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Introduction: Mixed connective tissue disease (MCTD) is a rare entity in children. There is a paucity of studies on juvenile‐onset MCTD (jMCTD) worldwide especially from Southeast Asia. Objectives: To describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India. Methods: A predesigned detailed case proforma in an excel format was prepared and was sent to all the Pediatric Rheumatology centers in India. Eleven centers provided the clinical and laboratory data of their jMCTD patients, which was then compiled and analyzed in detail. Results: Thirty‐one jMCTD patients from 11 centers were included in the study. Our cohort had 27 females and four male patients over 12 months (August 2021 to July 2022). The median age at presentation was 12 years (range 5–18 years) and the median duration of symptoms was 24 months at diagnosis (range 2–96 months). The common features included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%). At a mean follow‐up of 43 months (range 1–168 months), 45% of them were in remission. There were two deaths reported, due to macrophage activation syndrome and sepsis respectively. Conclusion: We present the largest multicenter experience on jMCTD from the Indian subcontinent. The study's findings serve as a crucial stepping stone toward unraveling the complexities of jMCTD and improving patient care and management strategies. [ABSTRACT FROM AUTHOR]

Background: Juvenile localized scleroderma (JLS) or morphoea, a rare chronic autoimmune disease predominantly affects skin, subcutaneous tissue and occasionally the adjacent muscle, fascia and bone. We report the largest single‐centre cohort of patients with JLS from India. Methods: Patients who were diagnosed to have JLS were enrolled from the Paediatric Dermatology Clinic and the Paediatric Rheumatology Clinic of a tertiary care referral hospital in India. Collected data included details of the clinical profile, laboratory investigations and management. Results: We analysed 84 patients with Juvenile localized scleroderma. Median age of disease onset was 5 years, and median age at diagnosis was 8 years. Commonest subtype was linear scleroderma (57 patients, 67.7%) followed by plaque morphoea and generalized morphoea. Fourteen patients (16.6%) were noted to have extracutaneous manifestations (ECMs). These included arthritis in eight (33.3%), brain parenchymal abnormalities in four (4.7%) and pulmonary involvement in two (8.3%) patients. Antinuclear antibody (ANA) was positive in eight/25 patients (32%; diffuse and speckled pattern in four patients each). One amongst these also had elevated anti‐dsDNA titres. Positive ANA was found to have no association with ECMs (p 1.000). Patients were treated using methotrexate (61 patients; 72.6%), dexamethasone oral mini‐pulse (OMP; 35 patients; 41.6%), calcipotriol (39 patients; 46.4%), topical corticosteroids (32 patients; 38%) and topical tacrolimus (three patients; 3.7%). Using linear regression analysis, administration of dexamethasone OMP and calcipotriol was found to be a predictor of good treatment response (p 0.034 and 0.019, respectively). Conclusion: Early use of systemic corticosteroids along with methotrexate may be more beneficial than methotrexate therapy alone. [ABSTRACT FROM AUTHOR]

The outcome for children with rheumatic diseases has been dramatically altered by the use of biological therapies. Increasing use of these agents will need careful monitoring for long term safety, particularly in children. Current data on safety of these drugs stem exclusively from Western literature. There is clear need for a registry of all children with rheumatic diseases who are commenced on biological agents to ensure appropriate pharmacovigilance. In this perspective, we discuss the need for and the role of a biologics registry for children with rheumatic diseases in India. [ABSTRACT FROM AUTHOR]

Introduction: Children with systemic lupus erythematosus have a more challenging and difficult course as compared to their adult counterparts. Today, the aim of therapy for any child with lupus is to keep the child in a state of sustained remission with minimal or no use of steroids. This laudable goal is often difficult to achieve for the child with lupus. In addition to the use of disease modifying agents, sometimes in combination, Rituximab (RTX) is also used as an off-label indication to manage such patients. Objectives: To study the use, efficacy and safety of RTX in a cohort of patients with pediatric lupus followed at a single tertiary level center in Northern India. Methods: This paper is a retrospective review looking at the use of RTX in children with systemic lupus at a tertiary level pediatric rheumatology center in North India over a period of seventeen years. This paper describes the indications, use, efficacy and safety of RTX in childhood systemic lupus erythematosus. Results: RTX was used in 17 of 225 pediatric lupus patients (7.5%), with the most common indication being resistant renal disease (53%). Significant improvement was seen in all domains studied: The mean SLEDAI was 16.25 prior to RTX and reduced to 1.43 six months after the RTX (p value 0.001), steroid use dropped from 100% pre- RTX to 33% at 2 years, there was a sustained reduction in proteinuria in the patients with nephritis from a mean urine spot protein creatinine ratio of 3.1 pre RTX to 0.4 at one year post RTX (p=.006). Finally, 82% of the children had no flare during the follow up (median 24 months). No patient had any adverse event. Conclusions: This study confirms that RTX is very effective in childhood lupus and can be safely used even in a country with a very high burden of infectious diseases. This data adds to the scarce literature in this area from the developing world. [ABSTRACT FROM AUTHOR]

