Paediatric rheumatologic emergencies are rare but potentially fatal, and may present as the first manifestation of an underlying rheumatic disease. These conditions frequently present with nonspecific constitutional or organ-specific symptoms, posing a diagnostic challenge—particularly in children without a prior diagnosis. The objectives of this study were to evaluate the clinical characteristics and outcomes of paediatric rheumatological emergencies, comparing patients with and without known pre-existing rheumatologic conditions, and secondarily to suggest warning signs that may aid in the recognition of these situations. We conducted a retrospective cohort study over three-years at a tertiary paediatric rheumatology centre in western India. Medical records of all children (<18 years) diagnosed with rheumatologic diseases were reviewed. Demographic, clinical, and outcome data were analysed. Emergencies were defined as life-threatening or organ-threatening events requiring urgent evaluation or intensive care. Descriptive and comparative analyses were performed to evaluate outcomes between first-presentation and known rheumatic disease groups. Of 420 patients screened, 41 (9.8

Background This study assessed safety and immunogenicity of Serum Institute of India Pvt Ltd (SIIPL)’s tetanus toxoid (TT), diphtheria toxoid (DT), and acellular pertussis booster vaccine (Tdap).Research Design and Methods In this Phase II/III, multicenter, randomized, active-controlled, open-label study, 1500 healthy individuals, aged 4–65 years, were randomized to receive a single dose of SIIPL Tdap or comparator Tdap vaccine (Boostrix®; GlaxoSmithKlines, India). Adverse events (AEs) during initial 30 minutes, 7-day, 30-day post-vaccination were assessed. Blood samples were taken before and 30 days post-vaccination for immunogenicity assessment.Results No significant differences in incidence of local and systemic solicited AEs were observed between the two groups; no vaccine-related serious AEs were reported. SIIPL Tdap was non-inferior to comparator Tdap in achieving booster responses to TT and DT in 75.2% and 70.8% of the participants, respectively, and to pertussis toxoid (PT), pertactin (PRN), and filamentous hemagglutinin (FHA) in 94.3%, 92.6%, and 95.0% of the participants, respectively. Anti-PT, anti-PRN, and anti-FHA antibody geometric mean titers in both the groups, were significantly higher post-vaccination compared to pre-vaccination.Conclusions Booster vaccination with SIIPL Tdap was non-inferior to comparator Tdap with respect to immunogenicity against tetanus, diphtheria, and pertussis and was well tolerated.

Multisystem inflammatory syndrome in children (MIS-C) has emerged as one of the several challenges thrown by the ongoing severe acute respiratory syndrome-coronavirus 2 pandemic. Although diagnostic criteria of MIS-C have now been established to raise the clinical suspicion for the condition, the Kawasaki disease (KD)-like phenotype of MIS-C presents with additional therapeutic dilemmas. The treatment guidance till date remains empirical with consideration of intravenous immunoglobulin (IVIG) and glucocorticoids. However, treatment of cases refractory to the current conventional therapy with respect to biologics remains uncertain. We describe here, an 8-year-old boy with MIS-C (KD-like phenotype) with severe cardiac dysfunction refractory to IVIG and pulse methylprednisolone who responded to tumor necrosis factor-α inhibition using infliximab.

Takayasu's arteritis (TA) is a rare primary vasculitis, typically affecting the aorta and its main branches causing progressive vessel wall inflammation with concentric wall thickening and stenosis producing a variety of ischemic symptoms or aneurysms. Although etiopathogenesis of this disease remains poorly understood, an autoimmune basis is widely suggested in addition to genetic and environmental factors, among which evidence implicating Mycobacterium tuberculosis (MT) has been provided. We discuss hereby a 12-year-old boy brought with refractory renovascular hypertension secondary to aortoarteritis as found on ultrasound Doppler and computed tomography angiogram, who, apart from fulfilling criteria for TA, was also found to have latent tuberculosis. He was managed with multiple antihypertensives, immunosuppressants, and antitubercular therapy, despite which he developed hypertensive crises. A renal angioplasty proved beneficial as hypertension was better controlled allowing a gradual taper of all antihypertensives over an 8-week period and improved renal blood flow and renal function.

