Venous sinus thrombosis in a case of immunoglobulin A vasculitis and a systemic review of literature

Abstract

Objectives: To describe venous sinus thrombosis involved in immunoglobulin A (IgA) vasculitis and identify the clinical features and imaging findings of this rare disease.

Methods: We describe a case with venous sinus thrombosis related to IgA vasculitis, and a systematic review of previously reported cases in the literature.

Results: A 10-year-old boy presented with recurrent petechiae of the lower extremities with abdominal pain, and was diagnosed as having IgA vasculitis. He had a sudden headache during the treatment of steroids, and venous sinus thrombosis was diagnosed according to magnetic resonance imaging. Venous sinus thrombosis is a rare complication of IgA vasculitis. Based on the systematic review, most of these reported cases who developed venous sinus thrombosis had multi-system involvement, which included skin, joints, gastrointestinal tract or kidneys. Sudden headache was the most common symptom of central venous sinus thrombosis. Some cases may also manifest as seizures and blindness. The sagittal sinus was the most common site of thrombosis. Magnetic resonance image, magnetic resonance venography, or computed tomography angiography were helpful for early diagnosis of this disease. Combination therapy of steroids and anticoagulation medication was effective in rapidly reliving clinical symptoms.

Conclusions: Sudden headache in patients with IgA vasculitis requires vigilance for the possibility of central venous sinus thrombosis. Anti-inflammatories combined with anticoagulant therapy were needed for these patients.

Keywords: Factor VII; IgA vasculitis; hypercoagulability; venous sinus thrombosis.