ADA2-deficient cells exhibit increased levels of cell death and metabolic disturbances.

Hematopoietic Stem Cell Transplantation in Rheumatic Diseases.

Deficiency of adenosine deaminase 2: Myriad faces in a single family.

Targeting cytokine pathways: the role of biologics in autoinflammatory disorders.

Hepatic Vascular Involvement in Adenosine Deaminase 2 Deficiency (DADA2): Case Reports and Literature Review.

The Natural History of Deficiency of Adenosine Deaminase 2 Vasculitis in a Large Cohort and Factors Associated With Disease-Related Damage.

Decoding neurovascular signatures: advanced imaging insights in DADA2-Related Cerebral Microangiopathy.

Gene therapy supports long-term reconstitution of patient hematopoietic stem cells in deficiency of adenosine deaminase 2.

Diverse Phenotypic Expressions of ADA2 Deficiency: Two Case Studies.

Phenotypes of patients with more than one autoinflammatory disease-associated gene variant: overlapping and mixed autoinflammatory disorders.

Diagnostic Implications and Correlates of Plasma Adenosine Deaminase 2 Activity and ADA2 Variants.

Early-onset neutropenia and mixed phenotype in ADA2 deficiency: diagnostic and therapeutic challenges.

Early-onset vasculitis: a toddler with ADA2 deficiency.

A Clinical Case Report of Deficiency of Adenosine Deaminase 2 Syndrome (DADA 2) Presenting as a Brachial Artery Aneurysm.

Dominant negative ADA2 mutations cause ADA2 deficiency in heterozygous carriers.

Dadaism catches up with DADA2.

Pediatric Sneddon syndrome presenting with early-onset liver fibrosis: a rare case report.

Vasculitis in autoinflammatory diseases.

Pediatric-Onset Polyarteritis Nodosa and Deficiency of Adenosine Deaminase 2: Clinical Overlap and Divergence.

Inhibition of lysosomal degradation increases expression of mutant ADA2 in DADA2 monocytes.

NET Proteomic Profiling Reveals New Pathways Potentially Implicated in Dendritic Cell-Mediated Inflammation in DADA2 Patients.

Nailfold videocapillaroscopy in patients with deficiency of adenosine deaminase 2 (DADA2): a case-control study.

The pathogenesis, clinical presentations and treatment of monogenic systemic vasculitis.

Arterial and venous thrombosis in systemic and monogenic vasculitis.

[ADA2, an Adenosine Deaminase Isozyme Acting as a Regulator of Autoinflammation].

Mimics and challenging presentations of DADA2.

Deficiency of Adenosine Deaminase 2 Masquerading as Behçet's Disease: Phenotypic Mimicry with HLA-B*51 Positivity.

Infantile Pure Red Cell Aplasia Secondary to Deficiency of Adenosine Deaminase2 (DADA2) Syndrome-Time to Think Beyond Diamond Blackfan Anemia.

Idiopathic polyarteritis nodosa - does it still exist? Viewpoint 1: as our knowledge makes progress, idiopathic polyarteritis nodosa is fading away.

Idiopathic polyarteritis nodosa-does it still exist? Viewpoint 2: idiopathic polyarteritis nodosa is rare, but still exists.

Deficiency of adenosine deaminase 2 skews adaptive immune repertoires toward specific sets of T- and B-cell receptors.

Case Report: Novel ADA2 variants cause atypical adenosine deaminase 2 deficiency.

Deficiency of adenosine deaminase 2 (DADA2) with recurrent ischemic stroke, fever and cutaneous changes: A case report.

Central nervous system vasculopathy: inherited or acquired? The DADA2 conundrum.

Extreme Phenotypic Variation in Siblings with Identical Homozygous Mutations Causing ADA2 Deficiency: A Case Series.

Misdiagnosed for 14 Years: Adenosine Deaminase 2 (ADA2) Deficiency in a Teen Mimicking Polyarteritis Nodosa.

Deficiency of adenosine deaminase 2 (DADA2) presented with bilateral renal subcapsular hematoma: a case report and literature review.

Bilateral cataracts in a three-year-old with deficiency of adenosine deaminase 2 (DADA2), hyperferritinemia, and prolonged steroid use.

