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Copyright of Die Dermatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Innere Medizin (2731-7080) is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Internist is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This article reports the case of a 30-year-old female patient who suffered for many years from initially unspecific symptoms, such as recurrent, nonallergic and noninfectious sinusitis, late-onset bronchial asthma and pronounced lymphadenopathy; however, the correct diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) could only be made by histological investigations after the appearance of skin symptoms. The EGPA is a severe systemic disease which, if left untreated, can cause multiple organ damage and even be fatal. With adequate treatment the disease is mild in more than 90% of cases and patients can even completely recover. By making the correct diagnosis, the patient could be successfully treated and the risk of late manifestations and subsequent damage with a potentially fatal outcome was reduced., (© 2024. The Author(s).)

Copyright of Die Nephrologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application. Globally, the recently updated EULAR recommendations represent the most up to date management guidelines. Therapeutically, rituximab and cyclophosphamide in combination with glucocorticoids remain the pillars of treatment in remission induction for severe organ-threatening and life-threatening diseases. For the first time, mepolizumab and avacopan represent approved treatment options for specific entities that make a significant contribution to steroid reduction. New attention has been paid to patient-reported outcomes, for which a disease-specific outcome questionnaire is now available., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

“Eosinophilie lung diseases" are a heterogeneous group of disorders characterized by eosinophilic infiltration of the alveolar space and interstitium of the lung. These entities present in the form of acute or chronic eosinophilic pneumonia, transient pulmonary infiltrates (“Löffler Syndrome") or in the context of systemic disease. The diagnosis relies on characteristic clinical and radiological features and the presence of eosinophilia in samples obtained by bronchoalveolar lavage. A common therapeutic denominator is the dramatic response to systemic corticosteroids and prompt cure in the vast majority of cases [ABSTRACT FROM AUTHOR]

Copyright of Der Nephrologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Vasculitides are inflammatory diseases of blood vessels caused by autoimmune or infectious processes, which are associated with alterations and destruction of the vascular wall. From a histopathological point of view, granulomatous vasculitides can be distinguished from necrotizing vasculitides with respect to the pattern of inflammation. Granulomatous vasculitides are characterized by intramural, predominantly lymphohistiocytic infiltrates with the formation of giant cells. They include giant cell arteritis (GCA) and Takayasu arteritis (TAK). By contrast, anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) belongs to the group of necrotizing vasculitides. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In addition to systemic necrotizing small vessel vasculitis, GPA and EGPA are characterized by extravascular granulomatous necrotizing inflammation mainly affecting the upper and/or lower respiratory tract, in EGPA with eosinophilic infiltrates. These granulomatous lesions are part of the autoimmune process and associated with tissue damage., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rheumatic diseases characterized by small-to-medium vessel vasculitis. Three different entities can be distinguished: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). While lung and renal involvement are typical manifestations of both GPA and MPA, EGPA usually shows paranasal sinus and lung involvement as well as a history of bronchial asthma. Furthermore, EGPA is frequently associated with cardiac disease and peripheral neuropathy. Cyclophosphamide or rituximab, combined with glucocorticoids, are used to induce remission of severe disease. Maintenance therapy options include rituximab as the first-line treatment, as well as methotrexate or azathioprine plus low-dose glucocorticoids., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Copyright of Der Pneumologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Small vessel vasculitis is characterized by a necrotizing inflammation of the vessel wall predominantly with involvement of small intraparenchymal arteries, arterioles, capillaries and venules. Medium-sized and occasionally large vessels can also be involved. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) are differentiated from immune complex vasculitides based on immunopathological and serological aspects. Immune complex vasculitides include IgA vasculitis, cryoglobulinemic vasculitis, hypocomplementemic urticarial vasculitis (anti-C1q vasculitis) and anti-glomerular basement membrane disease. Epidemiological and next-generation sequencing-based studies have significantly contributed to the identification of predisposing environmental factors and genetic risk factors in recent years. Under specific conditions ANCA and immune complexes can induce premature intravascular activation of neutrophilic granulocytes with degranulation and release of enzymes and reactive oxygen species, which leads to vascular damage. In granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis various factors, such as barrier dysfunction and dysbiosis of the microbiome contribute to extravascular granuloma formation predominantly affecting the respiratory tract., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

The pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides is complex; however, a better understanding in recent years has enabled new therapeutic approaches. In recent years priority was given to the minimization of treatment-associated toxicity. For induction of remission of severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), glucocorticoids are used as well as cyclophosphamide and rituximab. The current recommendations enable a more rapid tapering of steroid dose and advise caution in the use of plasmapheresis. Rituximab and azathioprine are available options for maintenance of remission. The choice of medication and duration of remission maintenance are oriented particularly to the risk of recurrence. The importance of low-dose steroids has not yet been finally clarified. New treatment approaches, such as the C5a receptor inhibitor avacopan could enable a minimized steroid treatment in the future. The treatment of eosinophilic granulomatosis with polyangiitis (EGPA) is less evidence-based and consists of glucocorticoids, immunosuppressive agents depending on the severity and increasingly more biologics, e.g. interleukin‑5 blockade. Supportive measures (e.g. vaccinations, infection prophylaxis, cardiovascular risk management) are increasing in importance. Future treatment strategies must take the individual risk (e.g. ANCA subtype, relapse risk) more into consideration for selection and duration of treatment., (© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2021.)