Preview

Translational Medicine

Advanced search

Infectious Complications in Systemic Scleroderma. Analysis of Two Autopsy Cases

https://doi.org/10.18705/2311-4495-2026-13-1-117-126

EDN: LUHTBR

Abstract

The article presents two autopsy cases of systemic scleroderma that led to the death of patients, as well as a review of the literature on the etiology and pathogenesis of the disease, with a focus on the development of infectious complications in these cases. Scleroderma is a rare connective tissue disorder, and one of the most common causes of death is infectious complications, including sepsis. The aim of this study was to analyze two clinical cases of systemic scleroderma that resulted in the death of patients. Materials and methods. The results of autopsies of two patients with a diagnosis of systemic sclerosis are presented: a 63-year-old woman and a 59-yearold man. In each case, an autopsy was performed with a macroscopic examination of all organs and tissues. For histological examination, in case 1, samples were taken from the lungs, heart, liver, kidneys, skin, esophagus, small and large intestines, right lumbar muscle, and bodies of the I and III lumbar vertebrae; in case 2, samples were taken from the lungs, heart, liver, kidneys, skin, and esophagus.

About the Authors

Ksenia A. Guseva
Federal State Budgetary Institution “V. A. Almazov National Medical Research Centre” of the Ministry of Health of the Russian Federation
Russian Federation

Ksenia A. Guseva — Postgraduate student of the Department of Pathological Anatomy with a clinic,

2, Akkuratova str., St. Petersburg, 197341.


Competing Interests:

The authors declares no conflict of interest.



Ekaterina A. Goncharova
Federal State Budgetary Institution “V. A. Almazov National Medical Research Centre” of the Ministry of Health of the Russian Federation
Russian Federation

Ekaterina A. Goncharova — Pathologist,

St. Petersburg.


Competing Interests:

The authors declares no conflict of interest.



Irina A. Danilova
Federal State Budgetary Institution “V. A. Almazov National Medical Research Centre” of the Ministry of Health of the Russian Federation
Russian Federation

Irina A. Danilova — MD, DSc, Professor, Professor of the department of pathological anatomy with a clinic, pathologist of the highest category, 

St. Petersburg.


Competing Interests:

The authors declares no conflict of interest.



Irina V. Antonova
Federal State Budgetary Institution “V. A. Almazov National Medical Research Centre” of the Ministry of Health of the Russian Federation
Russian Federation

Irina V. Antonova — MD, PhD, Associate Professor, Associate Professor of the department of pathological anatomy with a clinic, pathologist of the highest category, 

St. Petersburg.


Competing Interests:

The authors declares no conflict of interest.



References

1. Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304‒318. https://doi.org/10.1016/S0140-6736(22)01692-0

2. Adigun R, Goyal A, Hariz A. Systemic sclerosis (scleroderma). In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2024.

3. Shostak NA, Klimenko AA. Systemic sclerosis: actual classification and management. Medical practice. 2009;4:1‒12 (In Russ).

4. Ingegnoli F, Ugi N, Mihai S. Update on the epidemiology, risk factors and outcomes of systemic sclerosis. Best Practice & research: clinical rheumatology. 2018;32(2):223–240. https://doi.org/10.1016/j.berh.2018.08.005

5. Li X, Qian YQ, Liu N, et al. Survival rate, causes of death, and risk factors in systemic sclerosis: a large cohort study. Clinical Rheumatology. 2018;37(11):3051‒3056. https://doi.org/10.1007/s10067-018-4291-z

6. Potera J, Manadan AM. Reasons for hospitalization and in-hospital mortality in adults with systemic sclerosis: Analysis of the National Inpatient Sample. Journal of Scleroderma and Related Disorders. 2022;7(3):189‒196. https://doi.org/10.1177/23971983221083225

7. Shenavandeh S, Naseri R. Assessment of hospitalization and mortality of scleroderma in-patients: a thirteen-year study. Reumatologia. 2017;55(4):163‒168. https://doi.org/10.5114/reum.2017.69776

8. Voytsekhovskiy VV, Goborov ND, Pogrebnaya MV, et al. Lung damage in systemic scleroderma. Bulletin of physiology and pathology of respiration. 2018;70:83‒92 (In Russ.). https://doi.org/10.12737/article_5c12717d12a437.30758301

9. Bulavko YaE, Timofeev EV, Alkak KDYu, Isakov VA. Scleroderma Nephropathy: Unresolved Issues. Juvenis scientia. 2021;7(5):5‒18 (In Russ.).

10. Györfi AH, Filla T, Polzin A, et al. EUSTAR collaborators. Evaluation of systemic sclerosis primary heart involvement and chronic heart failure in the European scleroderma trials and research cohort. Journal of the American Heart Association. 2025;14(5):e036730. https://doi.org/10.1161/JAHA.124.036730

11. Frieri M, Angadi C, Paolano A, et al. Altered T cell subpopulations and lymphocytes expressing natural killer cell phenotypes in patients with progressive systemic sclerosis. The Journal of Allergy and Clinical Immunology. 1991;87(4):773‒779. https://doi.org/10.1016/0091-6749(91)90121-4

12. Almeida I, Silva SV, Fonseca AR, et al. T and NK cell phenotypic abnormalities in systemic sclerosis: a cohort study and a comprehensive literature review. Clinical Reviews in Allergy & Immunology. 2015;49(3):347‒369. https://doi.org/10.1007/s12016-015-8505-8

13. Simon D, Balogh P, Bognár A, et al. Reduced nonswitched memory B cell subsets cause imbalance in B cell repertoire in systemic sclerosis. Clinical and Experimental Rheumatology. 2016;100(5):30‒36.

14. Ram Poudel D, George M, Dhital R, et al. Mortality, length of stay and cost of hospitalization among patients with systemic sclerosis: results from the National Inpatient Sample. Rheumatology. 2018;9:1611–1622. https://doi.org/10.1093/rheumatology/key150

15. Czömpöly T, Simon D, Czirják L, Németh P. Anti-topoisomerase I autoantibodies in systemic sclerosis. Autoimmunity Reviews. 2009;8(8):692‒696. https://doi.org/10.1016/j.autrev.2009.02.018

16. Lipatov KV, Gostishchev VK, Asatryan AG, et al. Surgical treatment of the lower extremity extensive purulent and necrotic soft tissue lesions in a patient with systemic scleroderma. Surgery news. 2019;27(6):716‒722 (In Russ). https://doi.org/10.18484/2305-0047.2019.6.716

17. Giuggioli D, Manfredi A, Colaci M, et al. Osteomyelitis complicating scleroderma digital ulcers. Clinical Rheumatology. 2013;32(5):623‒27. https://doi.org/10.1007/s10067-012-2161-7


Review

For citations:


Guseva K.A., Goncharova E.A., Danilova I.A., Antonova I.V. Infectious Complications in Systemic Scleroderma. Analysis of Two Autopsy Cases. Translational Medicine. 2026;13(1):117-126. (In Russ.) https://doi.org/10.18705/2311-4495-2026-13-1-117-126. EDN: LUHTBR

Views: 207

JATS XML


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2311-4495 (Print)
ISSN 2410-5155 (Online)