eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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6/2019
vol. 57
 
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Monogenic autoinflammatory diseases in adults – a challenge to rheumatologic practice at the onset of the Polish national programme of interleukin 1 inhibitor treatment

Marcin Milchert
1
,
Joanna Makowska
2
,
Olga Brzezińska
2
,
Marek Brzosko
1
,
Ewa Więsik-Szewczyk
3

1.
Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Poland
2.
Department of Rheumatology, Medical University of Lodz, Poland
3.
Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, Warsaw, Poland
Data publikacji online: 2019/12/31
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Monogenic autoinflammatory diseases (AIDs, formerly known as hereditary periodic fever syndromes) cover a spectrum of diseases which lead to chronic or recurrent inflammation caused by activation of the innate immune system. The most common monogenic AID is familial Mediterranean fever. Monogenic autoinflammatory diseases are generally considered intracellular signalling defects. Some stereotypical knowledge may be misleading; e.g. monogenic AIDs are not exclusively found in children, family history is often negative, fever frequently is not a leading manifestation and frequency of attacks in adults is usually variable. Lack of genetic confirmation should not stop anti-inflammatory ex juvantibus therapy. The pattern of tissue injury in AIDs is basically different from that observed in autoimmunity. There is no autoaggression against organ-specific antigens, but substantial damage (amyloidosis, cachexia, premature cardiovascular disease) is secondary to long-lasting inflammation.

The Polish national programme of anti-interleukin 1 treatment opens new possibilities for the treatment. However, monogenic AIDs are frequently misdiagnosed and more awareness is needed.






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