Childhood Vasculitis.

Of the primary vasculitis pediatricians are familiar with, Kawasaki disease and IgA vasculitis are the most common. The other large, medium and small vessel vasculitis are seldom seen in practice. Though rare, early diagnosis and appropriate management is critical for the best outcome.

Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort.

Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.

Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months.

Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males.

Pediatric Rheumatic Disease in Lower to Middle-Income Countries: Impact of Global Disparities, Ancestral Diversity, and the Path Forward.

Pediatric rheumatology subspecialists treat chronic autoimmune diseases with onset in childhood. Prompt diagnosis and ongoing management of these conditions are imperative to prevent damage from ongoing inflammation. Here, we aim to describe the current landscape of pediatric rheumatic disease in lower to middle-income countries (LMICs) and explore current barriers to understanding global disease burden.

Rituximab use in pediatric systemic lupus erythematosus: Indications, efficacy and safety in an Indian cohort.

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.

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort.

Objective: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features.

Methods: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied.

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis.

Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc.

Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD.

Results: Eighty-six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison.

Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis.

Objective: To develop a composite disease activity score for systemic JIA (sJIA) and to provide preliminary evidence of its validity.

Methods: The systemic Juvenile Arthritis Disease Activity Score (sJADAS) was constructed by adding to the four items of the original JADAS a fifth item that aimed to quantify the activity of systemic features. Validation analyses were conducted on patients with definite or probable/possible sJIA enrolled at first visit or at the time of a flare, who had active systemic manifestations, which should include fever.

Systemic immunosuppressive therapies for uveitis in developing countries.

There are multiple approaches to inhibit inflammatory molecules and pathways in noninfectious uveitis. The cornerstone of local and systemic anti-inflammatory treatment is corticosteroid therapy. Corticosteroids remain the most potent and efficacious drugs for treating intraocular inflammation.

Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee.

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC).

Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists.

Factors impacting referral of JIA patients to a tertiary level pediatric rheumatology center in North India: a retrospective cohort study.

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.

Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Objective: To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA).

Methods: The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample.

The Hindi version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Hindi language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients.

Childhood Lupus--Diagnosis and Management.

Though rare, childhood lupus is a disease with the potential to have serious short and long term effects in children. These effects are to do with the disease itself, organ damage consequent to ongoing inflammation and/or because of side effects of medications. As children have an early disease onset, accrual organ damage over the years and growth and puberty issues are important aspects of care.

Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients.

Objective: To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).

Methods: In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database.

Results: A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries.

IgG4-related orbital inflammation presenting as unilateral pseudotumor.

IgG4 related systemic disease (IgG4-RSD) has been recognised in the last few years. Orbital pseudotumor as a presentation of IgG4-RSD is one of the rare complaints encountered in pediatric population. It is an inflammatory condition of unknown etiology characterized by tumorous swelling of the organs, characteristic histopathologic changes and elevated IgG4: IgG plasma cells ratio.

Seronegative arthritis in South Asia: an up-to-date review.

This article summarises the available information on seronegative arthritides from South Asian countries, namely India, Pakistan, Bangladesh, Sri Lanka, Nepal, and Bhutan. The diseases described are spondyloarthritides (SpA), including ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), inflammatory bowel disease-related arthritis (IBDa), enthesitis-related arthritis (ERA) of the paediatric age group, and undifferentiated spondyloarthritis (uSpA). Relevant information on SpA from South Asia is scarce.

Vertebral sarcoid mimicking ankylosing spondylitis or just a co-incidence?

Objective: To illustrate that among the protean musculoskeletal manifestations of sarcoidosis back pain that could mimic inflammatory back pain of ankylosing spondylitis (AS), should also be considered.

Method: A case report of a HLA B27 negative patient with classical features of inflammatory back pain that was initially diagnosed as AS is being presented. He showed poor response to standard treatment with nonsteroidal anti-inflammatory drugs and physiotherapy exercises with increasing symptoms of pain, stiffness and development of typical posture of AS.

Low-dose and high-dose methotrexate are two different drugs in practical terms.

Methotrexate (MTX) was originally synthesised as an anti-cancer drug. Soon it was also used in immunoinflammatory diseases, mainly in the field of rheumatology. However, the dose used in oncology is several-fold higher as compared to the dose used in systemic immunoinflammatory rheumatological diseases.

Human heme oxygenase-1 deficiency presenting with hemolysis, nephritis, and asplenia.

Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999.

Outcome measures in pediatric rheumatology.

Children with rheumatologic disorders need periodic systematic evaluation of their disease status so that all aspects of the child's life that are affected can be adequately assessed. The commonest rheumatologic disease that afflicts children is Juvenile Idiopathic Arthritis (JIA). The child with JIA should have several domains assessed at regular intervals.

The place of pediatric rheumatology in India.

Rheumatology is a relatively new specialty in the field of Pediatrics in India where there are only a few centres of excellence. Awareness about these conditions in childhood is lacking and patients are often referred late resulting in poor long term outcomes. This article highlights the needs of the paediatric rheumatology patients which are complex and should encompass both acute and chronic care and also plan a smooth transition to the adult rheumatology world.