Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis

Objective Pulmonary involvement in chronic nonbacterial osteomyelitis (CNO) is rare. Limited awareness results in diagnostic challenges, especially because malignancy or infection needs to be considered. Methods Based on a survey shared among centers participating in the Kerndokumentation Deutsches...

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Main Authors: Toni Hospach, Friederike Blankenburg, Anita Heinkele, Kristina Rücklová, Peter Müller‐Abt, Frank Weller‐Heinemann, Laura Buchtala, Jan Maier, Jasmin Kümmerle‐Deschner, Christiane Reiser, Ales Janda, Andreas Urban, Reiner Berendes, Andrea Skrabl‐Baumgartner, Jürgen Brunner, Ralf Trauzeddel, Johannes Peter Haas, Annette Holl‐Wieden, Henner Morbach, Anja Schnabel, Normi Brück, Gabriele Hahn, Wolfgang Emminger, Isabella Valent, Regine Borchers, Christian Klemann, Ilias Tsiflikas, Jens Klotsche, Kirsten Minden, Christian M. Hedrich, Thekla von Kalle
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:ACR Open Rheumatology
Online Access:https://doi.org/10.1002/acr2.11799
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author Toni Hospach
Friederike Blankenburg
Anita Heinkele
Kristina Rücklová
Peter Müller‐Abt
Frank Weller‐Heinemann
Laura Buchtala
Jan Maier
Jasmin Kümmerle‐Deschner
Christiane Reiser
Ales Janda
Andreas Urban
Reiner Berendes
Andrea Skrabl‐Baumgartner
Jürgen Brunner
Ralf Trauzeddel
Johannes Peter Haas
Annette Holl‐Wieden
Henner Morbach
Anja Schnabel
Normi Brück
Gabriele Hahn
Wolfgang Emminger
Isabella Valent
Regine Borchers
Christian Klemann
Ilias Tsiflikas
Jens Klotsche
Kirsten Minden
Christian M. Hedrich
Thekla von Kalle
author_facet Toni Hospach
Friederike Blankenburg
Anita Heinkele
Kristina Rücklová
Peter Müller‐Abt
Frank Weller‐Heinemann
Laura Buchtala
Jan Maier
Jasmin Kümmerle‐Deschner
Christiane Reiser
Ales Janda
Andreas Urban
Reiner Berendes
Andrea Skrabl‐Baumgartner
Jürgen Brunner
Ralf Trauzeddel
Johannes Peter Haas
Annette Holl‐Wieden
Henner Morbach
Anja Schnabel
Normi Brück
Gabriele Hahn
Wolfgang Emminger
Isabella Valent
Regine Borchers
Christian Klemann
Ilias Tsiflikas
Jens Klotsche
Kirsten Minden
Christian M. Hedrich
Thekla von Kalle
author_sort Toni Hospach
collection DOAJ
description Objective Pulmonary involvement in chronic nonbacterial osteomyelitis (CNO) is rare. Limited awareness results in diagnostic challenges, especially because malignancy or infection needs to be considered. Methods Based on a survey shared among centers participating in the Kerndokumentation Deutsches Rheumaforschungszentrum (Germany), this study investigated clinical and imaging presentations, demographic features, treatment response and outcomes of pulmonary involvement in CNO (pCNO). Magnetic resonance imaging and computed tomography images were read centrally by an experienced pediatric radiologist. Results Twenty‐two patients with pCNO were included in this study. Among patients with CNO, pulmonary involvement was more common in girls (91% vs 62.8%, P = 0.006) and patients with multifocal bone lesions (95% vs 65%, P <0.001) but was not associated with systemic inflammation or additional organ involvement. Forty‐two pulmonary lesions were counted with a median of two per patient (two to six). They displayed a median size of 1.8 cm (0.3–4.0 cm) and followed mono‐ (40%) and oligo‐focal (60%) patterns representing consolidations or nodules, abutting the pleura in 50%. Although prominent hilar lymph nodes were present (in 19% of patients), no pathologic enlargement (>1 cm) was seen. When available (3 of 22 patients), histology revealed granulomatous inflammation with lymphocyte infiltration. Development and courses of pCNO did not associate with treatments chosen. Complete remission was reported in 60% of patients, partial remission in 20%. Conclusion pCNO is usually asymptomatic. Although more common in girls and patients with multifocal CNO, pCNO is not associated with systemic parameters of inflammation or specific organ involvement. Prognosis of pCNO is favorable, and most lesions resolve over time. Thus, a careful watch‐and‐wait strategy may be appropriate.
