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.

Publication types

Letter

MeSH terms

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