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Article ; Online: Insights into B-cell ontogeny inferred from human immunology.

Dirks, Johannes / Viemann, Dorothee / Beyersdorf, Niklas / Härtel, Christoph / Morbach, Henner

2023 Volume 53, Issue 6, Page(s) e2250116

Abstract: Due to ontogenetic changes in B-cell developmental lineages, the mature B-cell compartment constitutes by functionally different B-cell subsets that emerged from prenatal, early postnatal or adult precursors. While negative selection processes operate ... ...

Abstract	Due to ontogenetic changes in B-cell developmental lineages, the mature B-cell compartment constitutes by functionally different B-cell subsets that emerged from prenatal, early postnatal or adult precursors. While negative selection processes operate primarily within the framework of B-cell tolerance checkpoints during B-cell development, further differentiation into distinct B-cell subsets is additionally induced by positive selection. In addition to endogenous antigens, contact with microbial antigens is also involved in this selection process, with intestinal commensals having a significant influence on the development of a large layer within the B-cell compartment. The decisive threshold that triggers negative selection seems to be relaxed during fetal B-cell development, thereby allowing recruitment of polyreactive and also autoreactive B-cell clones into the mature naïve B-cell compartment. Almost all of the concepts on B-cell ontogeny are based on observations in laboratory mice that not only differ from humans in their developmental timeline but also in their composition of commensal microorganisms or rather a lack of exposure to these. In this review, we summarize conceptual findings on B-cell ontogeny and particularly describe key insights into the developing human B-cell compartment and immunoglobulin repertoire formation.
MeSH term(s)	Mice ; Animals ; Adult ; Humans ; B-Lymphocytes ; B-Lymphocyte Subsets ; Antigens ; Immune Tolerance ; Cell Differentiation
Chemical Substances	Antigens
Language	English
Publishing date	2023-04-05
Publishing country	Germany
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	120108-6
ISSN	1521-4141 ; 0014-2980
ISSN (online)	1521-4141
ISSN	0014-2980
DOI	10.1002/eji.202250116
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Article ; Online: IgD shapes the pre-immune naïve B cell compartment in humans.

Dirks, Johannes / Andres, Oliver / Paul, Luisa / Manukjan, Georgi / Schulze, Harald / Morbach, Henner

Frontiers in immunology

2023 Volume 14, Page(s) 1096019

Abstract: B cell maturation and immunoglobulin (Ig) repertoire selection are governed by expression of a functional B cell receptor (BCR). Naïve B cells co-express their BCR as IgM and IgD isotype. However, the role of the additionally expressed IgD on naïve B ... ...

Abstract	B cell maturation and immunoglobulin (Ig) repertoire selection are governed by expression of a functional B cell receptor (BCR). Naïve B cells co-express their BCR as IgM and IgD isotype. However, the role of the additionally expressed IgD on naïve B cells is not known. Here we assessed the impact of IgD on naïve B cell maturation and Ig repertoire selection in 8 individuals from 3 different families with heterozygous loss-of-function or loss-of expression mutations in
MeSH term(s)	Humans ; Immunoglobulin D/metabolism ; Immunoglobulin M ; B-Lymphocytes ; Receptors, Antigen, B-Cell/genetics ; Receptors, Antigen, B-Cell/metabolism ; Immunoglobulin Isotypes/metabolism
Chemical Substances	Immunoglobulin D ; Immunoglobulin M ; Receptors, Antigen, B-Cell ; Immunoglobulin Isotypes
Language	English
Publishing date	2023-01-26
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2023.1096019
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Article: GPA und MPA bei Kindern und Jugendlichen

Holl-Wieden, Annette / Morbach, Henner / Benoit, Clemens / Schmalzing, Marc

Arthritis und Rheuma

2023 Volume 43, Issue 01, Page(s) 48–56

Abstract: Die Antineutrophile cytoplasmatische Antikörper (ANCA)-assoziierten Vaskulitiden (AAVs) sind systemische Vaskulitiden, die vor allem die kleinen Blutgefäße betreffen und mit dem Vorhandensein von Autoantikörpern, den sogenannten ANCA, assoziiert sind. Zu ...