There have been several reports across the globe regarding the presentation of a severe multi-system hyperinflammatory syndrome, resembling Kawasaki disease (KD), in the pediatric population during the SARS-CoV-2 pandemic. The exact pathophysiology is still unclear; however, children typically demonstrate multi-organ dysfunction and less respiratory system involvement compared to adults. The limited literature is available at present for the identification and management of such patients. In this study, we investigated four cases in children ages 11–15 years that fulfilled the case definition for the pediatric multi-system inflammatory syndrome. All were found negative for SARS-CoV-2 from oropharyngeal swabs and stool. As they were having symptoms of diarrhea, tests for bacterial and enteric viral infections were performed after SARS-CoV-2 testing. Molecular analysis revealed that all the children were infected with enterovirus (Echovirus-18). Early and exact diagnosis is vital for timely, effective, and potentially life-saving management of such cases.

COVID-19 pandemic witnessed rapid development and use of several vaccines. In India, a country-wide immunization was initiated in January 2021. COVISHIELD, the chimpanzee adenoviral-vectored vaccine with full-length SARS-COV-2 spike insert and COVAXIN, the whole virus-inactivated vaccines were used. To assess and compare immune response of health-care-workers to COVISHIELD (n=187) and COVAXIN (n=21), blood samples were collected pre-vaccination, 1month post-1/post-2 doses and 6months post-dose-2 and tested for IgG-anti-SARS-CoV-2 (ELISA) and neutralizing (Nab,PRNT50) antibodies. Spike-protein-specific T cells were quantitated by IFN-γ-ELISPOT. In pre-vaccination-antibody-negative COVISHIELD recipients (pre-negatives, n=120), %Nab seroconversion (median, IQR Nab titers) increased from 55.1% (16, 2.5-36.3) post-dose-1 to 95.6% (64.5, 4.5-154.2, p<0.001) post-dose-2 that were independent of age/gender/BMI. Nab response was higher among pre-positives with hybrid immunity at all-time points (p<0.01-0.0001) and independent of age/gender/BMI/Comorbidities. Post-dose-2-seroconversion (50%, p<0.001) and Nab titers (6.75, 2.5-24.8, p<0.001) in COVAXIN-recipients were lower than COVISHIELD. COVAXIN elicited a superior IFN-γ-T cell response as measured by ELISPOT (100%; 1226, 811-1532 spot forming units, SFU/million PBMCs v/s 57.8%; 21.7,1.6-169.2; p<0.001). At 6months, 28.3% (15/53) COVISHIELD and 3/3COVAXIN recipients were Nab-negative. T cell response remained unchanged. During immunization, COVID-19 cases were detected among COVISHIELD (n=4) and COVAXIN (n=2) recipients. At 6months, 9cases were recorded in COVISHIELD-recipients. This first-time, systematic, real-world assessment and long-term follow up revealed generation of higher neutralizing antibody titers by COVISHIELD and stronger T-cell response by COVAXIN. Diminished Nab titers at 6months emphasize early booster. Immunogenicity/efficacy of vaccines will change with the progression of the pandemic needing careful evaluations in the field-settings.

