Abstract
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.
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Abbreviations
- AAV :
-
ANCA-associated vasculitis
- ANA:
-
Antinuclear antibody
- ANCA:
-
Anti-neutrophil cytoplasmic antibody
- BAL:
-
Bronchoalveolar lavage
- BH:
-
Bland pulmonary hemorrhage
- c-ANCA:
-
Cytoplasmic anti-neutrophil cytoplasmic antibody
- CPK:
-
Creatine phosphokinase
- CTD:
-
Connective tissue disease
- DAD:
-
Diffuse alveolar damage
- DAH:
-
Diffuse alveolar hemorrhage
- DM:
-
Dermatomyositis
- GCA:
-
Giant cell arteritis
- IPH:
-
Idiopathic pulmonary hemosiderosis
- LVV:
-
Large vessel vasculitis
- MVV:
-
Medium vessel vasculitis
- PAN:
-
Polyarteritis nodosa
- p-ANCA:
-
Perinuclear anti-neutrophil cytoplasmic antibody
- PM:
-
Polymyositis
- RD:
-
Rheumatologic disease
- SLE:
-
Systemic lupus erythematosus
- TAK:
-
Takayasu arteritis
- VATS:
-
Video-assisted thoracoscopic surgery
- VVV:
-
Variable vessel vasculitis
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Saha, B.K., Chong, W.H. & Milman, N.T. Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach. Clin Rheumatol 41, 325–336 (2022). https://doi.org/10.1007/s10067-021-05895-1
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DOI: https://doi.org/10.1007/s10067-021-05895-1