Catastrophic antiphospholipid syndrome presenting with a stroke as a first presentation: case report

Area of Science:

• Rheumatology
• Immunology
• Nephrology

Background:

• Antiphospholipid Syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis.
• Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, life-threatening variant involving multiple organ systems.
• APS can be primary or secondary to other autoimmune diseases, such as Systemic Lupus Erythematosus (SLE).

Observation:

• A 31-year-old male presented with acute neurological deficits, including left-sided weakness, headache, and dysarthria.
• Diagnostic workup revealed myocardial injury (ST elevation, elevated cardiac enzymes), hemolytic anemia, and acute kidney injury.
• The patient also had elevated inflammatory markers and a prolonged activated partial thromboplastin time (aPTT).

Findings:

• The clinical presentation and laboratory findings were consistent with probable CAPS.
• The diagnosis was further supported by evidence of simultaneous brain, heart, and kidney involvement.
• Antiphospholipid antibodies (aPL) were positive, confirming the association with APS.
• The patient was diagnosed with probable CAPS secondary to SLE.

Implications:

• This case underscores the importance of early recognition and diagnosis of CAPS, even in young patients without prior medical history.
• The successful treatment with a combination of Methylprednisolone, Hydroxychloroquine, Colchicine, and Rituximab demonstrates a potential therapeutic strategy.
• Prompt management of CAPS secondary to SLE can lead to significant improvement in patient outcomes, particularly neurological function.