Development of the doctrine of auto-inflammatory diseases in the XXI century

Фёдоров, Е. С.; Салугина, С. О.; Кузьмина, Н. Н.

doi:10.14412/1995-4484-2018-5-18

Naučno-praktičeskaâ revmatologiâ

2019

DOI: 10.14412/1995-4484-2018-5-18

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Development of the doctrine of auto-inflammatory diseases in the XXI century

Е. С. Фёдоров¹,

С. О. Салугина²,

Н. Н. Кузьмина³

Abstract: The article presents the development of the doctrine of autoinflammatory diseases (AID) in the last decade. Data on interleukin 1, inflammasomes and their role in the development of AID are presented. The paper also contains the data about interferonopathies and, in particular, proteasomal illnesses as a new class of hereditary autoinflammatory diseases. Variety of AID variants, including diseases with the dominance of one system damage (skin, intestines, bones) is shown. Advances in the AID diagnosis and cour… Show more

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Cited by 9 publications

References 77 publications

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Monogenic auto-inflammatory diseases in children and adults: what a rheumatologist should know

Салугина¹,

Федоров²,

Agafonova³

2019

Naučno-praktičeskaâ revmatologiâ

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The article presents information on the current state of the problem of autoinflammatory diseases (AID) in rheumatology, reflects the clinical and demographic, laboratory and molecular genetic characteristics of the main monogenic AID (mAID), the most common in real clinical practice. Approaches to the diagnosis and differential diagnosis of these conditions are presented. The variants of classification and diagnostic criteria of the most studied mAID are discussed. The principles of treatment with an emphasis on the use of targeted therapy with interleukin 1 (IL1) inhibitors are shown. The clinical examples demonstrate the variants of late diagnosis of the mAID in adult patients with the disease onset in childhood. Different variants of multi-organ damage are considered. Effectiveness of therapy with IL1β inhibitor kanakinumab is demonstrated. Even in such cases it provided the possibility of significant symptoms regression and restoration of a satisfactory quality of life.

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Monogenic auto-inflammatory diseases in children and adults: what a rheumatologist should know

Салугина¹,

Федоров²,

Agafonova³

2019

Naučno-praktičeskaâ revmatologiâ

Self Cite

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Abdominal syndrome in monogenic autoinflammatory disease – is it just a familial Mediterranean fever?

Олеговна¹,

Федоров²,

Volf

2020

Sovremennaâ revmatologiâ

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Gastrointestinal (GI) manifestations, such as abdominal pain, nausea, vomiting, and diarrhea, are common autoinflammatory disease (AID) symptoms. The abdominal symptomatology reflecting serositis is one of the most important classification and diagnostic criteria for the classic monogenic AID (MAID) – familial Mediterranean fever (FMF). Failure to timely diagnose FMF frequently leads to unjustified surgical interventions. Other periodic fevers may also present as abdominal symptoms; however, the latter are outside their diagnostic features. These diseases include, first of all, tumor necrosis factor receptor-associated periodic syndrome (TRAPS). Interleukin 1 (IL1) inhibitors serve as the major targeted drugs for the treatment of TRAPS. Russia has registered the IL1 inhibitor canakinumab that prevents the development of organ damages, including those in the GI tract. The paper describes a clinical case of the classic manifestations of TRAPS (fever, rash, periorbital edema, arthritis, and elevated levels of acutephase inflammatory markers) concurrent with severe abdominalgia during attacks and with the development of severe peritoneal adhesions, which led to bowel perforation and emergency surgical intervention. The prolonged persistence of inflammatory attacks before the initiation of therapy, as well as violation of the IL1 inhibitor administration regimen facilitated the development of an urgent exacerbation. Thus, TRAPS should be included in the differential diagnostic circle for patients with severe gastrointestinal manifestations characterized by an attack-like course. These patients need timely prescription of targeted therapy, strict adherence to the dosing and intervals between drug administrations, and careful monitoring to prevent serious complications with the visceral organs, including the gastrointestinal tract, and their immediate correction.

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Clinical and histological variants of panniculitis in children

Егорова¹,

Тарасова²,

Datsina

2022

Sovremennaâ revmatologiâ

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The article discusses the clinical and diagnostic difficulties in verifying of panniculitis variants in children of different ages, including newborns. Successful diagnosis of the disease depends on a carefully collected anamnesis indicating information about previous diseases, background pathology, medications, as well as an adequate assessment of clinical symptoms, laboratory parameters and identification of typical morphological changes.

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Development of the doctrine of auto-inflammatory diseases in the XXI century

Cited by 9 publications

References 77 publications

Monogenic auto-inflammatory diseases in children and adults: what a rheumatologist should know

Monogenic auto-inflammatory diseases in children and adults: what a rheumatologist should know

Abdominal syndrome in monogenic autoinflammatory disease – is it just a familial Mediterranean fever?

Clinical and histological variants of panniculitis in children

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