Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus

Abstract

A 42-year-old woman, a diagnosed case of systemic lupus erythematosus (SLE), developed severe headache followed by left hemiparesis and cortical blindness. Magnetic resonance imaging (MRI) of brain demonstrated right parieto-occipital infarct and the patient was initiated on high-dose steroids and antiplatelet agents with which the patient had clinical and radiological deterioration. Magnetic Resonance angiography showed severe narrowing of bilateral anterior, middle, and posterior cerebral arteries (PCA) suggestive of reversible cerebral vasoconstriction syndrome (RCVS). Patient was treated with IV nimodipine and she recovered over a period of 2 weeks. Repeat MR-angiography done on day-30 was normal. The diagnosis of RCVS in patients with SLE is of practical importance, because treatment and prognosis for SLE-associated cerebral vasculitis differ strongly from the treatment of RCVS. Empirical high-dose glucocorticoid therapy should be avoided in patients with typical features of RCVS.

Reversible cerebral vasoconstriction syndrome in paediatric patients with systemic lupus erythematosus: implications for management

“…Previously RCVS has been mostly described in adults, in a broad range of clinical situations. Reported cases of RCVS in adults with SLE are scarce . To our knowledge, no case of RCVS has been described in children with SLE.…”

“…Reported cases of RCVS in adults with SLE are scarce. [4][5][6] To our knowledge, no case of RCVS has been described in children with SLE. We report on three children with SLE, acute neurological symptoms, and transient intracranial large artery stenoses, for which the diagnosis of SLE-associated RCVS was a posteriori retained.…”

A systemic lupus erythematosus patient with thunderclap headache: reversible cerebral vasoconstriction syndrome

“…12 in SLE have been published in the literature (Table 2). 3,[13][14][15][16][17] In conclusion, the diagnosis of RCVS in SLE patients is important, because it has different treatment and prognosis from CNS vasculitis. Clinicians should pay attention to the fact that RCVS can be a cause of headaches and neurological abnormalities in patients with inactive SLE in order to effect proper management.…”

“…To our knowledge, only eight cases of RCVS in SLE have been published in the literature (Table 2). 3,13–17…”

A Case Report of Reversible Cerebral Vasoconstriction Syndrome in a Patient With Systemic Scleroderma

“…Why RCVS occurs remains unclear. There are some case reports in patients with systemic lupus erythematosus (SLE) with RCVS [ 4 , 5 ], but reports of RCVS are very limited in patients with systemic scleroderma. Here, we report a case of RCVS in a patient with systemic scleroderma with typical radiological change and a good response to treatment.…”