Lupus & antiphospholipid syndrome

Risk and triggering factors for diffuse alveolar hemorrhage in primary antiphospholipid syndrome. An observational follow-up study and a systematic review of the literature

Semin Arthritis RheumPublished Observational follow-up study and systematic review of the literature

Authors' conclusion

Some risk factors and comorbidities characterizing PAPS-associated DAH patients were identified when the clinical and laboratory characteristics of a group of these subjects were compared with those of a control group and of PAPS-associated DAH patients pinpointed by a literature review.

Study context

Population
Primary APS patients fulfilling the Sydney criteria, all with thrombosis142 women and 55 men
Participants
197 patients
Comparison
Primary antiphospholipid syndrome (PAPS)-associated diffuse alveolar hemorrhage (DAH) vs PAPS without DAH (controls); and vs PAPS-associated DAH patients from literature review
  • DAHn = 8
  • Literature-review DAHn = 55
Outcome
Risk factors and comorbidities (left-sided heart valve diseases, left HF, hemolytic anemia) associated with DAH
Methodological detailscase-control comparison, logistic regression analysis
  • Case-control comparison
  • Logistic regression analysis

Pooled result

As reported in the abstract

DAH

4.1 %

8

Multiple types of vascular involvement profile, independent risk factor
P = 0.030
Left-sided heart valve diseases, independent risk factor
0.022
Hemolytic anemia, independent risk factor
0.007

Further pooled results

Characteristics of DAH vs controls

Male gender more prevalent in DAH
P = 0.0399
Multiple types of vascular involvement profile more prevalent in DAH
P = 0.0048
High aPL titers more prevalent in DAH
P = 0.0011
Left-sided heart valve diseases higher in DAH
0.0253
Left HF higher in DAH
0.0451
Hemolytic anemia higher in DAH
0.0358
Triple aPL positivity and microcirculation thrombosis more frequent in DAH, not significant

DAH patients vs literature-review DAH patients

Clinical and laboratory characteristics and comorbidities not significantly different
Only hemolytic anemia significantly less frequent in literature-review DAH patients
  • 24 studies (one case-control, three small case series, 20 case reports)

Original publication

Risk and triggering factors for diffuse alveolar hemorrhage in primary antiphospholipid syndrome. An observational follow-up study and a systematic review of the literature. Semin Arthritis Rheum. Published 25 February 2025. doi:10.1016/j.semarthrit.2025.152697

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