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spelling doaj-art-a2f2c93038ea424d8522fc9326adf4f72025-02-04T06:21:22ZengWileyACR Open Rheumatology2578-57452025-01-0171n/an/a10.1002/acr2.11799Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial OsteomyelitisToni Hospach0Friederike Blankenburg1Anita Heinkele2Kristina Rücklová3Peter Müller‐Abt4Frank Weller‐Heinemann5Laura Buchtala6Jan Maier7Jasmin Kümmerle‐Deschner8Christiane Reiser9Ales Janda10Andreas Urban11Reiner Berendes12Andrea Skrabl‐Baumgartner13Jürgen Brunner14Ralf Trauzeddel15Johannes Peter Haas16Annette Holl‐Wieden17Henner Morbach18Anja Schnabel19Normi Brück20Gabriele Hahn21Wolfgang Emminger22Isabella Valent23Regine Borchers24Christian Klemann25Ilias Tsiflikas26Jens Klotsche27Kirsten Minden28Christian M. Hedrich29Thekla von Kalle30Olgahospital Klinikum Stuttgart GermanyOlgahospital Klinikum Stuttgart GermanyOlgahospital Klinikum Stuttgart GermanyOlgahospital, Klinikum Stuttgart, Germany, and Charles University Prague Czech RepublicOlgahospital Klinikum Stuttgart GermanyProf.‐Hess‐Kinderklinik Bremen GermanyProf.‐Hess‐Kinderklinik Bremen GermanyChildren Medical Practice Leinfelden‐Echterdingen GermanyUniversity of Tübingen Tübingen GermanyUniversity of Tübingen, Tübingen, Germany, and Landeskrankenhaus Bregenz Bregenz AustriaUniversity Medical Center, Ulm University Ulm GermanyKlinikum St Marien Amberg GermanyCenter for Pediatric Rheumatology Landshut GermanyMedical University Graz Graz AustriaMedical University of Innsbruck, Innsbruck, Austria, and Danube Private University Krems AustriaHelios Klinikum Berlin‐Buch Berlin GermanyGerman Center for Pediatric and Adolescent Rheumatology Garmisch‐Partenkirchen GermanyUniversity of Wuerzburg Wuerzburg GermanyUniversity of Wuerzburg Wuerzburg GermanyTechnische Universität Dresden GermanyTechnische Universität Dresden GermanyTechnische Universität Dresden GermanyUniversity of Vienna Vienna AustriaUniversity of Vienna Vienna AustriaUniversity of Augsburg Augsburg GermanyUniversity of Leipzig Leipzig GermanyUniversity of Tübingen Tübingen GermanyCharité Universitätsmedizin Berlin Berlin GermanyCharité Universitätsmedizin Berlin and Rheuma‐Forschungszentrum Berlin Berlin GermanyUniversity of Liverpool and Alder Hey Children's NHS Foundation Trust Liverpool United KingdomOlgahospital Klinikum Stuttgart GermanyObjective Pulmonary involvement in chronic nonbacterial osteomyelitis (CNO) is rare. Limited awareness results in diagnostic challenges, especially because malignancy or infection needs to be considered. Methods Based on a survey shared among centers participating in the Kerndokumentation Deutsches Rheumaforschungszentrum (Germany), this study investigated clinical and imaging presentations, demographic features, treatment response and outcomes of pulmonary involvement in CNO (pCNO). Magnetic resonance imaging and computed tomography images were read centrally by an experienced pediatric radiologist. Results Twenty‐two patients with pCNO were included in this study. Among patients with CNO, pulmonary involvement was more common in girls (91% vs 62.8%, P = 0.006) and patients with multifocal bone lesions (95% vs 65%, P <0.001) but was not associated with systemic inflammation or additional organ involvement. Forty‐two pulmonary lesions were counted with a median of two per patient (two to six). They displayed a median size of 1.8 cm (0.3–4.0 cm) and followed mono‐ (40%) and oligo‐focal (60%) patterns representing consolidations or nodules, abutting the pleura in 50%. Although prominent hilar lymph nodes were present (in 19% of patients), no pathologic enlargement (>1 cm) was seen. When available (3 of 22 patients), histology revealed granulomatous inflammation with lymphocyte infiltration. Development and courses of pCNO did not associate with treatments chosen. Complete remission was reported in 60% of patients, partial remission in 20%. Conclusion pCNO is usually asymptomatic. Although more common in girls and patients with multifocal CNO, pCNO is not associated with systemic parameters of inflammation or specific organ involvement. Prognosis of pCNO is favorable, and most lesions resolve over time. Thus, a careful watch‐and‐wait strategy may be appropriate.https://doi.org/10.1002/acr2.11799
spellingShingle Toni Hospach
Friederike Blankenburg
Anita Heinkele
Kristina Rücklová
Peter Müller‐Abt
Frank Weller‐Heinemann
Laura Buchtala
Jan Maier
Jasmin Kümmerle‐Deschner
Christiane Reiser
Ales Janda
Andreas Urban
Reiner Berendes
Andrea Skrabl‐Baumgartner
Jürgen Brunner
Ralf Trauzeddel
Johannes Peter Haas
Annette Holl‐Wieden
Henner Morbach
Anja Schnabel
Normi Brück
Gabriele Hahn
Wolfgang Emminger
Isabella Valent
Regine Borchers
Christian Klemann
Ilias Tsiflikas
Jens Klotsche
Kirsten Minden
Christian M. Hedrich
Thekla von Kalle
Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
ACR Open Rheumatology
title Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
title_full Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
title_fullStr Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
title_full_unstemmed Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
title_short Clinical Presentation and Course of Pulmonary Involvement in Chronic Nonbacterial Osteomyelitis
title_sort clinical presentation and course of pulmonary involvement in chronic nonbacterial osteomyelitis
url https://doi.org/10.1002/acr2.11799
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