Carrier frequency and incidence estimation of deficiency of adenosine deaminase 2 in the Chinese population based on massive exome sequencing data.

Rare primary vasculitis: update on multiple complex diseases and the new kids on the block.

Early bone marrow alterations in patients with adenosine deaminase 2 deficiency across disease phenotypes and severities.

Clinical spectrum of and outcomes for Indian children with deficiency of adenosine deaminase 2 (DADA2): a multicentric study.

Deficiency of adenosine deaminase 2 (DADA2) with bilateral renal subcapsular hematoma: a case report and literature review.

Adalimumab-Associated Depressive Symptoms in a Patient With Vasculitis, Autoinflammation, Immunodeficiency, and Hematologic Defects Syndrome.

[Advances in gene therapy for inborn errors of immunity].

Deficiency of Adenosine Deaminase 2.

Update on ocular manifestations of the main monogenic and polygenic autoinflammatory diseases.

Human ADA2 Deficiency: Ten Years Later.

Deficiency of adenosine deaminase 2 leading to recurrent Hodgkin lymphoma: A case report.

Deficiency of adenosine deaminase 2 (DADA2) presenting with diffuse skeletal muscle vasculitis.

ADA2 regulates inflammation and hematopoietic stem cell emergence via the A2bR pathway in zebrafish.

Development of EBV Related Diffuse Large B-cell Lymphoma in Deficiency of Adenosine Deaminase 2 with Uncontrolled EBV Infection.

Deficiency of adenosine deaminase 2: a genetic autoinflammatory disorder mimicking childhood polyarteritis nodosa.

A case report on deficiency of adenosine deaminase 2 with relapse-remission course and analysis of genotype-phenotype correlation.

Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study.

Infliximab as successful treatment option in a case of adenosine deaminase 2 deficiency.

A Successful Infliximab Treatment of a Pediatric Case of Severe Polyarteritis Nodosa With a Cerebral Infarction and a Decreased Adenosine Deaminase 2 Activity.

Deficiency of Adenosine Deaminase 2: Clinical Manifestations, Diagnosis, and Treatment.

Ocular involvement in adult and paediatric patients with monogenic autoinflammatory diseases: a Spanish multicentre retrospective study.

Vasculitis associated with adenosine deaminase 2 deficiency: at the crossroads between Behçet's disease and autoinflammation. A viewpoint.

Deficiency of adenosine deaminase 2 as an unrecognized cause of early-onset stroke and cranial nerve palsy.

A new CECR1 mutation associated with severe hematological involvement in ADA2 deficiency.

Cerebral Aneurysms and Recurrent TIAs in a 42-Year-Old Patient With DADA2 Mutation: A Case Report.

A case with febrile attacks and vasculopathy associated with ADA2 and MEFV pathogenic variants.

Kidney Involvement in Autoinflammatory Diseases.

Adenosine Deaminase 2 Deficiency Caused by Biallele Variants Including Splicing Variant: The First Case in Korea.

Adenosine deaminase 2 deficiency in a Chinese patient: Report of one novel mutation and literature review.

Case Report: Patient with deficiency of ADA2 presenting leukocytoclastic vasculitis and pericarditis during infliximab treatment.

Deficiency of adenosine deaminase 2 (DADA2): Review.

A case series of ten plus one deficiency of adenosine deaminase 2 (DADA2) patients in Iran.

Brainstem Infarction in Immunodeficiency Identified as Adenosine Deaminase 2 Deficiency: Case Report.

Clinical Symptoms, Laboratory Parameters and Long-Term Follow-up in a National DADA2 Cohort.

Evaluation and Management of Deficiency of Adenosine Deaminase 2: An International Consensus Statement.

Finding a Quorum in Deficiency of Adenosine Deaminase 2 Management.

A brazilian nationwide multicenter study on deficiency of deaminase-2 (DADA2).

Case Report: Interindividual variability and possible role of heterozygous variants in a family with deficiency of adenosine deaminase 2: are all heterozygous born equals?

A wide spectrum of phenotype of deficiency of deaminase 2 (DADA2): a systematic literature review.

