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Upper-extremity digital ischemia in granulomatosis with polyangiitis
Sian Yik Lim1, Joon Hee Lim, Claire Horn
1From the *Department of Internal Medicine, Texas Tech University Health Science Center, Lubbock; and †Division of Rheumatic Diseases, University of Texas Southwestern Medical Center, Dallas, TX.
Summary
Granulomatosis with polyangiitis (GPA) can cause rare upper-extremity digital ischemia due to artery occlusion. This case highlights diagnostic and therapeutic challenges in managing this severe GPA complication.
Area of Science:
- Vascular Surgery
- Rheumatology
- Internal Medicine
Background:
- Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis.
- GPA typically affects the upper and lower respiratory tracts and kidneys.
- Digital ischemia is an uncommon manifestation of GPA.
Observation:
- A 48-year-old man presented with acute kidney injury, upper airway symptoms, and lower extremity swelling.
- The patient was diagnosed with GPA based on clinical presentation, renal biopsy, and positive ANCA.
- He developed cyanosis and digital ischemia in both upper extremities during his illness.
Findings:
- Histopathology revealed occlusion of middle- and large-sized arteries in the upper extremities.
- Despite immunosuppression, plasmapheresis, and antithrombotic therapy, digital ischemia progressed.
- The patient experienced autoamputation of his fingers.
Implications:
- This case underscores the potential for GPA to cause severe vascular complications affecting the extremities.
- The rarity of digital ischemia in GPA presents significant diagnostic and therapeutic challenges.
- Multidisciplinary management is crucial for patients with GPA and vascular compromise.
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