Sarcoidosis - a multisystem disease.

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State: Public
Version: Final published version
License: CC BY-NC-SA 4.0
Serval ID
serval:BIB_BE7643C6051D
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Sarcoidosis - a multisystem disease.
Journal
Swiss medical weekly
Author(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
Publication state
Published
Issued date
03/01/2022
Peer-reviewed
Oui
Volume
152
Pages
w30049
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
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.
Keywords
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
Yes
Create date
31/01/2022 10:21
Last modification date
25/11/2023 7:20
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