Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions.
Idiopathic pulmonary hemosiderosis
Mesenchymal cell transplant
Pulmonary hemorrhage
Rituximab
Treatment
Journal
Clinical rheumatology
ISSN: 1434-9949
Titre abrégé: Clin Rheumatol
Pays: Germany
ID NLM: 8211469
Informations de publication
Date de publication:
Jul 2021
Jul 2021
Historique:
received:
19
08
2020
accepted:
10
11
2020
revised:
31
10
2020
pubmed:
14
11
2020
medline:
12
6
2021
entrez:
13
11
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 33184706
doi: 10.1007/s10067-020-05507-4
pii: 10.1007/s10067-020-05507-4
doi:
Substances chimiques
Azathioprine
MRK240IY2L
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
2547-2557Références
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