Diffuse large and small bowel necrosis in catastrophic antiphospholipid syndrome

Area of Science:

• Rheumatology
• Vascular Medicine
• Gastroenterology

Background:

• Antiphospholipid syndrome (APS) is an autoimmune disorder associated with an increased risk of thrombosis.
• The catastrophic variant of APS (CAPS) is a rare, life-threatening condition characterized by rapid onset of widespread thrombosis affecting multiple organs.
• Subacute cutaneous lupus erythematosus (SCLE) is a photosensitive, non-scarring rash associated with systemic lupus erythematosus.

Observation:

• A patient with SCLE presented with acute abdominal pain, evolving into diffuse right colon and small bowel necrosis.
• The patient developed acute respiratory distress syndrome, thrombocytopenia, and disseminated intravascular coagulation, indicative of CAPS.
• Histopathology revealed extensive arteriolar and venous thrombosis in resected bowel tissue, with mesenteric artery stenosis due to intimal fibrosis.

Findings:

• The patient's condition rapidly progressed to multiorgan failure despite aggressive treatment including anticoagulation, steroids, hemofiltration, and plasma exchange.
• Diffuse intestinal necrosis was a significant complication, necessitating extensive surgical resection.
• Despite intensive medical and surgical interventions, the patient succumbed to the catastrophic complications of CAPS.

Implications:

• This case highlights the critical need for a high index of suspicion for CAPS in patients presenting with ischemic bowel and features of thrombotic microangiopathy.
• Early recognition and prompt initiation of treatment are paramount for improving outcomes in catastrophic antiphospholipid syndrome.
• The association between SCLE and CAPS underscores the complex interplay of autoimmune conditions and thrombotic events.