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Tytuł pozycji:

Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions.

Tytuł:
Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions.
Autorzy:
Saha BK; Division of Pulmonary and Critical Care Medicine, Ozarks Medical Center, 1100 Kentucky Avenue, West Plains, MO, 65775, USA. .
Milman NT; Department of Clinical Biochemistry, Næstved Hospital, University College Zealand, DK-4700, Næstved, Denmark.
Źródło:
Clinical rheumatology [Clin Rheumatol] 2021 Jul; Vol. 40 (7), pp. 2547-2557. Date of Electronic Publication: 2020 Nov 12.
Typ publikacji:
Journal Article; Review
Język:
English
Imprint Name(s):
Publication: <2008->: Heidelberg : Springer
Original Publication: Brussels : Acta Medica Belgica, [1982-
MeSH Terms:
Hemosiderosis*/drug therapy
Lung Diseases*/drug therapy
Adult ; Azathioprine/therapeutic use ; Hemoptysis ; Humans ; Hemosiderosis, Pulmonary
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Contributed Indexing:
Keywords: Idiopathic pulmonary hemosiderosis; Mesenchymal cell transplant; Pulmonary hemorrhage; Rituximab; Treatment
Substance Nomenclature:
MRK240IY2L (Azathioprine)
Entry Date(s):
Date Created: 20201113 Date Completed: 20210611 Latest Revision: 20231213
Update Code:
20240105
DOI:
10.1007/s10067-020-05507-4
PMID:
33184706
Czasopismo naukowe
This paper reviews the literature on the treatment modalities for idiopathic pulmonary hemosiderosis (IPH) used over the past 30 years, attempting to define treatment options that appear to be efficacious and safe, and in addition presents a treatment algorithm. IPH is an uncommon etiology of diffuse alveolar hemorrhage. IPH is a rare disease in adults and often associated with a significant temporal delay in diagnosis. Patients present with hemoptysis, radiographic chest abnormalities, and iron deficiency anemia. Although several pathogenetic hypotheses have been proposed, IPH appears to be an immunologic disease, possibly with a genetic component. Corticosteroid therapy represents the first line of treatment, including liposome-incorporated dexamethasone palmitate (liposteroid). Additional immunomodulatory/immunosuppressive medications have been used with varying success, especially in the setting of steroid-refractory disease. Cyclophosphamide, azathioprine, hydroxychloroquine, mycophenolate mofetil, and mesenchymal cell transplantation have been attempted to improve outcome and reduce side effects. Controlled studies are needed to assess the optimal combination of medications, which are effective to control the disease.

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