Comparison of disease phenotypes and mechanistic insight on causal variants in patients with DADA2.

Deficiency of adenosine deaminase 2: a challenging differential diagnosis of polyarteritis nodosa.

A Cohort Study on Deficiency of ADA2 from China.

ADA2 deficiency (DADA2) misdiagnosed as systemic onset juvenile idiopathic arthritis in a child carrying a novel compound heterozygous ADA2 mutation: a case report.

A Unique Case of Deficiency of Adenosine Deaminase 2 Single in a Young Adult Patient.

A novel frameshift variant in the ADA2 gene of a patient with a neurological phenotype: a case report.

ADA2 Deficiency Mimicking Acute Disseminated Encephalomyelitis.

A Rare Genetic Mutation Leading to a Deficiency of Adenosine Deaminase 2 Enzyme in a Long-Standing Case of Cutaneous Polyarteritis Nodosa: A Case Report.

Recent topics related to etiology and clinical manifestations of cutaneous arteritis.

Case Report: Consistent disease manifestations with a staggered time course in two identical twins affected by adenosine deaminase 2 deficiency.

CellCallEXT: Analysis of Ligand-Receptor and Transcription Factor Activities in Cell-Cell Communication of Tumor Immune Microenvironment.

Case Report: Susceptibility to viral infections and secondary hemophagocytic lymphohistiocytosis responsive to intravenous immunoglobulin as primary manifestations of adenosine deaminase 2 deficiency.

Break down the barriers of auto-inflammation: How to deal with a monogenic auto-inflammatory disease and immuno-haematological features in 2022?

Allogeneic Hematopoietic Cell Transplantation for Patients With Deficiency of Adenosine Deaminase 2 (DADA2): Approaches, Obstacles and Special Considerations.

Adenosine Deaminase 2 Deficiency (DADA2): A Crosstalk Between Innate and Adaptive Immunity.

Case Report: Deficiency of Adenosine Deaminase 2 (DADA2) as a Cause of Brainstem Stroke in a 3-Year-Old Girl and the Importance of Early Fast-Track Genetic Diagnostics to Influence Therapy.

Deficiency of Adenosine Deaminase 2 Presenting as Periodic Fever at the Mainland of Familial Mediterranean Fever.

Unveiling Deficiency of Adenosine Deaminase 2: An Adult Patient With Recurrent Strokes, Vasculitic Ulcers, and Bowel Perforation.

Clinical presentation of children with Deficiency of Adenosine deaminase 2: A case series.

Evaluation of subclinical ocular involvement in patients with deficiency of adenosine deaminase 2 (DADA2).

Early onset is an indication of the severity of DADA2 disease.

Young Stroke and Systemic Manifestations: Deficiency of Adenosine Deaminase-2 (DADA-2).

Importance of the determination of enzymatic activity in the diagnosis of deficiency of adenosine deaminase 2 (DADA2).

Mechanisms of vascular inflammation in deficiency of adenosine deaminase 2 (DADA2).

The Spectrum of the Deficiency of Adenosine Deaminase 2: An Observational Analysis of a 60 Patient Cohort.

A Case of Deficiency of Adenosine Deaminase 2: 28 years of Diagnostic Challenges.

Novel Adenosine Deaminase 2 (ADA2) Mutations Associated With Hematological Manifestations.

TNF inhibition in vasculitis management in adenosine deaminase 2 deficiency (DADA2).

[Autoinflammation-differences between children and adults].

Single-cell profiling of T lymphocytes in deficiency of adenosine deaminase 2.

Case Report: Deficiency of Adenosine Deaminase 2 Presenting With Overlapping Features of Autoimmune Lymphoproliferative Syndrome and Bone Marrow Failure.

Internuclear Ophthalmoplegia in a Child With Deficiency of Adenosine Deaminase 2.

DADA2 diagnosed in adulthood versus childhood: A comparative study on 306 patients including a systematic literature review and 12 French cases.

Treatment and Outcome in Deficiency of Adenosine Deaminase 2: A Literature Review.

Deficiency of adenosine deaminase 2 (DADA2): two cases of multisystem vasculitis managed in a South Indian tertiary care centre.

