Sarcoidosis - a multisystem disease.

Détails

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Etat: Public
Version: Final published version
Licence: CC BY-NC-SA 4.0
ID Serval
serval:BIB_BE7643C6051D
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Sarcoidosis - a multisystem disease.
Périodique
Swiss medical weekly
Auteur⸱e⸱s
Franzen D.P., Brutsche M., Nilsson J., Böni C., Daccord C., Distler O., Elsener D., Funke-Chambour M., Gruner C., Hayward-Könnecke H., Hostettler K.E., Kündig T., Ribi C., Seebach J.D., Seeger H., Vrugt B., Kolios AGA
ISSN
1424-3997 (Electronic)
ISSN-L
0036-7672
Statut éditorial
Publié
Date de publication
03/01/2022
Peer-reviewed
Oui
Volume
152
Pages
w30049
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: epublish
Résumé
Sarcoidosis is a systemic inflammatory disease, characterised by granuloma formation upon an unknown trigger in genetically predisposed individuals. The inflammation is characterised by an activation of both the innate immune system, with macrophages differentiating into epitheloid cells and dendritic cells, and the adaptive immune system, particularly T helper (Th) 1 and Th17 cells. Since all organs can be affected to varying extents, clinical presentation is often diverse. Most commonly, the lungs, lymph nodes, skin and eyes are involved, whereas cardiac, renal and neurological manifestations are less common but associated with higher morbidity. Depending on the clinical symptoms, a detailed evaluation including thorough clinical examination, imaging and laboratory tests should explore all possible organ involvements. In some patients, fatigue manifests as a para-sarcoidosis symptom impacting quality of life, even if sarcoidosis is in remission. Some acute syndromic presentations, such as Löfgren's syndrome, have a good prognosis and are commonly self-limiting. If possible, a topical treatment, for example for cutaneous sarcoidosis or bronchial involvement, should be applied. Treatment of severe cases with persisting disease activity necessitates long-term immunosuppressive drugs, with glucocorticoids as the first-line option. Steroid-sparing and second-line drugs include methotrexate, azathioprine, mycophenolate mofetil and immunomodulators such hydroxychloroquine, with the latter being first-line therapy in cutaneous sarcoidosis. Tumour necrosis factor-alpha inhibitors (particularly adalimumab and infliximab) are used as third-line agents but are administered earlier in cases of persistent disease activity, severe organ-involvement or intolerance to conventional drugs. Treatment decisions should be based on a multidisciplinary approach, depending on organ involvement and treatment tolerability. Para-sarcoidosis manifestations, particularly fatigue, should also be carefully addressed, where the patient could also be enrolled in multidimensional rehabilitation programmes. With various organ involvement and different phenotypes, larger studies including real-world data from registries are necessary to evaluate different sarcoidosis endotypes and preferential treatment pathways.
Mots-clé
Azathioprine/therapeutic use, Humans, Immunosuppressive Agents/therapeutic use, Quality of Life, Sarcoidosis/diagnosis, Sarcoidosis/drug therapy, Sarcoidosis, Pulmonary/drug therapy
Pubmed
Web of science
Open Access
Oui
Création de la notice
31/01/2022 11:21
Dernière modification de la notice
25/11/2023 8:20
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