Abstract	Die Antineutrophile cytoplasmatische Antikörper (ANCA)-assoziierten Vaskulitiden (AAVs) sind systemische Vaskulitiden, die vor allem die kleinen Blutgefäße betreffen und mit dem Vorhandensein von Autoantikörpern, den sogenannten ANCA, assoziiert sind. Zu den AAV werden die Granulomatose mit Polyangiitis (GPA, früher Wegener-Granulomatose), die Mikroskopische Polyangiitis (MPA) und die Eosinophile Granulomatose mit Polyangiitis (EGPA, früher Churg-Strauss-Syndrom) gerechnet. AAV sind im Kindes- und Jugendalter sehr selten. Sie müssen aber bei unklaren Organmanifestationen und unklarer Inflammation in die differenzialdiagnostischen Überlegungen miteinbezogen werden. Die frühzeitige Diagnosestellung und richtige Therapie sind entscheidend, da die Erkrankungen lebens- oder organbedrohend verlaufen können. Obwohl sich die klinische Symptomatik bei Kindern und bei Erwachsenen ähnelt, gibt es einige Unterschiede. Die Therapie der AAV im Kindes- und Jugendalter basiert auf Studien von Erwachsenen, wodurch insbesondere in der optimalen Dosierung der eingesetzten Medikamente Fragen offen bleiben. Die aktuellen Therapiestrategien haben das Outcome signifikant verbessert, dennoch besteht ein hohes Risiko für Rezidive und vor allem bei Kindern ein hohes Risiko für Vaskulitis-assoziierte Folgeschäden. Intensivere Forschungsaktivitäten zur Pathogenese und Behandlung der AAV bei Kindern sind dringend notwendig, um die Effektivität und das Risiko der Behandlung bei Kindern zu beurteilen und das langfristige Outcome der komplexen Erkrankung zu verbessern.
Keywords	ANCA ; Antineutrophile cytoplasmatische Antikörper ; AAV ; ANCA-assoziierte Vaskulitis ; GPA ; Granulomatose mit Polyangiitis ; MPA ; Mikroskopische Polyangiitis
Language	German
Publishing date	2023-03-01
Publisher	Georg Thieme Verlag KG
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	2223481-0
ISSN	2567-5753 ; 0176-5167
ISSN (online)	2567-5753
ISSN	0176-5167
DOI	10.1055/a-1990-4741
Database	Thieme publisher's database

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Article: GPA und MPA bei Kindern und Jugendlichen

Holl-Wieden, Annette / Morbach, Henner / Benoit, Clemens / Schmalzing, Marc

Arthritis + Rheuma

2023 Volume 43, Issue 1, Page(s) 48

Language	German
Document type	Article
ZDB-ID	605764-0
ISSN	0176-5167
Database	Current Contents Medicine

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Article: Bericht der Kommission Klinische Studien/Forschung

Tenbrock, Klaus / Brunner, Jürgen / Morbach, Henner

Arthritis und Rheuma

2022 Volume 42, Issue 03, Page(s) 197–197

Language	German
Publishing date	2022-05-01
Publisher	Georg Thieme Verlag KG
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	2223481-0
ISSN	2567-5753 ; 0176-5167
ISSN (online)	2567-5753
ISSN	0176-5167
DOI	10.1055/a-1816-5855
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Article ; Online: VEXAS-Syndrom, eine neu beschriebene autoinflammatorische Systemerkrankung mit dermatologischen Manifestationen.

Baur, Vera / Stoevesandt, Johanna / Hueber, Axel / Hüffmeier, Ulrike / Kneitz, Hermann / Morbach, Henner / Schultz, Erwin / Goebeler, Matthias

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG

2023 Volume 21, Issue 12, Page(s) 1456–1464

Language	German
Publishing date	2023-12-11
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	2093479-8
ISSN	1610-0387 ; 1610-0379
ISSN (online)	1610-0387
ISSN	1610-0379
DOI	10.1111/ddg.15227_g
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Article ; Online: VEXAS-Syndrome, a newly described autoinflammatory systemic disease with dermatologic manifestations.

Baur, Vera / Stoevesandt, Johanna / Hueber, Axel / Hüffmeier, Ulrike / Kneitz, Hermann / Morbach, Henner / Schultz, Erwin / Goebeler, Matthias

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG

2023 Volume 21, Issue 12, Page(s) 1456–1463

Abstract: VEXAS syndrome is a recently identified autoinflammatory systemic disease caused by an acquired somatic mutation of the X-linked UBA1 gene, the key enzyme of the first step of ubiquitylation. The acronym VEXAS stands for the characteristics Vacuoles, E1 ... ...