Background: JIA studies demonstrate that there is a "window of opportunity" early in the disease course during which appropriate management improves outcomes. No data is available regarding patients' pathway, before first pediatric rheumatology (PR) evaluation in India, a country where health-care costs are self- paid by patients and where a significant shortage of pediatric rheumatologists (PRsts) is known. This study aimed to describe time from onset of symptoms to first PR visit of JIA patients to a tertiary center in India and factors that impact this. Methods: This retrospective study is from data collected at the PR center, Sir Ganga Ram Hospital (SGRH) in New Delhi. JIA patients fulfilling ILAR 2004 criteria and seen at least twice from 1st October 2013 to 30th September 2018 were included. Data collected were: demographic details, history of disease, referral practitioner, clinical and laboratory features, treatments. Mann-Whitney U-test, Chi square and logistic regression were used as appropriate to study factors that determined time to first PR visit. Results: Five hundred and twenty patients were included: 396 were diagnosed at this PR center (group A), 124 were previously diagnosed as JIA and managed by non PRsts before first PR visit (group B). Median time from symptom onset to first PR visit was 4.1 months and median distance travelled 119.5 km. Despite ongoing treatment, group B patients had more aggressive disease and resided further away as compared to Group A patients. On univariate analysis, factors that predicted PR visit within 3 months were private patients, short distance to travel, family history of inflammatory disease, history of fever, history of acute uveitis or high ESR. On multivariate analysis all these factors were significant except high ESR and acute uveitis. Conclusion: Time to first PR assessment at this center was comparable to that seen in western countries. Cost of care and long distance to the center delayed consultation; acuity of complaints and family history of rheumatologic condition hastened referral. Possible solutions to improve referral to PR centers would be to increase the number of PRsts and to improve medical insurance coverage. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease (KD) is emerging as the most common medium vessel vasculitis in children. Recurrence of KD (0.8%‐3.0%) is unusual and not commonly reported. Objective: To study the clinico‐laboratory profile of children with recurrent KD. Methods: Case records of 714 children with KD, registered from January 1994 to April 2018 in the Pediatric Rheumatology Clinic at the Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research Chandigarh, were reviewed and data were analyzed for children having had recurrent KD. Results: Seven (0.98%) children (five boys; two girls) had recurrent KD in our cohort of 714 patients. Mean age at diagnosis was 4.3 years (range, 2‐9 years). Six (85.7%) had recurrence of KD within 1.5 years. Incomplete KD was more common during recurrence (71.4%) as compared to the first episode (33.3%). Five children with recurrent KD developed desquamation during the first week of illness. Laboratory investigations did not show any significant difference during the two episodes of KD. Six of the seven children in the first episode received treatment with intravenous immunoglobulin (IVIG). Five of the seven children received IVIG during recurrence. One child had transient left main coronary artery ectasia during the first episode of KD as well as during recurrence and it normalized after treatment on both occasions. Conclusion: Recurrences in KD are unusual. Over a period of 24 years the recurrence rate in our cohort of children with KD was 0.98%. Further, children with recurrences tended to have milder and incomplete form of the disease. [ABSTRACT FROM AUTHOR]

Background: Published Indian studies on the economic burden of juvenile idiopathic arthritis (JIA) are lacking. Methods: A prospective observational study recruited pediatric patients aged from 1 to 12 years with JIA in the pediatric rheumatology clinic of a public sector tertiary care hospital. Direct healthcare costs and indirect costs for transportation, accommodation of the caregivers, and productivity loss for work absenteeism were assessed. Results: The proportions of direct annualized cost assessed in 60 patients (mean (SD) age 8.46 (2.24) year) spent on outpatient visits, blood tests, imaging investigations, other tests, medications and hospitalization were 0.85%, 12.8%, 9.0%, 2.9%, 41.7% and 32.7%, respectively. Direct healthcare costs for blood tests and medicine were lowest in oligoarticular JIA and highest in systemic onset JIA and (P=0.043 and 0.001 respectively). The direct and indirect costs were higher with the use of biologic agents (n=9) than in those without (n=51). Conclusions: JIA imposes considerable economic burden with the largest share attributable to medicines, and maximum in those with systemic onset JIA. [ABSTRACT FROM AUTHOR]

