Scleroderma's Possible Dual Role in the Pathophysiology of Intracranial Aneurysms: Case Report and Literature Review

BACKGROUND: Scleroderma, or systemic sclerosis, is an autoimmune disorder affecting connective tissues, including blood vessels. Although the exact mechanism is not understood, it results in the production of an abnormal amount of collagen. Cases have been reported in which patients with scleroderma also had intracranial aneurysms. We wish to gain insight into any potential association between the 2 diseases. CASE DESCRIPTION: We reviewed the literature of scleroderma cases with cerebral aneurysms and added our own case, focusing on patient and aneurysm characteristics. Including the present case, this paper pertains to 11 cases with 26 aneurysms. Fifty-five percent had multiple aneurysms, of which two thirds had >2. When data were available, 35% of aneurysms were >= 1 cm, 12% of which were giant. Four aneurysms were fusiform (15%). Patients presented with subarachnoid hemorrhage in 45% of cases. - CONCLUSIONS: Scleroderma may lead to the formation of an aneurysm, and the abnormal growth of collagen may protect it from early rupture, allowing it to reach a large size.