Publications by authors named "Ariharan Anantharachagan"

Background: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterisation of the AAE-C1-INH cohort in UK is required to inform management.

Objectives: To describe the disease burden of AAE-C1-INH, long-term prophylaxis (LTP) and the clinical, immunochemical and treatment profiles of AAE-associated diseases in UK.

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Background: Seafood allergy (SA), including allergy to shellfish (crustacean and mollusks) and fish, is among the 4 most common food allergies causing anaphylaxis, but there are limited data showing SA clinical management in different countries.

Objective: We sought to characterize a large cohort of patients with fish and shellfish allergy and to facilitate standardization of future care for this increasingly common allergic disease.

Methods: We performed a retrospective, observational, noninterventional study from 945 patients from 2015 to 2019 in 7 hospitals in the United States and the United Kingdom to evaluate SA.

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Regardless of their age, adult patients with Wiskott-Aldrich syndrome should be considered for hematopoietic stem cell transplantation if clinically indicated.

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Article Synopsis
  • - The survey aimed to gather comprehensive demographic data on hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the UK to enhance service planning and patient care.
  • - A total of 1152 patients with HAE were identified, with a prevalence of 1:59,000 for HAE-1/2 and 1:734,000 for acquired C1 inhibitor deficiency, revealing significant patient demographics and treatment patterns.
  • - Findings showed that 45% of HAE patients were on long-term prophylaxis, primarily using danazol, and a significant number had acute treatment supplies available at home, highlighting the need for improved healthcare services for these conditions.
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Article Synopsis
  • In March 2020, the UK Primary Immunodeficiency Network created a registry to track outcomes of individuals with Primary Immunodeficiency Diseases (PID) and Secondary Immunodeficiency Diseases (SID) after infection with SARS-CoV-2, reporting 310 cases.
  • The overall mortality rate was 17.7%, with certain groups experiencing higher rates; for example, Common Variable Immunodeficiency Disease (CVID) had an infection fatality rate (IFR) of 18.3%.
  • Individuals with PID and SID faced greater risks in terms of inpatient mortality and generally died at younger ages than the broader population, with risk factors including older age, low lymphocyte count before infection, and existing co-morbidities
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Protein losing enteropathy can present as an immunodeficiency. This report describes a rare cause of protein losing enteropathy due to a lymphoenteric fistula and how a novel use of a pre-existing combined imaging technique of single-photon emission computed tomography/computed tomography lymphoscintigraphy helped in making the diagnosis.

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Common variable immune deficiency (CVID) is a primary immunodeficiency disease, characterized by hypogammaglobulinemia, recurrent infections and various complications. The clinical heterogeneity of CVID has hindered identification of an underlying immune defect; diagnosis relies on clinical judgement, alongside evidence-based criteria. The lack of pathognomonic clinical or laboratory features leads to average diagnostic delays of 5 years or more from the onset.

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