Research Topic

Exploring the Link between Autoinflammatory Diseases and Vasculitis

About this Research Topic

Autoinflammatory diseases (AID) are diseases caused by impaired innate immune function, characterized by recurrent episodes of local or systemic inflammation in the absence of autoantibodies or infections. Vasculitis is inflammation and necrosis of the blood vessels causing impaired blood flow, ischemia, and infarction of the dependent tissues and vasculitis.

Familial Mediterranean fever (FMF), the most common monogenic AID, is frequently associated with IgA-associated vasculitis (IgAV) and polyarteritis nodosa (PAN) involving small and/or medium-sized vessels. In addition, Haploinsufficiency of A20 (also called "Familial Behcet-like Autoinflammatory Syndrome") has a phenotype very similar to that of variable vasculitis of Behcet's disease with recurrent oral-genital ulcers, in addition to, skin rash, uveitis, and polyarthritis. Accordingly, AID may be associated with vasculitis, and the cause of the association between vasculitis and monogenic AID remains controversial.

The aim of this Research Topic is to better understand the following components of vasculitis caused by autoinflammatory diseases:

• epidemiology
• pathogenesis
• genetics
• clinical manifestations
• diagnostic approach
• treatment of vasculitis in autoinflammatory disease
• immunologic mechanisms

The scope in this Research Topic covers clinical, genetic, and immunological features of vasculitis and vasculitis-like manifestations in autoinflammatory syndrome. Autoinflammatory diseases of interest include but are not limited to:

• Familial Mediterranean fever
• Cryopyrin-Associated Periodic Fever Syndrome (CAPS)
• Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
• Mevalonate Kinase Deficiency (MKD) - also known as Hyper IgD syndrome (HIDS)
• Deficiency of IL-1 Receptor Antagonist (DIRA) and Pyogenic Arthritis
• Pyroderma gangrenosum, and Acne (PAPA)
• Deficiency of Adenosine Deaminase 2 (DADA2)
• Haploinsufficiency of A 20
• Interferonopathies such as STING-associated vasculopathy with onset in infancy (SAVI).


Keywords: autoinflammatory diseases, vasclitis, familial Mediterranean fever, Deficiency of Adenosine Deaminase 2


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

Autoinflammatory diseases (AID) are diseases caused by impaired innate immune function, characterized by recurrent episodes of local or systemic inflammation in the absence of autoantibodies or infections. Vasculitis is inflammation and necrosis of the blood vessels causing impaired blood flow, ischemia, and infarction of the dependent tissues and vasculitis.

Familial Mediterranean fever (FMF), the most common monogenic AID, is frequently associated with IgA-associated vasculitis (IgAV) and polyarteritis nodosa (PAN) involving small and/or medium-sized vessels. In addition, Haploinsufficiency of A20 (also called "Familial Behcet-like Autoinflammatory Syndrome") has a phenotype very similar to that of variable vasculitis of Behcet's disease with recurrent oral-genital ulcers, in addition to, skin rash, uveitis, and polyarthritis. Accordingly, AID may be associated with vasculitis, and the cause of the association between vasculitis and monogenic AID remains controversial.

The aim of this Research Topic is to better understand the following components of vasculitis caused by autoinflammatory diseases:

• epidemiology
• pathogenesis
• genetics
• clinical manifestations
• diagnostic approach
• treatment of vasculitis in autoinflammatory disease
• immunologic mechanisms

The scope in this Research Topic covers clinical, genetic, and immunological features of vasculitis and vasculitis-like manifestations in autoinflammatory syndrome. Autoinflammatory diseases of interest include but are not limited to:

• Familial Mediterranean fever
• Cryopyrin-Associated Periodic Fever Syndrome (CAPS)
• Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
• Mevalonate Kinase Deficiency (MKD) - also known as Hyper IgD syndrome (HIDS)
• Deficiency of IL-1 Receptor Antagonist (DIRA) and Pyogenic Arthritis
• Pyroderma gangrenosum, and Acne (PAPA)
• Deficiency of Adenosine Deaminase 2 (DADA2)
• Haploinsufficiency of A 20
• Interferonopathies such as STING-associated vasculopathy with onset in infancy (SAVI).


Keywords: autoinflammatory diseases, vasclitis, familial Mediterranean fever, Deficiency of Adenosine Deaminase 2


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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Submission Deadlines

07 November 2020 Abstract
07 March 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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Topic Editors

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Submission Deadlines

07 November 2020 Abstract
07 March 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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