Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study

Abstract Background Atraumatic avascular necrosis (AVN) is a severe condition that may complicate the course of rheumatic diseases and contribute to long-term damage. However, there is a lack of evidence on this rare event in pediatric rheumatology. The aim of our study was to evaluate the occurrenc...

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Main Authors: Ivan Taietti, Federico Zini, Emilio Amleto Conti, Enrica Cristini, Irene Borzani, Giulia Ramponi, Claudia Bracaglia, Raffaele Pecoraro, Riccardo Papa, Jessica Tibaldi, Serena Pastore, Gabriele Simonini, Marco Cattalini, Antonella Meini, Achille Marino, Stefano Lanni, Francesca Minoia, Giovanni Filocamo
Format: Article
Language:English
Published: BMC 2025-01-01
Series:Italian Journal of Pediatrics
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Online Access:https://doi.org/10.1186/s13052-025-01845-8
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author Ivan Taietti
Federico Zini
Emilio Amleto Conti
Enrica Cristini
Irene Borzani
Giulia Ramponi
Claudia Bracaglia
Raffaele Pecoraro
Riccardo Papa
Jessica Tibaldi
Serena Pastore
Gabriele Simonini
Marco Cattalini
Antonella Meini
Achille Marino
Stefano Lanni
Francesca Minoia
Giovanni Filocamo
author_facet Ivan Taietti
Federico Zini
Emilio Amleto Conti
Enrica Cristini
Irene Borzani
Giulia Ramponi
Claudia Bracaglia
Raffaele Pecoraro
Riccardo Papa
Jessica Tibaldi
Serena Pastore
Gabriele Simonini
Marco Cattalini
Antonella Meini
Achille Marino
Stefano Lanni
Francesca Minoia
Giovanni Filocamo
author_sort Ivan Taietti
collection DOAJ
description Abstract Background Atraumatic avascular necrosis (AVN) is a severe condition that may complicate the course of rheumatic diseases and contribute to long-term damage. However, there is a lack of evidence on this rare event in pediatric rheumatology. The aim of our study was to evaluate the occurrence of avascular necrosis in the context of rheumatologic diseases in Italy and to describe the main demographic and clinical features of AVN patients, with a particular focus on treatment background. Methods All centres part of the Italian Society of Pediatric Rheumatology were invited to participate in a retrospective case collection of children with rheumatic diseases complicated by a pediatric-onset AVN. Demographic, clinical, laboratory and imaging data were recorded, together with outcome and treatment background, particularly steroid exposure. Population collected was further evaluated according to the different underlying rheumatologic disease and to the time of AVN onset. Results Fourteen patients (SLE = 7; JIA = 4; others = 3) were collected from 7 centres. Females were predominantly affected (71%) with a median age at AVN diagnosis of 14.3 years. Multifocal involvement was mostly reported (93%), mainly involving femoral heads (44%) and knees (28%). All patients had a severe rheumatologic background and received systemic glucocorticoids with a median cumulative prednisone equivalent dose of 457.5 mg/kg. In all patients but one imaging showed persistence of abnormalities, despite the complete resolution of symptoms in 6 of them. Bisphosphonates were the most used therapeutic approach; orthopedic surgery was required in 2 cases. Conclusions Despite its rarity, AVN may be a severe complication of pediatric rheumatic diseases. Active monitoring is crucial to promptly identify patients and to prevent long-term damage. Prospective large sample studies are required to better understand the impact of steroid exposure and its complex interplay with other potential contributing factors.