Hematopoietic Cell Transplantation Cures Adenosine Deaminase 2 Deficiency: Report on 30 Patients.

Deficiency of Adenosine Deaminase 2-a Monogenic Cause of Wunderlich Syndrome.

A Novel Variant of Adenosine Deaminase 2 Deficiency Presented With Chronic Thrombocytopenia, Anemia, and Early-Onset Stroke.

Vasculitic peripheral neuropathy in deficiency of adenosine deaminase 2.

Enzyme activity in dried blood spot as a diagnostic tool for adenosine deaminase 2 deficiency.

The role of vascular inflammation markers in deficiency of adenosine deaminase 2.

Deficiency of Adenosine Deaminase 2 (DADA2): One Disease, Several Faces.

Comprehensive analysis of ADA2 genetic variants and estimation of carrier frequency driven by a function-based approach.

Case Report: An Adult Patient With Deficiency of Adenosine Deaminase 2 Resembled Unilateral Frosted Branch Angiitis.

Analysis of deficiency of adenosine deaminase 2 pathogenesis based on single-cell RNA sequencing of monocytes.

[Corrigendum] Role of adenosine deaminase 2 gene variants in pediatric deficiency of adenosine deaminase 2: A structural biological approach.

Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa.

Systematic review of childhood-onset polyarteritis nodosa and DADA2.

Spectrum of Systemic Auto-Inflammatory Diseases in India: A Multi-Centric Experience.

Expanding spectrum of DADA2: a review of phenotypes, genetics, pathogenesis and treatment.

Diagnosis and management of adenosine deaminase 2 deficiency children: the experience from China.

Adenosine Deaminase Type II Deficiency: Severe Chronic Neutropenia, Lymphoid Infiltration in Bone Marrow, and Inflammatory Features.

Deficiency of adenosine deaminase 2 diagnosed at 65 years of age.

Adult-onset deficiency of adenosine deaminase 2-a case report and literature review.

Systematic evaluation of nine monogenic autoinflammatory diseases reveals common and disease-specific correlations with allergy-associated features.

Clinical features and outcomes of childhood polyarteritis nodosa: A single referral center experience.

Detailed analysis of Japanese patients with adenosine deaminase 2 deficiency reveals characteristic elevation of type II interferon signature and STAT1 hyperactivation.

Novel ADA2 Compound Heterozygous Mutations Resulting in Deficiency of Adenosine Deaminase 2 in a Pair of Siblings.

Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments.

Anti-tumour necrosis factor treatment for the prevention of ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2).

Sequence-Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in ADA2.

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

The Many Faces of a Monogenic Autoinflammatory Disease: Adenosine Deaminase 2 Deficiency.

Deficiency of Adenosine Deaminase 2 (DADA2) - A New Autoinflammatory Disease with Multisystem Features.

Growth Parameters of Turkish Children With an Autoinflammatory Disease Before and After Canakinumab Treatment.

Successful treatment with cyclosporine and anti-tumour necrosis factor agent for deficiency of adenosine deaminase-2.

Deficiency of Adenosine Deaminase 2 (DADA2): Hidden Variants, Reduced Penetrance, and Unusual Inheritance.

Two cases of ADA2 deficiency presenting as childhood polyarteritis nodosa: novel ADA2 variant, atypical CNS manifestations, and literature review.

[Research progress in deficiency of adenosine deaminase 2].

[Treatment of deficiency of adenosine deaminase 2 caused by CECR1 mutation with myeloablative hematopoietic stem cell transplantation].

Deficiency of adenosine deaminase 2: Is it an elephant after all?

Clinical and pathological dermatological features of deficiency of adenosine deaminase 2: A multicenter, retrospective, observational study.

Polyarteritis nodosa and deficiency of adenosine deaminase 2 - Shared genealogy, generations apart.

Ischemic Stroke in Young Adults.

Role of adenosine deaminase 2 gene variants in pediatric deficiency of adenosine deaminase 2: A structural biological approach.

Genotype and functional correlates of disease phenotype in deficiency of adenosine deaminase 2 (DADA2).

Atypical phenotype of an old disease or typical phenotype of a new disease: deficiency of adenosine deaminase 2.

