Autoimmune markers and vascular immune deposits in Finkelstein-Seidlmayer vasculitis

Bronz, Gabriel; Gianini, Jvan; Passi,  Alberto G.; Rizzi, Mattia; Bergmann, Marcel M.; Milani, Gregorio P.; Lava, Sebastiano A. G.; Bianchetti, Mario G; Terziroli Beretta-Piccoli, Benedetta; Vanoni, Federica

doi:10.1016/j.jaut.2023.103002

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Journal article

Autoimmune markers and vascular immune deposits in Finkelstein-Seidlmayer vasculitis : systematic literature review

Bronz, Gabriel Pediatric Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland
Gianini, Jvan Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Switzerland - Department of Medicine and Surgery, University of Insubria, Varese, Italia
Passi, Alberto G. Department of Medicine and Surgery, University of Insubria, Varese, Italia
Rizzi, Mattia ORCID Pediatric Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
Bergmann, Marcel M. Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Centro Pediatrico Del Mendrisiotto, Mendrisio, Switzerland - Pediatric Allergy Unit, Department of Woman, Child and Adolescent, University Hospitals of Geneva, Geneva, Switzerland
Milani, Gregorio P. ORCID Pediatric Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy - Department of Clinical Sciences and Community Health, Università Degli Studi di Milano, Milan, Italy
Lava, Sebastiano A. G. ORCID Pediatric Cardiology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland - Heart Failure and Transplantation, Department of Pediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom
Bianchetti, Mario G Institute of Family Medicine, Faculty of Biomedical Sciences, Università Della Svizzera Italiana, Lugano, Switzerland
Terziroli Beretta-Piccoli, Benedetta ORCID Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Epatocentro Ticino, Lugano, Switzerland - Faculty of Life Sciences & Medicine, King's College London, King's College Hospital, London, United Kingdom
Vanoni, Federica Pediatric Institute of Southern Switzerland, Ente Ospedaliero Cantonale, Bellinzona, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland

2023

Published in:

Journal of autoimmunity. - 2023, vol. 136, p. 103002

Finkelstein-Seidlmayer vasculitis

Acute Hemorrhagic Edema Bibliographic Database

English Finkelstein-Seidlmayer vasculitis, also called acute hemorrhagic edema of young children or infantile immunoglobulin A vasculitis, is habitually a benign skin-limited small vessel leukocytoclastic vasculitis that mainly affects infants 24 months or less of age. Since this disease is commonly triggered by an infection, an immune-mediated origin has been postulated. To better appreciate the possible underlying immune mechanism of this vasculitis, we addressed circulating autoimmune markers and vascular immune deposits in patients contained in the Acute Hemorrhagic Edema BIbliographic Database, which incorporates all original reports on Finkelstein-Seidlmayer vasculitis. A test for at least one circulating autoimmune marker or a vascular immune deposit was performed in 243 cases. Subunits of complement system C4 resulted pathologically reduced in 4.7% and C3 in 1.4%, rheumatoid factor was detected in 6.1%, and antinuclear antibodies in 1.9% of cases. Antineutrophil cytoplasmic antibodies were never demonstrated. Immunofluorescence studies were performed on 125 skin biopsy specimens and resulted positive for complement subunits in 46%, fibrinogen in 45%, immunoglobulin A in 25%, immunoglobulin M in 24%, immunoglobulin G in 13%, and immunoglobulin E in 4.2% of cases. Infants testing positive for vascular immunoglobulin A deposits did not present a higher prevalence of systemic involvement or recurrences, nor a longer disease duration. In conclusion, we detected a very low prevalence of circulating autoimmune marker positivity in Finkelstein-Seidlmayer patients. Available immunofluorescence data support the notion that immune factors play a relevant role in this vasculitis. Furthermore, vascular immunoglobulin A deposits seem not to play a crucial role in this disease.

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