Background: Data on rheumatological disorders in children from developing countries like India are scarce. Hence, this study aimed to understand the clinical and epidemiological profile of rheumatological disorders in children as this can help organize comprehensive evidence-based health care services. Methodology: A retrospective hospital-based study was designed in pediatric rheumatology clinic of the All India Institute of Medical Sciences, Patna, India, from January 2015 to December 2016. Clinical and laboratory findings and response to therapy of all children with rheumatological disorders were evaluated. Results: A total of 60 children with various rheumatological disorders were included in the study. Juvenile idiopathic arthritis (JIA) was the most common pediatric rheumatology disorder observed, and polyarticular JIA was the predominate subtype observed in our patients. The mean age of disease onset was 9.1 ± 3.6 years (age range: 1-16 years). Methotrexate was the most common disease modifying antirheumatic drug used. None of the children received newer biological agents due to financial constraints. The use of alternative medicine was observed in 10% of cases. Conclusion: The clinical and epidemiological profile of children with rheumatological disorders in our patient group was different from the European countries and Western world. There is a need for introspecting the lack of using biological agents and its potential impact in managing JIA in our patient group. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is now the commonest cause of acquired heart disease in children in developed countries. KD occurs all over the world, including developing countries. The present study steps out to explore our hypothesis, driven by clinical observation over the last 18 years, whether children with KD in North India are of a higher socioeconomic status than children with other rheumatologic diseases. One hundred consecutive children with KD, registered in Pediatric Rheumatology Clinic before January 2011, were enrolled as cases. Children with other rheumatologic diseases were taken as controls. Assessment of socioeconomic status was done by administering the Aggarwal scale. Data were collected through interview. Statistical analysis was done using SPSS package version 16. On univariate analysis, male sex, higher educational status of parents, urban residence, immunization status being complete, and higher scores on Aggarwal scale were found to be significantly associated with KD. On multivariate analysis, only male sex and urban residence were found to be significantly associated with KD ( p < 0.001). Families of children with KD tend to have a better sociodemographic profile when compared with other pediatric rheumatologic disorders in North India. These results, however, need to be replicated in a multicentric study for any firm conclusions to be drawn. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls. Study design: Prospective study. Setting: Pediatric rheumatology clinic of a tertiary care hospital in Eastern part of India. Participants: Seventy-five children (2-12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sexmatched controls. Intervention: Weight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years. Main outcome measures: weight, height and body mass index. Results: Subtype distribution of juvenile idiopathic arthritis was: oligoarthritis (49%, n=37), rheumatoid factor negative polyarthritis (27%, n=20), rheumatoid factor positive polyarthritis (8%, n=6), systemic onset (15%, n=11) and enthesitis related arthritis (1.3%, n=1). Anthropometric parameters in children with juvenile idiopathic arthritis were not significant different from controls. Comparison between the subtypes showed significant differences in height ( P=0.011), weight ( P=0.005), and growth velocity ( P=0.005), but not in body mass index. Systemic onset disease led to significant restriction in height ( P=0.018; 95% CI 2.13-33.77) and weight ( P=0.008; 95% CI 1.47-14.43) compared to controls. Growth velocity was significantly affected in rheumatoid factor positive polyarthritis ( P=0.003; 95% CIO. 46-3.14). Conclusions: Children with juvenile idiopathic arthritis do not have significantly lower values of anthropometric parameters compared to controls. Significant restriction in height and weight is seen in systemic onset disease, and growth velocity is significantly reduced in rheumatoid factor positive subjects. [ABSTRACT FROM AUTHOR]

Abstract: The concept of paediatric rheumatology clinics is relatively new to India. With the specialty still in its formative years, referrals are still delayed and children usually present after having visited multiple facilities. Our centre, which has been one of the “early birds” in setting up a paediatric rheumatology clinic in a tertiary hospital in Mumbai, reports on the spectrum of cases seen since its inception in 2003 and thereby provides a framework for those wanting to open such centres in India or for that matter in countries where paediatric rheumatology is coming of age. [Copyright &y& Elsevier]

Abstract: Paediatric rheumatology has come of age. In a country as large as India with its large childhood population with rheumatic diseases, there is a great need to develop training programmes in paediatric rheumatology. Fundamental research is needed to study the incidence, prevalence and variations in clinical features of common rheumatic diseases in the Indian population. Newer modalities of therapy need to be developed to suit local needs and conditions. Tools to evaluate outcomes need to be developed to suit the cultural conditions of the land. Resources have to be developed to provide continuity of care to these children with chronic diseases in remote villages. All of this requires planning, leadership, sustained effort and collaboration between patients and physicians and between private and public agencies. [Copyright &y& Elsevier]

The article offers information on a seminar and conferences to be held in India from February to September 2011 including the Training on basic understanding of yoga techniques and training in neurophysiological techniques relevant to yoga research to be held in Bangalore, the III Biennial Conference of Gastrointestinal Infection Society of India, GISICON 2011 in Manipal and the IX National Conference on Pediatric Rheumatology in Lucknow.