Lupus & antiphospholipid syndrome
Diffuse alveolar haemorrhage in antiphospholipid syndrome: a French series of 26 patients
Authors' conclusion
The immediate prognosis of DAH, mainly treated with anticoagulants and steroids, was good. Patients with CAPS had a higher mortality rate in the long-term follow-up. Triple-positive and/or arterial phenotype patients more frequently experienced DAH relapses even when treated with anticoagulants.
Study context
- Population
- APS patients (Sydney criteria) with a history of definite diffuse alveolar haemorrhage (DAH), France
- Participants
- 26 patients
- Exposure
- Catastrophic APS (CAPS) vs Without CAPS
- CAPSn = 13
- Outcome
- Clinical presentation, treatment and outcome of DAH in APS
Methodological detailsmulticentre
- Multicentre
Main finding
As reported in the abstract
- Female
- 15 (58%)
- Median age, years
- 45.5 (95% CI, 35-58.1)
- History of thrombotic events
- 23 (88%)
- CAPS
- 13 (50%)
- Triple-positive for aPL
- 20 (77%)
- SLE
- 5 (19%)
- DAH inaugural
- 7 (27%)
- Treated with anticoagulants
- 22 (85%)
- Treated with steroids
- 22 (85%)
- Treated with immunosuppressive therapy
- 8 (31%)
- Complete remission
- 18 (69%)
- DAH relapses
- 14 (54%)
- Median time to relapse, years
- 1.8 (95% CI 1.1-3.6)
Observational data: associations, not effects.
Further findings
Risk factors for DAH relapse
| HR (95% CI) | ||
|---|---|---|
| History of arterial thrombosis | 4.6 (1.2–17) | |
| History of transient ischaemic attack or stroke | 7.8 (2.2–28) | |
| Mechanical ventilation during DAH episode | 6.1 (0.99–37) | |
| 0.050.21520Hazard ratio (log scale) |
| Outcome | HR (95% CI) | P value | |
|---|---|---|---|
| DAH relapse, withoutvs with CAPS (multivariate analysis)Multivariate analysis | 6.8 (1.0–45.3) | P = 0.048 | |
| 0.050.21520Hazard ratio (log scale) |
Complete remission
- Complete remission achieved
- 18 (69%)
DAH relapse
- Patients with DAH relapse
- 14 (54%)
- Median time to relapse, years
- 1.8 (95% CI 1.1-3.6)
Risk factors for DAH relapse
- Triple APL positivity was a risk factor for DAH relapse
Overall mortality
- Mortality with CAPS at 1 year
- 7.7%
- Mortality with CAPS at 5 years
- 37.1%
- Deaths without CAPS
- 0
Original publication
Diffuse alveolar haemorrhage in antiphospholipid syndrome: a French series of 26 patients. Rheumatology (Oxford). Published 14 December 2025. doi:10.
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