Intravenous immunoglobulin therapy for severe gastrointestinal involvement in systemic sclerosis

摘要

The pathogenesis of GI involvement in SSc is poorly understood, but is characterised by vascular and neuronal damage, inflammation and fibrosis. There is some evidence supporting mucosal hypoxia as the initial event in GI pathology, supported by studies showing reduced mucosal blood flow to the stomach and duodenum (4) and vascular insufficiency preceding smooth muscle atrophy (5). In addition, neuronal dysfunction may also play a role; with vascular damage to the vasa nervorum resulting in is-chaemia and nerve injury in addition to compression of the nerves by collagen tissue. Smooth muscle cells are often fragmented and atrophied, with no increase in collagen deposition (6). This is consistent with neural damage occurring before fibrosis, but whether the primary insult is vascular or neural remains uncertain.