved outcomes associated with vasculitis, and more patients are living longer than ever. Primary systemic vasculitis in women of reproductive age group poses a special challenge. The diseases as well as the drugs used in their treatment affect fertility. Vasculitis increases maternal and fetal complications during pregnancy. However, there is some immunological rationale to suggest that normal hormonal and immunological changes in the maternal body may improve the tolerance to certain Th1-modulated syndromes. The successful outcome of pregnancy depends on vasculitis activity status, type of immunosuppressive medications, and maternal comorbidities. The inherent risks and ethical dilemmas of conducting clinical trials in this group of patients remain an obstacle in collecting high-quality evidence. The pregnancy should be monitored closely by a specialist team comprising a rheumatologist, an obstetrician, and other specialties as per organ involvement to ensure a successful outcome.
  350 48 -
CASE-BASED REVIEW
Uncommon clinical presentation of cryoglobulinemia vasculitis successfully treated with rituximab and mycophenolate mofetil
María Ahijón Lana, Carmen De La Cruz Tapiador, Lucio González Sanz, Aurelio Hernández Laín, Alejandro Jesús González Gutierrez, Bárbara Gutierrez Ruano, José Ramón Rodríguez Franco
September 2016, 11(3):167-170
DOI:10.4103/0973-3698.187416  
We report the case of a 80-year-old woman with a severe mononeuritis multiplex over bilateral lower limb, with no other clinical signs or symptoms of vasculitis. Laboratory test showed elevated erythrocyte sedimentation rate and C reactive protein, low C4 levels, positive rheumatoid factor and polyclonal IgG and IgA cryoglobulins at a high cryocrit. Malignancies disorders and infectious diseases screening was negative. Nerve biopsy revealed signs of vasculitis with inflammatory infiltrate of epineural vessels, axonal degeneration and moderate loss of myelinated fibres. The diagnosis of essential mixed cryoglobulinemia vasculitis was established. She received treatment with plasmapheresis and high doses of steroids with progression of the symptoms to the upper limbs and persistence of positive cryoglobulins, low C4 levels and high acute phase reactants. She required treatment with rituximab and mycophenolate mofetil to control the disease. In conclusion we report an uncommon clinical presentation of a cryoglobulinemia vasculitis successfully treated with two B- cell depleting therapies.
  346 42 -
IMAGES IN RHEUMATOLOGY
Temporomandibular joint in rheumatoid arthritis: Clinicoradiological aspects
S Jayachandran, Priyanka Khobre
September 2016, 11(3):174-176
DOI:10.4103/0973-3698.187415  
  295 66 -
BRIEF REPORTS
Use of art as therapeutic intervention for enhancement of hand function in patients with rheumatoid arthritis: A pilot study
Sayali Khedekar, Apurv P Shimpi, Ashok Shyam, Parag Sancheti
Ahead of print schedule 0, 0(0):0-0
DOI:10.4103/0973-3698.199130  
Background: Conventional physiotherapy treatment for patients with rheumatoid hand emphasizes on single-plane movements and strengthening exercises directed toward improvement of function and prevention of deformities. This may be nondirective and lacks creativity and hence may predispose to a high attrition from therapy. The current study aimed to evaluate the efficacy of art as a creative therapeutic procedure for enhancement of hand functions, self-perception, and quality of life in patients with rheumatoid arthritis (RA). Methods: A single-blinded, randomized controlled pilot study was conducted on 17 participants suffering from RA. The control group received conventional physiotherapy while the experimental group received art-based intervention with bimanual projects (viz., origami, paper quelling, clay modeling, and oil painting). Both groups received intervention for 45 min daily for 4 weeks. Pre- and post-interventional assessment was done using grip and pinch strength, Grip Ability Test (GAT), Jebsen–Taylor Hand Function Test (JHFT), Australian-Canadian Osteoarthritis Hand Index (AUSCAN), Michigan Hand Outcome Questionnaire (MHQ), and Health Assessment Questionnaire (HAQ). Results: There was an improvement in hand functions (grip strength [P = 0.042], two-point pinch strength [P = 0.039], three-point pinch strength [P = 0.043], GAT [P = 0.043], JHFT [P = 0.043]), self-perception (AUSCAN [P = 0.043], MHQ [P = 0.043]), and quality of life (HAQ; P = 0.043) in art therapy patients which was similar to conventional therapy patients. Conclusion: Art is an equally effective therapeutic intervention to conventional therapy for the enhancement of hand functions, self-perception, and quality of life in patients with RA.
  342 12 -
REVIEW ARTICLES
Neonatal lupus: An update
Sathish Kumar
November 2016, 11(6):139-144
DOI:10.4103/0973-3698.194548  
Neonatal lupus erythematosus (NLE) is a syndrome that usually presents in the fetus or neonates that is caused by transplacental passage of autoantibodies from the mother. It is a clinical spectrum of cutaneous, cardiac, and systemic abnormalities observed in the newborn or infants whose mothers have autoantibodies against Ro/SSA and/or La/SSB. Congenital complete heart block is the most serious manifestation of NLE that can develop in utero or after birth. Multidisciplinary team involvement is indicated. This article will provide an overview the presentation of NLE and will review the evidence for current therapies.
  270 61 -
Fertility and pregnancy in systemic sclerosis and other autoimmune rheumatic diseases
Vijay K R Rao
November 2016, 11(6):150-155
DOI:10.4103/0973-3698.194550  
Autoimmune rheumatic diseases usually occur in women of childbearing age. In the past, pregnancy in women with autoimmune rheumatic diseases was not considered safe and was discouraged because gestation could worsen maternal disease and vice versa, and the disease could negatively affect the gestational outcome. However, women with rheumatic diseases often wish to have children even when functional disability is present. The great improvement in the systematic approach to pregnancy over the past few decades has allowed an increasing number of affected women to fulfill their family plan. Women should be informed about potential risks related to their disease and also should be reassured that a good pregnancy outcome is possible if conception occurs when they are in a stable remission state, teratogenic medications have been properly withdrawn, and immunosuppressant drugs that are safe in pregnancy have been maintained to prevent disease flare. The interaction of pregnancy and the rheumatic diseases can vary from spontaneous improvement to flare of disease symptoms. Rheumatic diseases differ in regard to the occurrence of complications during pregnancy and to pregnancy outcome. This review describes the maternal course of systemic sclerosis, ankylosing spondylitis, polymyositis and dermatomyositis during pregnancy, fetal outcome, and therapy during pregnancy and postpartum.
  276 43 -