Abstract	VEXAS syndrome is a recently identified autoinflammatory systemic disease caused by an acquired somatic mutation of the X-linked UBA1 gene, the key enzyme of the first step of ubiquitylation. The acronym VEXAS stands for the characteristics Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic. The disease occurs in advanced adulthood preferentially in men and is characterized by hematological, rheumatological and dermatological symptoms. The latter include neutrophil-rich lesions reminiscent of Sweet's syndrome, erythema nodosum- and panniculitis-like skin manifestations and recurrent polychondritis of the nose and auricles. The presence of cytoplasmic vacuoles in myeloid and erythroid precursors in the bone marrow is characteristic. In up to half of the cases, VEXAS syndrome is associated with myelodysplastic syndrome. Dermatologists should be familiar with the clinical picture, as skin symptoms are often the first indicator of the disease. Molecular diagnostics are essential for confirming the diagnosis and risk stratification of affected patients. In this minireview we provide an overview of the pathophysiology, diagnosis and therapy of VEXAS syndrome and illustrate its clinical picture with two own cases.
MeSH term(s)	Male ; Humans ; Adult ; Sweet Syndrome/diagnosis ; Autoimmune Diseases ; Cartilage Diseases ; Ear Auricle ; Mutation
Language	English
Publishing date	2023-11-12
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	2093479-8
ISSN	1610-0387 ; 1610-0379
ISSN (online)	1610-0387
ISSN	1610-0379
DOI	10.1111/ddg.15227
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Article ; Online: Immunization of preterm infants: current evidence and future strategies to individualized approaches

Fortmann, Mats Ingmar / Dirks, Johannes / Goedicke-Fritz, Sybelle / Liese, Johannes / Zemlin, Michael / Morbach, Henner / Härtel, Christoph

Semin Immunopathol. 2022 Nov., v. 44, no. 6, p. 767-784

2022 , Page(s) 767–784

Abstract: Preterm infants are at particularly high risk for infectious diseases. As this vulnerability extends beyond the neonatal period into childhood and adolescence, preterm infants benefit greatly from infection-preventive measures such as immunizations. ... ...

Abstract	Preterm infants are at particularly high risk for infectious diseases. As this vulnerability extends beyond the neonatal period into childhood and adolescence, preterm infants benefit greatly from infection-preventive measures such as immunizations. However, there is an ongoing discussion about vaccine safety and efficacy due to preterm infants’ distinct immunological features. A significant proportion of infants remains un- or under-immunized when discharged from primary hospital stay. Educating health care professionals and parents, promoting maternal immunization and evaluating the potential of new vaccination tools are important means to reduce the overall burden from infectious diseases in preterm infants. In this narrative review, we summarize the current knowledge about vaccinations in premature infants. We discuss the specificities of early life immunity and memory function, including the role of polyreactive B cells, restricted B cell receptor diversity and heterologous immunity mediated by a cross-reactive T cell repertoire. Recently, mechanistic studies indicated that tissue-resident memory (Trm) cell populations including T cells, B cells and macrophages are already established in the fetus. Their role in human early life immunity, however, is not yet understood. Tissue-resident memory T cells, for example, are diminished in airway tissues in neonates as compared to older children or adults. Hence, the ability to make specific recall responses after secondary infectious stimulus is hampered, a phenomenon that is transcriptionally regulated by enhanced expression of T-bet. Furthermore, the microbiome establishment is a dominant factor to shape resident immunity at mucosal surfaces, but it is often disturbed in the context of preterm birth. The proposed function of Trm T cells to remember benign interactions with the microbiome might therefore be reduced which would contribute to an increased risk for sustained inflammation. An improved understanding of Trm interactions may determine novel targets of vaccination, e.g., modulation of T-bet responses and facilitate more individualized approaches to protect preterm babies in the future.
Keywords	B-lymphocytes ; T-lymphocytes ; adolescence ; childhood ; fetus ; health services ; hospitals ; humans ; immunity ; inflammation ; macrophages ; memory ; microbiome ; postpartum period ; premature birth ; risk ; transcription (genetics) ; vaccination ; vaccines
Language	English
Dates of publication	2022-11
Size	p. 767-784
Publishing place	Springer Berlin Heidelberg
Document type	Article ; Online
Note	Review
ZDB-ID	2316828-6
ISSN	1863-2300 ; 1863-2297
ISSN (online)	1863-2300
ISSN	1863-2297
DOI	10.1007/s00281-022-00957-1
Database	NAL-Catalogue (AGRICOLA)

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Article ; Online: New Insights into Adult and Paediatric Chronic Non-bacterial Osteomyelitis CNO.

Hedrich, Christian M / Morbach, Henner / Reiser, Christiane / Girschick, Hermann J

Current rheumatology reports

2020 Volume 22, Issue 9, Page(s) 52

Abstract: Purpose of review: To describe in detail the clinical synopsis and pathophysiology of chronic non-bacterial osteomyelitis and SAPHO syndrome.: Recent findings: Chronic non-bacterial osteomyelitis (CNO) has been identified as a disease entity for ... ...