Despite high level vaccination and the availability of two different types of vaccines, whole cell (wP) and acellular vaccines (aP), the resurgence of pertussis has been reported in many countries. Antigenic variation within circulating and vaccine strains is the most documented reason reported for the resurgence of pertussis. Research on genetic divergence among circulating and vaccine strains has largely been reported in countries using aP vaccines. There are inadequate data available for antigenic variation in B. pertussis from wP-using countries. India has used wP for more than 40 years in their primary immunization program. The present study reports five clinical isolates of B. pertussis from samples of pediatric patients with pertussis symptoms observed in India. Genotypic and phenotypic characterization of clinical isolates were performed by serotyping, genotyping, whole genome analyses and comparative genomics. All clinical isolates showed serotype 1, 2 and 3 based on the presence of fimbriae 2 and 3. Genotyping showed genetic similarities in allele types for five aP genes within vaccine strains and clinical isolates reported from India. The presence of the ptxP3 genotype was observed in two out of five clinical isolates. Whole-genome sequencing was performed for clinical isolates using the hybrid strategy of combining Illumina (short reads) and oxford nanopore (long reads) sequencing strategies. Clinical isolates (n = 5) and vaccine strains (n = 7) genomes of B. pertussis from India were compared with 744 B. pertussis closed genomes available in the public databases. The phylogenomic comparison of B. pertussis genomes reported from India will be advantageous in better understanding pertussis resurgence reported globally with respect to pathogen adaptation.

Adult-onset Still’s disease (AOSD) is an uncommon inflammatory condition presenting with high grade fever, arthralgia, skin rash, and leukocytosis. Autoimmune thyroid disease (AITD) is commonly seen in females in their third to fifth decade and usually missed to screen in other autoimmune diseases. In this study, the case of a 17-year-old female patient from Pune is reported who presented to Bharati hospital in November 2020, with a six-month history of high-grade fever, arthritis, and elevated acute phase reactants along with hypothyroidism. She was diagnosed with AOSD (based on Yamaguchi criteria) and AITD with positive anti-thyroid peroxidase (anti-TPO) antibodies. She responded well to oral steroids and thyroid supplements. This case draws attention to the rare association between AOSD and AITD.

Multisystem inflammatory syndrome in children (MIS-C) is one of the many challenges thrown by the ongoing SARS CoV-2 pandemic. A high index of suspicion is warranted for the diagnosis of MIS-C as presenting features overlap with Kawasaki disease and toxic shock syndrome. The treatment guidance is empirical with consideration of intravenous immunoglobulin (IVIG) and glucocorticoids. However, treatment of refractory MIS-C remains eluded. We describe here a 7-year-old boy with MIS-C refractory to IVIG and pulse methylprednisolone who responded to interleukin-6 inhibition using tocilizumab.

Background: Being a long-term preventive measure, COVID-19 vaccines are used in global populations. In India, country-wide immunization drive was initiated in January 2021. Methods: To assess immune response of health-care-workers to COVISHIELD(n=187) and COVAXIN(n=21), blood samples collected pre-vaccination and 1month-post-1/post-2 dose, administered 28days apart, were tested for IgG-anti-SARS-CoV-2 (ELISA) and neutralizing (Nab, PRNT50) antibodies. Spike protein-specific T cells were quantitated by IFN-γ ELISPOT and Flow-cytometry-based Intra-cellular-secretion (ICS) for IFN-γ/IL2. Findings: Among pre-vaccination-antibody negative (pre-negatives, n=120) and positive (pre-positives, n=67) COVISHIELD recipients, %Nab seroconversion and median (IQR) Nab titers were 55.1%/95.6% and 16(IQR 2.5-36.3)/(64.5, IQR 34.5-154.2, p<0.000) and independent of age/gender. In pre-positives, Nab titers increased from 75 (IQR 29-129) before vaccination to 3050 (1282-3998, p< 0.001, n=42) post-1st dose, but declined to 1740(911-3116, p<0.05) post-2nd dose. Though the number of COVAXIN recipients was small, post-2nd dose humoral response was lower than COVISHIELD (50% seroconversion and median titer 6.75, IQR 2.5-24.75, p<0.0001). Despite higher age, COVAXIN recipients elicited superior IFN-γ-T cell response than COVISHIELD, as measured by ELISPOT (100% bsp;1226 bsp;522-3628 spot forming units, SFU/million PBMCs v/s 57.8%; 21.7, 0-2149; p<0.001) and ICS (56.9%, 24.8-78.6 v/s 30%, 8.1-90.4, p<0.05). During immunization, COVID-19 cases were detected among COVISHIELD (n=4) and COVAXIN (n=2) recipients. Interpretation: This first-time, systematic, real-world assessment revealed stronger humoral (COVISHIELD) and cellular (COVAXIN) immune responses respectively. Relation of dose interval and post-2nd decline in Nab titers in pre-positives (COVISHIELD) needs evaluation. Immunogenicity/efficacy of vaccines will change with the progression of the pandemic and needs to be assessed in field-setting. Funding Information: Biotechnology Industry Research Assistance Council (BIRAC), Grant/Award Number: BIRAC/BT/NBM0095/02/18. Declaration of Interests: Authors declare no any commercial or financial conflict of interest. Ethics Approval Statement: The study was approved by the “Human Ethics Committee” of BVDUMCH. Written informed consent was obtained from all the participants.

