Blockades in the Pathway of Specialty Care in Rheumatoid Arthritis: A Report Based on Indian Rheumatology Association Database.

AIM: The study aims to identify factors influencing referral patterns and delays in rheumatoid arthritis (RA) patients across clinical settings in India. MATERIALS AND METHODS: A prospective, multicenter, observational study collected data from eight centers using the Indian Rheumatology Association (IRA) database. Patient-related factors and referral factors were determined based on patient narration. The modified PRASAD scale categorized patients' socioeconomic status. RESULTS: The study included 4,643 RA patients from eight centers. Data from 35 patients were excluded due to inconsistent reporting of diagnosis and delay. Lack of awareness was the predominant factor causing the delay in referral. Approximately, 39% of patients were referred to the rheumatology specialty within 6 months of disease onset, while 26% reported later, and 34% reported over 2 years. Referral delays were linked to socioeconomic factors in Madhya Pradesh (21.43%) and West Bengal (28.57%). Lack of awareness about the disease and rheumatology specialty was highest in West Bengal (100%), followed by Delhi and Rajasthan (93.70%). Misconceptions about modern medicine, reluctance to refer patients to the rheumatologist, and previous treatment by other specialities were other factors influencing referral delay. Primary care clinicians' unawareness of the rheumatology specialty was the primary reason for referral delay in Gujarat (33.56%) and Delhi and Rajasthan (25.18%). CONCLUSION: Both patient and healthcare professional-related factors contribute to referral delays in RA patients. Major factors causing referral delays include reluctance to refer and inadequate knowledge about rheumatology among primary care physicians and the general public. Patients' education and occupation also influence the timing of referrals to specialty care.

Life and Times of Yellapragada SubbaRow.

Dr Yellapragada SubbaRow was an Indian-born American biochemist who made numerous discoveries in various fields of science. Born in 1895 into a poor family, he went on to have medical training at the Madras Medical College, Chennai, Tamil Nadu, India. After many hardships, he arrived in the United States of America and earned a diploma in tropical medicine and a PhD in biochemistry from Harvard. His illustrious career began with the isolation of phosphorus in body fluids along with Fiske. They discovered phosphocreatine and adenosine triphosphate (ATP) as well. He later became the director of Lederle Laboratories, where he discovered the tetracycline antibiotic group, which saved many lives. He synthesized many vitamins, including folic acid, niacin, and biotin. In 1948, he discovered methotrexate (MTX) for childhood cancer, which paved the way for many chemotherapeutics and is also used in the management of several autoimmune conditions. Despite numerous medical advancements made by SubbaRow, awareness in the scientific world regarding his life and many contributions remains underrecognized. This article discusses SubbaRow's life, academics, and achievements in a nutshell.

Management of simultaneous bilateral immune-mediated peripheral ulcerative keratitis.

PURPOSE: Peripheral ulcerative keratitis (PUK) is a sight threatening disease, often immune mediated and can rapidly progress to corneal perforation. Here we report the successful management of simultaneous bilateral peripheral ulcerative keratitis presenting with corneal perforation with surgical intervention and systemic immunosuppression. CASE DESCRIPTION: A 41-year-old female presented with similarly located super-nasal peripheral ulcerative keratitis (PUK) in both eyes and focal area of adjoining scleritis in the left eye. Serological investigations for systemic immune-mediated conditions showed positive rheumatoid factor test. She underwent cyanoacrylate glue application in both eyes, and was started on topical and oral corticosteroids and oral immunomodulators, and the right eye stabilized. The left eye required a second surgical intervention with a corneal patch graft following which the condition stabilized. CONCLUSION AND IMPORTANCE: This case highlights the importance of investigating for an underlying systemic etiology in PUK and the role of systemic immunosuppression prior to and after surgical intervention in order to arrest the disease and prevent recurrences.

Deficiency of Adenosine Deaminase 2 in Adults and Children: Experience From India.

OBJECTIVE: Deficiency of adenosine deaminase 2 (DADA2) is a potentially fatal monogenic syndrome characterized by variable manifestations of systemic vasculitis, bone marrow failure, and immunodeficiency. Most cases are diagnosed by pediatric care providers, given the typical early age of disease onset. This study was undertaken to describe the clinical phenotypes and treatment response both in adults and in children with DADA2 in India. METHODS: A retrospective analysis of pediatric and adult patients with DADA2 diagnosed at various rheumatology centers across India was conducted. Clinical characteristics, diagnostic findings, and treatment responses were analyzed in all subjects. RESULTS: In total, 33 cases of DADA2 were confirmed in this cohort between April 2017 and March 2020. Unlike previous studies, nearly one-half of the confirmed cases presented during adulthood. All symptomatic patients exhibited features of vasculitis, whereas constitutional symptoms and anemia were more common in pediatric patients. Cutaneous and neurologic involvement were common, and 18 subjects had experienced at least one stroke. In addition, the clinical spectrum of DADA2 was expanded by recognition of novel features in these patients, including pancreatic infarction, focal myocarditis, and diffuse alveolar hemorrhage. Treatment with tumor necrosis factor inhibitors (TNFi) was initiated in 25 patients. All of the identified disease manifestations showed marked improvement after initiation of TNFi, and disease remission was achieved in 19 patients. Two cases were complicated by tuberculosis infection, and 2 deaths were reported. CONCLUSION: This report presents the first case series of patients with DADA2 from India, diagnosed by adult and pediatric care providers. The findings raise awareness of this syndrome, particularly with regard to its presentation in adults.

Rituximab therapy combined with methotrexate for severe necrotizing scleritis in a case of granulomatosis with polyangiitis.

A 19-year-old girl presented with severe pain and redness in her right eye and also with nasal congestion and epistaxis since the past 5 months. The patient was systemically investigated and tested positive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR-3 ANCA) with raised erythrocyte sedimentation rate (ESR). The provisional diagnosis of granulomatosis with polyangiitis (GPA) was made, and she was started on systemic steroids and azathioprine after consultation with the rheumatologist. However on steroid taper, she developed severe reactivation of the scleritis and the corneal involvement was noted in the form of peripheral ulcerative keratitis. Instead of starting another course of high dose oral cortico-steroids or pulse cyclophosphomide, she was started on rituximab infusion (two doses), and oral methotrexate was added, leading to the successful remission of the disease.