Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach.
Connective tissue disease
Diffuse alveolar hemorrhage
Idiopathic pulmonary hemosiderosis
Rheumatic disease
Vasculitis
Journal
Clinical rheumatology
ISSN: 1434-9949
Titre abrégé: Clin Rheumatol
Pays: Germany
ID NLM: 8211469
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
received:
08
12
2020
accepted:
23
08
2021
revised:
20
08
2021
pubmed:
8
9
2021
medline:
27
1
2022
entrez:
7
9
2021
Statut:
ppublish
Résumé
This narrative review provides an overview of diffuse alveolar hemorrhage (DAH) associated with rheumatologic and autoimmune diseases and their differentiation from idiopathic pulmonary hemosiderosis (IPH). Relevant immunologic diseases associated with DAH are discussed, and a diagnostic flowchart is proposed to establish a "definitive" diagnosis of IPH within the spectrum of DAH. IPH is a rare cause of recurrent DAH both in children and adults. In adults, a definitive diagnosis of IPH requires a lung biopsy and histopathologic examination demonstrating intraalveolar hemorrhage, hemosiderin-laden macrophages, and a variable degree of fibrosis in the absence of both capillaritis and cellular inflammation. The presence of small vessel vasculitis points towards immunologic, well-differentiated, or sometimes undifferentiated rheumatologic diseases. However, it is essential to recognize that many rheumatologic diseases may in the initial phase present with DAH without any evidence of capillaritis, thus mimicking IPH. Although not definitely established, it is likely that immunologic processes are involved in IPH, and we, therefore, suggest the consideration of a more suitable term for the disease, e.g., "Immune-mediated Pulmonary Hemosiderosis" to acknowledge the aberrancy in the immune parameters and a positive response to immunosuppressive therapy.
Identifiants
pubmed: 34491458
doi: 10.1007/s10067-021-05895-1
pii: 10.1007/s10067-021-05895-1
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
325-336Informations de copyright
© 2021. International League of Associations for Rheumatology (ILAR).
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