Abstract	Purpose of review: To describe in detail the clinical synopsis and pathophysiology of chronic non-bacterial osteomyelitis and SAPHO syndrome. Recent findings: Chronic non-bacterial osteomyelitis (CNO) has been identified as a disease entity for almost 50 years. This inflammatory bone disorder is characterized by osteolytic as well as hyperostotic/osteosclerotic lesions. It is chronic in nature, but it can present with episodic flairs and phases of remission, which have led to the denomination "chronic recurrent osteomyelitis", with its severe multifocal form "chronic recurrent multifocal osteomyelitis" (CRMO). For almost three decades, an infectious aetiology had been considered, since especially Propionibacterium acnes had been isolated from bone lesions of individual patients. However, this concept has been challenged since long-term antibiotic therapy did not alter the course of disease and modern microbiological techniques (including PCR) failed to confirm bone infection as an underlying cause. Over recent years, a profound dysregulation of cytokine expression profiles has been demonstrated in innate immune cells of CNO patients. A hallmark of monocytes from CNO patients is the failure to produce immune regulatory cytokines interleukin-10 (IL-10) and IL-19, which have been linked with genetic and epigenetic alterations. Subsequently, a significant upregulation of pro-inflammatory, NLRP3 inflammasome-dependent cytokines (IL-1β and TNF-α), has been demonstrated. The current knowledge on CNO, the underlying molecular pathophysiology, and modern imaging strategies are summarized; differential diagnoses, treatment options, outcome measures, as well as quality of life studies are discussed.
MeSH term(s)	Acquired Hyperostosis Syndrome/diagnosis ; Acquired Hyperostosis Syndrome/physiopathology ; Adult ; Child ; Chronic Disease ; Cytokines ; Epigenesis, Genetic ; Humans ; Inflammasomes ; Osteomyelitis/diagnosis ; Osteomyelitis/physiopathology ; Quality of Life
Chemical Substances	Cytokines ; Inflammasomes
Language	English
Publishing date	2020-07-23
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2057357-1
ISSN	1534-6307 ; 1523-3774
ISSN (online)	1534-6307
ISSN	1523-3774
DOI	10.1007/s11926-020-00928-1
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Zs.A 5531: Show issues

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Article: ESPED survey on newly diagnosed immune thrombocytopenia in childhood: how much treatment do we give?

von Lukowicz, Hannah / Schlegel, Paul-Gerhardt / Härtel, Christoph / Morbach, Henner / Haubitz, Imme / Wiegering, Verena

Molecular and cellular pediatrics

2021 Volume 8, Issue 1, Page(s) 11

Abstract: Background: Immune thrombocytopenia (ITP) is an autoimmune disease associated with isolated thrombocytopenia, which is caused by an imbalance between platelet production and platelet destruction. Petechial and mucous membrane hemorrhages are ... ...

Abstract	Background: Immune thrombocytopenia (ITP) is an autoimmune disease associated with isolated thrombocytopenia, which is caused by an imbalance between platelet production and platelet destruction. Petechial and mucous membrane hemorrhages are characteristic of ITP, but life-threatening bleeding rarely occurs. Depending on the bleeding symptoms, ITP can be treated with glucocorticoids (GC), intravenous immunoglobulins (IVIG), or in severe cases, platelet transfusions. Mild bleeding does not necessarily require therapy. Using the German Surveillance Unit for rare Pediatric Diseases (ESPED) we conducted a prospective survey on ITP patients in all German Children's Hospitals between September 2018 and August 2019. We collected data on ITP, including the clinical course, therapy implementation recommendations (according to the Association of German Scientific Medical Societies guidelines), outcome, and influence of treatment regimens depending on the treating physician´s experience with ITP patients. Results: Of the 287 recorded cases of children with ITP, 268 questionnaires were sent to the authors. Two hundred seventeen of the questionnaires fulfilled the inclusion criteria. ITP affected boys and girls similarly, and the median age of manifestation was 3.5 years. The main reasons for hospitalization were thrombocytopenia, bleeding signs, hematomas, and/or petechiae. Bleeding scores were ≤ 3 in 96% of children, which corresponded to a low-to-moderately low risk of bleeding. No life-threatening bleeding was documented. The most common therapies were IVIG (n = 59), GC (n = 33), or a combination of these (n = 17). Blood products (i.e., red blood cells, platelet concentrate, and fresh frozen plasma) were given to 13 patients. Compared to the established guidelines, 67 patients were over-treated, and 2 patients were under-treated. Conclusions: Adherence to German ITP treatment guidelines is currently limited. To improve patient safety and medical care, better medical training and dissemination of the guidelines are required in line with targeted analyses of patients with serious bleeding events to identify potential risk constellations.
Language	English
Publishing date	2021-09-05
Publishing country	Germany
Document type	Journal Article
ZDB-ID	2785551-X
ISSN	2194-7791
ISSN	2194-7791
DOI	10.1186/s40348-021-00121-z
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