Rheumatologic diseases and posterior reversible encephalopathy syndrome: two case reports and review of the literature

Abstract

Rheumatologic diseases have varied clinical presentations, and posterior reversible encephalopathy syndrome (PRES) can be one of their presentations. The exact etiology of PRES is unknown, but endothelial dysfunction and immunosuppressive medications seem to be the likely cause in rheumatologic diseases. Clinical features include headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. The diagnosis of PRES can be difficult because several neuropsychiatric illnesses are generally prevalent in rheumatologic diseases; however, a high index of suspicion among physicians along with neuroimaging can help in the accurate diagnosis. Treatment guidelines are lacking, but in a few case series, lowering the blood pressure, controlling the seizures, and removing the immunosuppressive drugs have shown good results. There is need for randomized controlled trials addressing the treatment of PRES in rheumatologic diseases. Medline search was done from year 1950 to March 2011 using "posterior reversible encephalopathy" as keyword, and articles relevant to rheumatology were reviewed. We found 48 case reports showing PRES in patients with rheumatologic disease. Most of the patients were female. Age range was from 6 to 59 years. Out of the 48 case reports, 38 patients had systemic lupus erythematosus and most of them had renal disease. Five patients with autoimmune diseases presented with PRES after being started on immunomodulatory drugs. The most frequent symptoms were headache, seizures, and visual changes.

Corticosteroid therapy and severity of vasogenic edema in posterior reversible encephalopathy syndrome

“…Our finding of frequent steroid exposure prior to PRES is consistent with numerous case reports demonstrating a temporal association between steroid exposure and PRES [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48]. In our cohort and in the literature, steroid therapy is often co-administered with other potential culprit drugs or administered in the context of nephritis, which causes hypertension.…”

“…However, steroids are also used in the treatment of vasogenic edema, such as in patients with intracranial mass lesions [9]. Case reports have implicated steroids as both the precipitant [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48] and treatment [1, 6, 8, 12, 13, 18, 25, 28, 30, 33, 36, 37] of PRES. Thus, the role of steroids in PRES remains unclear.…”

The Skin and Neurologic Disease

“…In addition, there is the more localized posterior reversible encephalopathy syndrome (PRES), presenting with headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. 73 Although occurring with other autoimmune disorders, this rare encephalopathy is encountered most commonly in association with systemic lupus.…”

Steroid-responsive Thalamic Lesions Accompanying Microbleeds in a Case of Hashimoto's Encephalopathy with Autoantibodies Against α-enolase

“…Because sympathetic innervation of the vertebrobasilar circulation is relatively sparse, it is possible that blood-brain barrier disruption predominantly develops in the posterior circulation in patients with renal disease. Dhillon et al recently reviewed the clinical features of posterior reversible encephalopathy syndrome in patients with autoimmune disease (17). Notably, 38 of 48 patients with autoimmune disease had systemic lupus erythematosus and most also had renal disease.…”