ReviewCurrent rheumatology reports2017
Deficiency of Adenosine Deaminase 2 (DADA2), an Inherited Cause of Polyarteritis Nodosa and a Mimic of Other Systemic Rheumatologic Disorders.
Review in Current rheumatology reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
34 citing papers in PubMed, 70 citations in OpenAlex.
- DADA2 as a Model of Monogenic Immune Vasculopathy: From Immunopathogenesis to Precision Therapeutics.Biomolecules · 2026Review
- Structural basis for the substrate specificity of Helix pomatia AMP deaminase and a chimeric ADGF adenosine deaminase.The Journal of biological chemistry · 2025Article
- Two Unrelated Iranian Patients with Adenosine Deaminase 2 Deficiency: A Case Report and Review of Treatment.Case reports in immunology · 2024Article
- Case Report: NovelFrontiers in genetics · 2024Article
- A Narrative Review of the Neurological Manifestations of Human Adenosine Deaminase 2 Deficiency.Journal of clinical immunology · 2023Review
- A new CECR1 mutation associated with severe hematological involvement in ADA2 deficiency.Immunity, inflammation and disease · 2023Article
- A case series of ten plus one deficiency of adenosine deaminase 2 (DADA2) patients in Iran.Pediatric rheumatology online journal · 2023Article
- A Cohort Study on Deficiency of ADA2 from China.Journal of clinical immunology · 2023Article
- Letter to the Editor: Comments on "Acute Partial Oculomotor Nerve Palsy and Optic Neuritis Preceding Juvenile Idiopathic Arthritis: A Case Report".Korean journal of ophthalmology : KJO · 2023Article
- Deficiency of adenosine deaminase 2: a challenging differential diagnosis of polyarteritis nodosa.Reumatologia · 2023Review
- Mechanisms of vascular inflammation in deficiency of adenosine deaminase 2 (DADA2).Seminars in immunopathology · 2022Review
- Allogeneic Hematopoietic Cell Transplantation for Patients With Deficiency of Adenosine Deaminase 2 (DADA2): Approaches, Obstacles and Special Considerations.Frontiers in immunology · 2022Review
- Editorial: DADA2 and other monogenic vasculitides.Frontiers in immunology · 2022Article
- Lentiviral correction of enzymatic activity restrains macrophage inflammation in adenosine deaminase 2 deficiency.Blood advances · 2021Article
- Molecular mechanisms of phenotypic variability in monogenic autoinflammatory diseases.Nature reviews. Rheumatology · 2021Review
- Anti-TNF-Related Leukocytoclastic Vasculitis in Ulcerative Colitis: A Case Report.International journal of environmental research and public health · 2021Article
- A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report.BMC rheumatology · 2021Article
- Case Report: An Adult Patient With Deficiency of Adenosine Deaminase 2 Resembled Unilateral Frosted Branch Angiitis.Frontiers in medicine · 2021Article
- Anakinra and canakinumab for patients with R92Q-associated autoinflammatory syndrome: a multicenter observational study from the AIDA Network.Therapeutic advances in musculoskeletal disease · 2021Article
- Two cases of ADA2 deficiency presenting as childhood polyarteritis nodosa: novel ADA2 variant, atypical CNS manifestations, and literature review.Clinical rheumatology · 2020Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purpose of reviewA new autoinflammatory disease, deficiency of adenosine deaminase 2 (DADA2), caused by mutations in the CECR1 gene, was first reported in 2014. This review aims to update progress in defining, treating, and understanding this multi-faceted disorder. RECENT
findingsDADA2 was first described in patients with systemic inflammation, mild immune deficiency, and vasculopathy manifested as recurrent stroke or polyarteritis nodosa (PAN). More than 125 patients have now been reported, and the phenotype has expanded to include children and adults presenting primarily with pure red cell aplasia (PRCA), or with antibody deficiency. Age of onset and clinical severity vary widely, even among related patients, and are not clearly related to CECR1 genotype. Inflammatory features often respond to anti-TNF agents, but marrow failure and severe immune deficiency may require hematopoietic stem cell transplantation. ADA2 is expressed and secreted by monocytes and macrophages, but its biological function and the pathogenesis of DADA2 are uncertain and will remain an important area of research. Pre-clinical investigation of ADA2 replacement therapy and CECR1-directed gene therapy are warranted, but complicated by the absence of a suitable animal model.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.