Background: Aim was to study the clinical profile, laboratory parameters and outcome of children with pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS).Methods: This is an observational study done in a PICU of a tertiary care teaching hospital in Pune conducted over seven months starting from 1st June to 31st December, 2020 and which included the children meeting the case definition of PIMS-TS.Results: A total 25 children were included of which 13 (52%) were males. Median age was 7 years. Fever of short duration was present in all children and 23 (92%) had gastrointestinal symptoms. 21 (84%) had features of shock while myocardial dysfunction was seen in eight (32%) children. Kawasaki disease like features were present in seven (28%) while five (20%) showed coronary artery dilatation. All had neutrophilia and lymphopenia while 10 (40%) showed thrombocytopenia. Interleukin-6 levels, done in six, and were found to be raised in all. SARS-CoV-2 RT-PCR and IgG antibody were positive in two while only IgG was positive in 14 (56%) children. Seven required mechanical ventilation while eleven were managed with HFNO. 16 (64%) children received both IVIG and steroids, two (8%) children received only IVIG while seven (28%) received only steroids. Two children each additionally received Tocilizumab and infliximab. 24 (96%) children survived.Conclusions: Fever, gastrointestinal symptoms and hypotensive shock were predominant features of PIMS-TS. 2/3rd of children had demonstrable SARS-CoV-2 antibodies. Cardiac dysfunction was seen in 1/3rd of the children. It is a life-threatening illness requiring critical interventions, multi-organ support and various immunomodulators.

Autoimmune thrombocytopenia (ITP) is one of the best characterized autoimmune diseases which is classified into primary (idiopathic) and secondary forms. A high index of suspicion is warranted for the diagnosis of secondary ITP. There is limited data on association of ITP with Psoriasis. We herein describe a 11 years old girl who presented with a rare association of ITP and Psoriasis who responded to oral steroids.

Chronic exposure to minocycline, a commonly used anti-acne medication, results in a variety of autoimmune syndromes. Minocycline-induced vasculitis often associated with the presence of antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) has been reported in Western literature, mainly in adults. Herein, we report a rare case of a 15-year-old male child on minocycline treatment for 1 year, who presented with fever, skin rash, and polyarthralgia for 4 months. His erythrocyte sedimentation rate (ESR) level was elevated, ANA and myeloperoxidase ANCA were positive, and skin biopsy revealed leukocytoclastic vasculitis. Upon discontinuation of minocycline and prescribing a short course of oral steroids, all his symptoms resolved and ESR normalized within 2 weeks. The child remained asymptomatic with normal acute-phase reactants at 2-month follow-up. This outcome suggested that minocycline was the main cause of ANCA-associated vasculitis.

Multisystem inflammatory syndrome in children (MIS-C) is a challenge thrown by the ongoing SARS CoV-2 pandemic. We need to diagnosis MIS-C with a high suspicion as presenting features overlap with Kawasaki disease (KD) and Toxic shock syndrome (TSS). The management in general is empirical use of IVIG and glucocorticoids. However, treatment of refractory MIS-C remains unexplored. We herein describe, a 12 year old boy presented to Bharati hospital, Pune, India in November 2020 with MIS-C refractory to IVIG and pulse methylprednisolone who responded to IL-6 inhibition using tocilizumab.