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Various patterns of acute alveolar haemorrhage in patients with microscopic polyangiitis: a clinicopathological study
Abstract:
It is well known that acute alveolar haemorrhage (AAH) is attributed to capillaritis in most cases with microscopic polyangiitis (MPA). In this article, we explore the cause of alveolar haemorrhage in MPA patients. In the present study, we extracted four autopsy cases of MPA with AAH. Patient's sex and age, cause of alveolar haemorrhage, therapy, follow-up duration, and cause of death were investigated. As a result, alveolar haemorrhage was caused by diffuse alveolar damage (DAD) due to candidiasis or influenza virus infection, haemorrhagic infarct due to aspergillosis, capillaritis due to MPA, vasculitis due to cytomegalovirus (CMV), and herpes simplex virus (HSV) infection. All patients received corticosteroid therapy, and one patient additionally underwent administration of cyclophosphamide. The duration of follow-up ranged from one to 26 months with a mean of eight months. All patients died of respiratory failure. In summary, clinicians and pathologists should recognise some causes of alveolar haemorrhage in MPA patients, which include DAD, haemorrhagic infarct, virus-associated vasculitis, or MPA-associated capillaritis.
Insights
Acute alveolar haemorrhage (AAH) in microscopic polyangiitis (MPA) can stem from various causes beyond capillaritis. This study highlights infections and other conditions contributing to AAH in MPA patients, all of whom died from respiratory failure.
Area of Science:
- Pulmonary Medicine
- Pathology
- Rheumatology
Background:
- Microscopic polyangiitis (MPA) is a small-vessel vasculitis often associated with acute alveolar haemorrhage (AAH).
- Traditionally, AAH in MPA is attributed to capillaritis.
- This study investigates less common causes of AAH in MPA patients.
Observation:
- Analysis of four autopsy cases of MPA with AAH.
- Investigated factors included patient demographics, cause of AAH, treatment, follow-up, and cause of death.
- All patients received corticosteroid therapy, with one also receiving cyclophosphamide.
Findings:
- AAH causes included diffuse alveolar damage (DAD) from candidiasis or influenza, haemorrhagic infarct from aspergillosis, MPA-associated capillaritis, and virus-associated vasculitis (CMV, HSV).
- All four patients experienced respiratory failure as the cause of death.
- Follow-up duration ranged from 1 to 26 months (mean 8 months).
Implications:
- Clinicians and pathologists must consider diverse etiologies for AAH in MPA.
- Recognizing causes like DAD, infections, and virus-associated vasculitis is crucial for patient management.
- This broadens the differential diagnosis for AAH in MPA, potentially improving diagnostic accuracy.
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