Comparison between primary Sjögren’s disease patients with high or low level of dryness

Objectives To describe primary Sjögren’s disease (SjD) patients presenting no or low level of dryness and to compare them with SjD patients with oral or ocular dryness features.Methods All patients diagnosed with SjD according to AECG or ACR/EULAR criteria in our tertiary reference centre were included. Patients with high or low subjective symptoms or objective signs of dryness were compared.Results Overall, 509 patients were included for the comparison of patients with high (n=456) or low (n=53) level of subjective dryness and 472 for the comparison of patients with (n=359) or without (n=113) high objective dryness. Compared with patients with subjective dryness, patients without high subjective dryness were significantly younger (median 49 (39–62) years vs 58 (47–67) years, p<0.01), diagnosed earlier (median time from first symptoms to diagnosis 2 (0.5–4.5) years vs 4 (1–9.25), p=0.0056), more frequently anti-SSA positive ((83% vs 64%, p=0.008) and had less focal sialadenitis in minor salivary gland biopsy (69% vs 83%, p=0.02).The patients without high level of objective dryness (n=113) were also younger (51 (41–60) vs 58 (47–67) years, p<0.001) and were more frequently anti-SSA positive (79% vs 63%, p=0.002).In both groups, no difference was observed regarding disease activity.Conclusions Among the patients with SjD, those without high subjective or objective dryness features had a younger profile, a faster diagnosis which may result from a more acute onset, were more frequently anti-SSA positive than patients with high dryness features

Investigation of Vestibular Function in Adult Patients with Gitelman Syndrome: Results of an Observational Study

International audienceGitelman syndrome (GS) is a rare salt-losing tubulopathy caused by an inactivating mutation in the SLC12A3 gene, encoding the thiazide-sensitive sodium chloride cotransporter (NCC). Patients with GS frequently complain of vertigo, usually attributed to hypovolemia. Because NCC is also located in the endolymphatic sac, we hypothesized that patients with GS might have vestibular dysfunction. Between April 2013 and September 2016, 20 (22%) out of 90 patients followed at the reference center complained of vertigo in the absence of orthostatic hypotension. Sixteen of them were referred to an otology department for investigation of vestibular function. The vertigo was of short duration and triggered in half of them by head rotation. Seven patients (44%) had a vestibular syndrome. Vestibular syndrome was defined: (1) clinically, as nystagmus triggered by the head shaking test (n = 5); and/or (2) paraclinically, as an abnormal video head impulse test (n = 0), abnormal kinetic test (n = 4) and/or abnormal bithermal caloric test (n = 3). Five patients had associated auditory signs (tinnitus, aural fullness or hearing loss). In conclusion, we found a high frequency of vestibular disorder in GS patients suffering from vertigo, suggesting a role of NCC in the inner ear. Referent physicians of these patients should be aware of this extrarenal manifestation that requires specific investigations and treatment

Investigation of Vestibular Function in Adult Patients with Gitelman Syndrome: Results of an Observational Study

International audienceGitelman syndrome (GS) is a rare salt-losing tubulopathy caused by an inactivating mutation in the SLC12A3 gene, encoding the thiazide-sensitive sodium chloride cotransporter (NCC). Patients with GS frequently complain of vertigo, usually attributed to hypovolemia. Because NCC is also located in the endolymphatic sac, we hypothesized that patients with GS might have vestibular dysfunction. Between April 2013 and September 2016, 20 (22%) out of 90 patients followed at the reference center complained of vertigo in the absence of orthostatic hypotension. Sixteen of them were referred to an otology department for investigation of vestibular function. The vertigo was of short duration and triggered in half of them by head rotation. Seven patients (44%) had a vestibular syndrome. Vestibular syndrome was defined: (1) clinically, as nystagmus triggered by the head shaking test (n = 5); and/or (2) paraclinically, as an abnormal video head impulse test (n = 0), abnormal kinetic test (n = 4) and/or abnormal bithermal caloric test (n = 3). Five patients had associated auditory signs (tinnitus, aural fullness or hearing loss). In conclusion, we found a high frequency of vestibular disorder in GS patients suffering from vertigo, suggesting a role of NCC in the inner ear. Referent physicians of these patients should be aware of this extrarenal manifestation that requires specific investigations and treatment

A contribution to the debate on tinnitus definition

International audienceTinnitus is generally defined as an auditory perception in the absence of environmental sound stimulation. However, this definition is quite incomplete as it omits an essential aspect, the patient's point of view. This point of view constitutes, first and foremost, a global and unified lived experience, which is not only sensory (localization, loudness, pitch and tone), but also cognitive (thoughts, attentiveness, behaviors) and emotional (discomfort, suffering). This experience can be lived in a very unpleasant way and consequently have a very negative impact on quality of life. This article proposes and justifies a new definition for tinnitus elaborated by a group of French clinicians and researchers, which is more in line with its phenomenology. It also provides a minimum knowledge base, including possibilities for clinical care, hoping to eradicate all misinformation, misconceptions and inappropriate attitudes or practices toward this condition. Here is the short version of our definition: Tinnitus is an auditory sensation without an external sound stimulation or meaning, which can be lived as an unpleasant experience, possibly impacting quality of life

The 2016–2019 ImmunoTOX assessment board report of collaborative management of immune-related adverse events, an observational clinical study

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