A monogenic autoinflammatory disease with fatal vasculitis: deficiency of adenosine deaminase 2.

The same mutation in a family with adenosine deaminase 2 deficiency.

A homozygote novel L451W mutation in CECR1 gene causes deficiency of adenosine deaminase 2 in a pediatric patient representing with chronic lymphoproliferation and cytopenia.

Deficiency of adenosine deaminase 2: a case series revealing clinical manifestations, genotypes and treatment outcomes from Turkey.

Testicular ischemia in deficiency of adenosine deaminase 2 (DADA2).

A Monogenic Disease with a Variety of Phenotypes: Deficiency of Adenosine Deaminase 2.

Deficiency of adenosine deaminase 2 triggers adenosine-mediated NETosis and TNF production in patients with DADA2.

Identification of Novel Adenosine Deaminase 2 Gene Variants and Varied Clinical Phenotype in Pediatric Vasculitis.

Treatment Strategies for Deficiency of Adenosine Deaminase 2.

Novel ADA2 mutation presenting with neutropenia, lymphopenia and bone marrow failure in patients with deficiency in adenosine deaminase 2 (DADA2).

Diagnostic utility of a targeted next-generation sequencing gene panel in the clinical suspicion of systemic autoinflammatory diseases: a multi-center study.

Adenosine Deaminase Two and Immunoglobulin M Accurately Differentiate Adult Sneddon's Syndrome of Unknown Cause.

A Chinese DADA2 patient: report of two novel mutations and successful HSCT.

Vasculitis in Systemic Autoinflammatory Diseases.

What's new in autoinflammation?

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2.

Renal Amyloidosis in Deficiency of Adenosine Deaminase 2: Successful Experience With Canakinumab.

Novel CECR1 gene mutations causing deficiency of adenosine deaminase 2, mimicking antiphospholipid syndrome.

A misleading case of deficiency of adenosine deaminase 2 (DADA2): the magnifying glass of the scientific knowledge drives the tailored medicine in real life.

ADA2 Deficiency Mimicking Idiopathic Multicentric Castleman Disease.

A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts.

Deficiency of Adenosine Deaminase 2 in Adult Siblings: Many Years of a Misdiagnosed Disease With Severe Consequences.

Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment.

Disrupted N-linked glycosylation as a disease mechanism in deficiency of ADA2.

Deficiency of adenosine deaminase 2; special focus on central nervous system imaging.

Periodic fever syndromes.

A decision tree for the genetic diagnosis of deficiency of adenosine deaminase 2 (DADA2): a French reference centres experience.

Diagnosis of deficiency of adenosine deaminase 2 with early onset polyarteritis nodosa in an adult patient with a novel compound heterozygous CECR1 mutation.

Hematologic Manifestations of Deficiency of Adenosine Deaminase 2 (DADA2) and Response to Tumor Necrosis Factor Inhibition in DADA2-Associated Bone Marrow Failure.

[Multiple facets of ADA2 deficiency: Vasculitis, auto-inflammatory disease and immunodeficiency: A literature review of 135 cases from literature].

Vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes.

Deficiency of Adenosine Deaminase 2 (DADA2), an Inherited Cause of Polyarteritis Nodosa and a Mimic of Other Systemic Rheumatologic Disorders.

Hematopoietic stem cell transplantation rescues the hematological, immunological, and vascular phenotype in DADA2.

Successful reduced intensity hematopoietic cell transplant in a patient with deficiency of adenosine deaminase 2.

Recent advances in childhood vasculitis.

Screening of 181 Patients With Antibody Deficiency for Deficiency of Adenosine Deaminase 2 Sheds New Light on the Disease in Adulthood.

Refractory Pure Red Cell Aplasia Manifesting as Deficiency of Adenosine Deaminase 2.

Stroke as Initial Manifestation of Adenosine Deaminase 2 Deficiency.

Monogenic polyarteritis: the lesson of ADA2 deficiency.

Early-onset stroke, polyarteritis nodosa (PAN), and livedo racemosa.

Old Dogs, New Tricks: Monogenic Autoinflammatory Disease Unleashed.