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spelling doaj-art-32746df96e744f47bf62ba30c458475b2025-02-02T12:34:53ZengBMCItalian Journal of Pediatrics1824-72882025-01-015111810.1186/s13052-025-01845-8Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre studyIvan Taietti0Federico Zini1Emilio Amleto Conti2Enrica Cristini3Irene Borzani4Giulia Ramponi5Claudia Bracaglia6Raffaele Pecoraro7Riccardo Papa8Jessica Tibaldi9Serena Pastore10Gabriele Simonini11Marco Cattalini12Antonella Meini13Achille Marino14Stefano Lanni15Francesca Minoia16Giovanni Filocamo17Pediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoDepartment of Orthopaedics and Traumatology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Radiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Unit, Fondazione IRCCS San GerardoDivision of Rheumatology, IRCCS Ospedale Pediatrico Bambino GesùDivision of Rheumatology, IRCCS Ospedale Pediatrico Bambino GesùPediatric Clinic and Rheumatology, IRCCS Istituto Giannina Gaslini, University of GenoaPediatric Clinic and Rheumatology, IRCCS Istituto Giannina Gaslini, University of GenoaInstitute for Maternal and Child Health - IRCCS “Burlo Garofolo”Rheumatology Unit, ERN-ReCONNET center Meyer Children’s Hospital IRCCS, University of FlorencePediatric Clinic and Molecular Medicine Institute ‘A. Nocivelli’, University of BrixiaPediatric Clinic and Molecular Medicine Institute ‘A. Nocivelli’, University of BrixiaUnit of Pediatric Rheumatology, ASST G. Pini-CTOPediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoPediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore PoliclinicoAbstract Background Atraumatic avascular necrosis (AVN) is a severe condition that may complicate the course of rheumatic diseases and contribute to long-term damage. However, there is a lack of evidence on this rare event in pediatric rheumatology. The aim of our study was to evaluate the occurrence of avascular necrosis in the context of rheumatologic diseases in Italy and to describe the main demographic and clinical features of AVN patients, with a particular focus on treatment background. Methods All centres part of the Italian Society of Pediatric Rheumatology were invited to participate in a retrospective case collection of children with rheumatic diseases complicated by a pediatric-onset AVN. Demographic, clinical, laboratory and imaging data were recorded, together with outcome and treatment background, particularly steroid exposure. Population collected was further evaluated according to the different underlying rheumatologic disease and to the time of AVN onset. Results Fourteen patients (SLE = 7; JIA = 4; others = 3) were collected from 7 centres. Females were predominantly affected (71%) with a median age at AVN diagnosis of 14.3 years. Multifocal involvement was mostly reported (93%), mainly involving femoral heads (44%) and knees (28%). All patients had a severe rheumatologic background and received systemic glucocorticoids with a median cumulative prednisone equivalent dose of 457.5 mg/kg. In all patients but one imaging showed persistence of abnormalities, despite the complete resolution of symptoms in 6 of them. Bisphosphonates were the most used therapeutic approach; orthopedic surgery was required in 2 cases. Conclusions Despite its rarity, AVN may be a severe complication of pediatric rheumatic diseases. Active monitoring is crucial to promptly identify patients and to prevent long-term damage. Prospective large sample studies are required to better understand the impact of steroid exposure and its complex interplay with other potential contributing factors.https://doi.org/10.1186/s13052-025-01845-8Avascular necrosis (AVN)Systemic glucocorticoidsPediatric rheumatologyPediatric rheumatic diseases
spellingShingle Ivan Taietti
Federico Zini
Emilio Amleto Conti
Enrica Cristini
Irene Borzani
Giulia Ramponi
Claudia Bracaglia
Raffaele Pecoraro
Riccardo Papa
Jessica Tibaldi
Serena Pastore
Gabriele Simonini
Marco Cattalini
Antonella Meini
Achille Marino
Stefano Lanni
Francesca Minoia
Giovanni Filocamo
Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
Italian Journal of Pediatrics
Avascular necrosis (AVN)
Systemic glucocorticoids
Pediatric rheumatology
Pediatric rheumatic diseases
title Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
title_full Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
title_fullStr Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
title_full_unstemmed Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
title_short Avascular necrosis in pediatric rheumatic diseases: an Italian retrospective multicentre study
title_sort avascular necrosis in pediatric rheumatic diseases an italian retrospective multicentre study
topic Avascular necrosis (AVN)
Systemic glucocorticoids
Pediatric rheumatology
Pediatric rheumatic diseases
url https://doi.org/10.1186/s13052-025-01845-8
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