Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.
Sjogren's Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.
The persistent primitive hypoglossal artery (PPHA) is a carotid-basilar anastomosis. It may be associated with aneurysms, arteriovenous malformations and atherosclerotic disease 1 . We report a case of brain stem ischemia in a young patient due to internal carotid stenosis and PPHA.
CASE REPORTA 34-year-old Caucasian male with hypertension due to polycystic kidney disease was admitted into the hospital presenting sudden left paresthesia, quickly followed by transient left hemiplegia and hypoesthesia. Magnetic resonance imaging (MRI) at emergency showed a slight hypersignal in T2 and FLAIR on the topography of the pons. Magnetic Resonance Angiography displayed the presence of the PPHA and the stenosis of the right internal carotid artery, inferior to 50% (Figs 1 and 2). The patient was prescribed to aspirin 325 mg/day and atorvastatin 80 mg/day, with progressive recovering of the neurological deficits and with a good outcome in 12-month follow-up period.
DISCUSSIONThe PPHA is one of the carotid-basilar anastomosis, which also include the persistent trigeminal, otic and proatlantal intersegmental arteries 1 . These arteries are named according to their associated cranial nerves. They serve as anastomoses between the primitive internal carotid artery and the basilar artery . PPHA may also be associated to kidney polycystic disease, which was observed in this case. It may be also associated with
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