OBJECTIVE: To review the most common types of primary and secondary headaches that are associated with Sjogren's disease (SjD). BACKGROUND: Sjogren's disease is a chronic systemic autoimmune disease that typically presents with xerophthalmia, xerostomia, and arthralgias. Sensory and motor neuropathies are commonly reported neurological complications with SjD. Primary and secondary headaches are also frequent neurological complications associated with SjD, which may be under-recognized. METHODS: In this study, researchers conducted a literature review through the platforms of PubMed and Google Scholar with the keywords: (1) Sjogren's syndrome, (2) Sjogren's disease, (3) headache, (4) migraine, (5) tension-type headache, (6) aseptic meningitis, (7) cerebral venous sinus thrombosis, and (8) idiopathic intracranial hypertension. Included articles involved a study population of patients with both SjD and headache. There were no exclusion criteria. A total of 54 articles were identified, and 31 articles were utilized for study analysis. RESULTS: In patients with SjD, headaches were a frequently reported neurological complaint. The most common types of primary headaches associated with SjD were found to be migraine and tension-type headache. Patients with SjD were more likely to report severe, debilitating headaches compared to the general population. Furthermore, patients with SjD have been found to experience dangerous types of secondary headaches, including aseptic meningitis and cerebral venous sinus thrombosis. CONCLUSIONS: Headaches are a common neurological complication in patients with SjD. Current literature suggests that there is an increased risk of both primary and secondary headaches in individuals with SjD compared to the general population and that headaches may occur throughout the clinical course of the disease. There are also reports of patients who experience the onset of primary and secondary headaches prior to the diagnosis of SjD. SjD has been linked to dangerous, life-threatening conditions that cause headaches, which require urgent recognition and treatment. Therefore, it is important to have a higher index of suspicion for patients who present with headaches and clinical symptoms of SjD, such as xerophthalmia and xerostomia. Utilizing a detailed history, physical examination, and neuroimaging can assist clinicians to recognize and diagnose types of headaches in patients with SjD to prevent dangerous complications.
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