Lupus & antiphospholipid syndrome
Pulmonary involvement in catastrophic antiphospholipid syndrome: A descriptive analysis from the “catastrophic antiphospholipid syndrome (CAPS) Registry”
Authors' conclusion
Pulmonary involvement may include both TMA and non-thrombotic inflammation, which can be differentiated into three patterns: PE, DAH with systemic TMA with hypocomplementemia or DAH without systemic TMA with/without hypocomplementemia.
Study context
- Population
- Patients with CAPS in the CAPS Registry with PE and/or diffuse alveolar hemorrhage (DAH)
- Outcome
- Pulmonary involvement in CAPS episodes
Methodological detailsretrospective, registry-based (CAPS Registry)
- Retrospective
- Registry-based (CAPS Registry)
Main finding
As reported in the abstract
PE
129 (48.6 %) episodes
DAH
75 (28.3 %) episodes
Overlap (DAH plus PE)
7 (2.6 %) episodes
Observational data: associations, not effects.
Further findings
Diagnostic procedures
- Bronchoalveolar lavage performed
- 35 (4.9 %) episodes
- Lung pathology samples (including autopsies)
- 84 (10.5 %) episodes
Associations with DAH and pathological thrombotic microangiopathy (TMA)
%
- Triple aPL positivity and pathological TMA
- 26.5
- Hypocomplementemia and DAH
- 24
- Significant relationship between DAH and laboratory features of thrombotic microangiopathy (TMA)
Original publication
Pulmonary involvement in catastrophic antiphospholipid syndrome: A descriptive analysis from the “CAPS Registry”. Semin Arthritis Rheum. Published 7 October 2023. doi:10.
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