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Primary biliary cirrhosis associated with antiphospholipid syndrome
1Department of Medicine A, Sourasky Medical Center, Tel Aviv University, Israel.
Israel Journal of Medical Sciences
|December 16, 1997
Summary
Primary antiphospholipid syndrome caused gangrene in a patient with liver enzyme abnormalities. Further tests excluded lupus, confirming the diagnosis and its association with primary biliary cirrhosis.
Area of Science:
- Vascular Medicine
- Autoimmune Diseases
- Hepatology
Background:
- Antiphospholipid syndrome is an autoimmune disorder associated with an increased risk of blood clots and pregnancy complications.
- Microvasculopathy is a disease of small blood vessels, often seen in autoimmune conditions.
- Primary biliary cirrhosis is a chronic liver disease affecting the bile ducts.
Observation:
- A 47-year-old female presented with severe pain and gangrene in her right foot's toes.
- Laboratory results showed liver enzyme abnormalities and positive antibodies including anti-mitochondrial, anti-phospholipid, and anti-nuclear.
- Systemic lupus erythematosus was excluded after a 6-year clinical follow-up.
Findings:
- The patient was diagnosed with primary antiphospholipid syndrome.
- The condition was complicated by microvasculopathy, leading to toe gangrene.
- The syndrome was associated with primary biliary cirrhosis.
Implications:
- This case highlights the potential for antiphospholipid syndrome to manifest with severe peripheral vascular complications.
- Early diagnosis and management of antiphospholipid syndrome are crucial to prevent devastating outcomes like gangrene.
- The co-occurrence of antiphospholipid syndrome and primary biliary cirrhosis suggests potential shared etiological factors or disease interactions.
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