Indian Rheumatology Association guidelines for the management of ANCA associated vasculitis.

Background: The ACR in 2021 and the EULAR in 2022 published recommendations for management of ANCA-associated vasculitis. Given the differences in the demographic, clinical profiles, and the socio-economic realities between various countries, there is a need for development of guidelines for the management of AAV for less economically developed regions of the world.

Methods: These guidelines were made following the GRADE methodology.

Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data.

Background: Neurological involvement can occur in systemic lupus erythematosus (SLE) due to co-existing neuromyelitis optica spectrum disorder (NMOSD). The symptoms can mimic those of neuropsychiatric manifestations of SLE. Pathogenic anti-aquaporin-4 (AQP4) antibodies, commonly found in NMOSD, are responsible for the neuroinflammatory response and secondary demyelinating lesions.

Deficiency of adenosine deaminase 2 (DADA2): Review.

The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disease caused by loss-of-function (LOF) mutations in the ADA2 gene and was first described in 2014. Initially, it was described as vasculopathy/vasculitis that mostly affected infants and young children and closely resembled polyarteritis nodosa (PAN). Skin rash and ischemic/hemorrhagic stroke are predominant symptoms.

Vascular dysfunction in juvenile idiopathic arthritis: a systematic review and meta-analysis.

We performed a systematic review and meta-analysis of studies evaluating vascular function in patients with JIA. Relevant literature published from 1st January 1965 to 1st March 2022 was searched systematically utilizing PubMed, Web of Science, and Embase databases. Observational studies were included-patients with JIA (classified according to the International League of Associations for Rheumatology criteria) were included as cases (study population) and age/sex-matched healthy participants as controls (comparator group).

Prognostic Role of Measurement of Renal Resistive Index in Systemic Sclerosis.

Objective: The spectrum of vascular involvement in systemic sclerosis (SSc) includes digital ulcers, gangrene, Raynaud's phenomenon, renovascular disease, and pulmonary hypertension (PH). Recognition of markers of subclinical vascular disease in SSc is an area of active research, but such studies are limited. This study assesses the role of measurement of the renal resistive index (RRI) as an early marker of renal and systemic vasculopathy.

Managing ANCA-associated vasculitis during the COVID-19 pandemic: results from an online survey.

Management of ANCA-associated vasculitis (AAV) during the COVID-19 pandemic poses unique therapeutic challenges. An online survey was conducted to understand physician's choices for treating AAV during the COVID-19 pandemic. Web-based survey featuring nineteen questions was circulated amongst physicians across various specialties.

Rare case of autoimmune hypothyroidism presenting with pancytopenia.

Hypothyroidism can involve any organ system in the body with the involvement of haematopoietic system seen in about 30% of the cases. Anaemia is the most common haematological involvement with the affection of other cell lines being exceedingly rare and limited to occasional case reports. Here we present a case of a 14-year-old boy who presented with fever and pancytopenia and was later diagnosed to be a case of autoimmune hypothyroidism.

Pattern of respiratory muscle activity during exercise tests in children born prematurely.

Introduction: Preterm children display only slightly lower exercise capacity than term children do during their development, despite their previous cardiopulmonary impairments. This raises doubts about the role of the respiratory muscles' influence on exercise capacity. This study aimed to compare respiratory muscle activity in preterm and term children using an exercise test.

Ovarian dysfunction with moderate-dose intravenous cyclophosphamide (modified NIH regimen) and mycophenolate mofetil in young adults with severe lupus: a prospective cohort study.

Background: Ovarian toxicity is a dreaded complication of cyclophosphamide (CYC). With the use of lower cumulative doses of intravenous CYC (modified NIH regimens) and availability of better markers of ovarian toxicity, the incidence of ovarian dysfunction needs reassessment. Lupus disease activity, by itself, is also believed to affect ovarian function negatively.

Anti-Jo-1 Syndrome Often Misdiagnosed as Rheumatoid Arthritis (for Many Years): A Single-Center Experience.

Background: Antisynthetase syndrome is characterized by a triad of myositis, arthritis, and interstitial lung disease. Anti-Jo-1 is the most common associated autoantibody. This study planned to look at the presentation of anti-Jo-1 antisynthetase syndrome in a single Indian center.

Prevalence of Metabolic Syndrome in Rheumatoid Arthritis Patients: A Case Control Study from a Tertiary Care Centre in North India.

Background: Cardiovascular disease (CVD) is the leading cause of mortality in patients with rheumatoid arthritis (RA). Along with traditional cardiovascular risk factors and systemic inflammation, metabolic syndrome (MetS) contributes to CVD and increased mortality in patients with RA. In this study we determine the prevalence of MetS in RA patients presenting to a tertiary care centre in north India.

Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis-related pulmonary artery hypertension and cutaneous vascular complications.

Pulmonary artery hypertension (PAH) remains the leading cause of morbidity and mortality in systemic sclerosis, while Raynaud's phenomenon and digital ulcers significantly add to the morbidity in systemic sclerosis (SSc). This study was undertaken to evaluate the role of sildenafil in PAH, Raynaud's phenomenon, and digital ulcers in systemic sclerosis patients. A prospective, open-label, uncontrolled pilot study was done at a tertiary care centre in India to study the safety and efficacy of oral sildenafil in PAH, Raynaud's phenomenon, digital infarcts, and ulcers in SSc.