Epidemiology and geographic clustering of Erdheim-Chester disease in Italy and France

Francesco Peyronel; Julien Haroche; Corrado Campochiaro; Francesco Pegoraro; Zahir Amoura; Alessandro Tomelleri; Martina Mazzariol; Matthias Papo; Giulio Cavalli; Giuseppe D Benigno; Paride Fenaroli; Chrysanthos Grigoratos; Maria C Mengoli; Arturo Bonometti; Emilio Berti; Gustavo Savino; Mauro Cives; Iria Neri; Gaetano Pacinella; Antonino Tuttolomondo; Massimo Marano; Francesco Muratore; Andreina Manfredi; Alessandro Broccoli; Pier L Zinzani; Biagio Didona; Chiara Massaccesi; Andrea Buono; Enrico Ammirati; Vito Di Lernia; Lorenzo Dagna; Augusto Vaglio; Fleur Cohen-Aubart

doi:10.1182/blood.2023021670

Epidemiology and geographic clustering of Erdheim-Chester disease in Italy and France

Blood. 2023 Dec 14;142(24):2119-2123. doi: 10.1182/blood.2023021670.

Authors

Francesco Peyronel^{1

2}, Julien Haroche³, Corrado Campochiaro^{4

5}, Francesco Pegoraro⁶, Zahir Amoura³, Alessandro Tomelleri^{4

5}, Martina Mazzariol⁷, Matthias Papo³, Giulio Cavalli⁴, Giuseppe D Benigno⁸, Paride Fenaroli⁹, Chrysanthos Grigoratos¹⁰, Maria C Mengoli¹¹, Arturo Bonometti^{12

13}, Emilio Berti¹⁴, Gustavo Savino^{15

16}, Mauro Cives^{17

18}, Iria Neri¹⁹, Gaetano Pacinella²⁰, Antonino Tuttolomondo²⁰, Massimo Marano^{21

22}, Francesco Muratore²³, Andreina Manfredi²⁴, Alessandro Broccoli^{25

26}, Pier L Zinzani^{25

26}, Biagio Didona²⁷, Chiara Massaccesi²⁸, Andrea Buono²⁹, Enrico Ammirati^{29

30}, Vito Di Lernia³¹, Lorenzo Dagna^{4

5}, Augusto Vaglio^{1

32}, Fleur Cohen-Aubart³

Affiliations

¹ Nephrology and Dialysis Unit, Azienda Ospedaliera Universitaria Meyer IRCCS, Florence, Italy.
² Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
³ Sorbonne University, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Internal Medicine Department 2, French National Referral Center for Rare Systemic Diseases and Histiocytoses, Centre d'immunologie et des maladies infectieuses (Cimi INSERM Unité Mixte de Recherche Scientifique-1135), Paris, France.
⁴ Unit of Immunology, Rheumatology, Allergy and Rare Diseases, Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute, Milan, Italy.
⁵ Vita-Salute San Raffaele University, Milan, Italy.
⁶ Department of Health Science, University of Florence, Florence, Italy.
⁷ Department of Medical Sciences, University of Turin, Turin, Italy.
⁸ Renal Unit, Parma University Hospital, Parma, Italy.
⁹ Nephrology and Dialysis Unit, Azienda Unità Sanitaria Locale-Istituto di Ricovero e Cura a Carattere Scientifico Reggio Emilia, Reggio Emilia, Italy.
¹⁰ Cardiology Division, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.
¹¹ Operative Unit of Pathology, Azienda Unità Sanitaria Locale-Istituto di Ricovero e Cura a Carattere Scientifico Reggio Emilia, Reggio Emilia, Italy.
¹² Pathology Unit, Istituto di Ricovero e Cura a Carattere Scientifico Humanitas Clinical and Research Hospital, Rozzano, Italy.
¹³ Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy.
¹⁴ Dermatology Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
¹⁵ Ocular Oncology Unit, Fondazione Policlinico Universitario A. Gemelli Istituto di Ricovero e Cura a Carattere Scientifico, Rome, Italy.
¹⁶ Università Cattolica del Sacro Cuore, UCSC, Rome, Italy.
¹⁷ Division of Medical Oncology, Azienda Ospedaliero-Universitaria Consorziale Policlinico di Bari, Bari, Italy.
¹⁸ Department of Interdisciplinary Medicine, University of Bari "Aldo Moro," Bari, Italy.
¹⁹ Dermatology Unit, Istituto di Ricovero e Cura a Carattere Scientifico Azienda Ospedaliero Universitaria di Bologna, Policlinico S. Orsola-Malpighi, Bologna, Italy.
²⁰ Internal Medicine and Stroke Care Ward, Department of Promoting Health, Maternal-Infant, Excellence and Internal and Specialized Medicine G. D'Alessandro, University of Palermo, Palermo, Italy.
²¹ Unit of Neurology, Neurophysiology, Neurobiology and Psychiatry, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome, Italy.
²² Fondazione Policlinico Universitario Campus Bio-Medico, Rome, Italy.
²³ Rheumatology Unit, Azienda Unità Sanitaria Locale-Istituto di Ricovero e Cura a Carattere Scientifico di Reggio Emilia, University of Modena and Reggio Emilia, Reggio Emilia, Italy.
²⁴ Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Policlinico of Modena, Modena, Italy.
²⁵ Istituto di Ricovero e Cura a Carattere Scientifico Azienda Ospedaliero-Universitaria di Bologna Istituto di Ematologia "Seràgnoli", Bologna, Italy.
²⁶ Dipartimento di Scienze Mediche e Chirurgiche, Università di Bologna, Bologna, Italy.
²⁷ Istituto Dermopatico dell'Immacolata-Istituto di Ricovero e Cura a Carattere Scientifico Rare Disease Center, Rome, Italy.
²⁸ Pulmonology Unit, "C. & G. Mazzoni" Hospital, Ascoli Piceno, Italy.
²⁹ De Gasperis Cardio Center, Transplant Center, Niguarda Hospital, Milan, Italy.
³⁰ Department of Health Sciences, University of Milan-Bicocca, Monza, Italy.
³¹ Dermatology Unit, S. Maria Nuova, Azienda Unità Sanitaria Locale-Istituto di Ricovero e Cura a Carattere Scientifico Reggio Emilia, Italy.
³² Department of Biomedical, Experimental and Clinical Sciences "Mario Serio," University of Florence, Florence, Italy.

PMID: 37871575
DOI: 10.1182/blood.2023021670

Abstract

This geoepidemiological study, performed in Italy and France, shows that Erdheim-Chester disease is increasingly diagnosed and cases cluster in specific geographic areas, namely southern Italy and central France. Disease frequency inversely correlates with the Human Development Index.

MeSH terms

Erdheim-Chester Disease* / diagnostic imaging
Erdheim-Chester Disease* / epidemiology
France / epidemiology
Humans
Italy / epidemiology