Lung Disease in Antiphospholipid Syndrome

Area of Science:

• Rheumatology
• Pulmonology
• Hematology

Background:

• Antiphospholipid syndrome (APS) is an acquired autoimmune disorder characterized by recurrent thrombosis and/or pregnancy morbidity.
• Persistent positivity of circulating antiphospholipid antibodies (aPLs) is the hallmark of APS.

Observation:

• Pulmonary manifestations are increasingly recognized in APS patients.
• Pulmonary embolism (PE) is the most common lung complication, occurring in 14.1% of APS patients.
• Pulmonary hypertension (PH) affects 1.8–3.5% of APS patients, with varied underlying mechanisms.

Findings:

• PE in APS is primarily thrombotic, suggesting aPL testing in young individuals with unprovoked PE.
• PH in APS can stem from post-PE, connective tissue disease, or Libman-Sacks endocarditis.
• Rare lung manifestations like alveolar hemorrhage and fibrosis are noted, with unclear pathogenic roles for aPLs.

Implications:

• Understanding aPL-associated lung disease is vital for timely diagnosis and treatment.
• Further research is needed to clarify the role of aPLs in rarer pulmonary complications.
• This review highlights the clinical spectrum, etiology, and management of lung manifestations in APS.