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Macrophage activation syndrome during pregnancy as a fatal complication of systemic idiopathic arthritis

https://doi.org/10.17749/2313-7347/ob.gyn.rep.2024.498

Abstract

According to the current concept, juvenile arthritis with a systemic onset (systemic juvenile idiopathic arthritis, sJIA) and adult­onset Still's disease (ASD) are considered as rare autoinflammatory diseases, with the underlying immunopathogenesis being based on the mechanisms bridging both of such diseases. Despite they develop in childhood and young age, the issues of reproduction occurring during these pathologies virtually unexplored. The article presents a clinical case of maternal death in a 22-year-old primiparous woman and analyzes the tactics of sJIA management, which was complicated by pregnancy-related macrophage activation syndrome.

About the Authors

P. A. Shesternya
Professor V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Health Ministry of Russian Federation
Russian Federation

Pavel A. Shesternya. MD, Dr Sci Med, Prof. 

WoS ResearcherID: M-3898-2014

1 Partizana Zheleznyaka Str., Krasnoyarsk 660022



V. B. Tskhay
Professor V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Health Ministry of Russian Federation
Russian Federation

Vitaly B. Tskhay, MD, Dr Sci Med, Prof. 

WoS ResearcherID: C-4869-2014

1 Partizana Zheleznyaka Str., Krasnoyarsk 660022



M. I. Bazina
Professor V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Health Ministry of Russian Federation
Russian Federation

Marina I. Bazina, MD, Dr Sci Med.

1 Partizana Zheleznyaka Str., Krasnoyarsk 660022



I. V. Matveeva
Krasnoyarsk Regional Clinical Hospital
Russian Federation

Irina V. Matveeva, MD.

3 Partizana Zheleznyaka Str., Krasnoyarsk 660022



References

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What is already known about this subject?

► For a long time, continuity of therapy for patients with systemic juvenile idiopathic arthritis (sJIA) upon transition to the adult healthcare remained a serious problem, because most often it was accompanied by a change in diagnosis.

► Issues of reproduction in sJIA remain extremely poorly studied.

► The widespread introduction of anticytokine biological therapy into clinical practice is accompanied by new interdisciplinary tasks.

What are the new findings?

► The development of macrophage activation syndrome (MAS) during pregnancy is one of the most serious and potentially fatal complications.

► Treatment of MAS requires active and careful monitoring by entire interdisciplinary team.

How might it impact on clinical practice in the foreseeable future?

► It is necessary to focus on alertness of doctors from various specialties on the features of sJIA and MAS diagnostics and management while planning preconception preparation.

Review

For citations:


Shesternya P.A., Tskhay V.B., Bazina M.I., Matveeva I.V. Macrophage activation syndrome during pregnancy as a fatal complication of systemic idiopathic arthritis. Obstetrics, Gynecology and Reproduction. 2024;18(6):898-907. (In Russ.) https://doi.org/10.17749/2313-7347/ob.gyn.rep.2024.498

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ISSN 2313-7347 (Print)
ISSN 2500-3194 (Online)