Objectives: First, establishment and validation of a novel questionnaire documenting the burden of xerostomia and sialadenitis symptoms, including quality of life. Second, to compare two versions regarding the answering scale (proposed developed answers Q3 vs. 0-10 visual analogue scale Q10) of our newly developed questionnaire, in order to evaluate their comprehension by patients and their reproducibility in time.Study Design: The study is a systematic review regarding the evaluation of the existing questionnaire and a cohort study regarding the validation of our new MSGS questionnaire.Materials and Methods: A Multidisciplinary Salivary Gland Society (MSGS) questionnaire consisting of 20 questions and two scoring systems was developed to quantify symptoms of dry mouth and sialadenitis. Validation of the questionnaire was carried out on 199 patients with salivary pathologies (digestive, nasal, or age-related xerostomia, post radiation therapy, post radioiodine therapy, Sjögren's syndrome, IgG4 disease, recurrent juvenile parotitis, stones, and strictures) and a control group of 66 healthy volunteers. The coherence of the questionnaire's items, its reliability to distinguish patients from healthy volunteers, its comparison with unstimulated sialometry, and the time to fill both versions were assessed.Results: The novel MSGS questionnaire showed good internal coherence of the items, indicating its pertinence: the scale reliability coefficients amounted to a Cronbach's alpha of 0.92 for Q10 and 0.90 for Q3. The time to complete Q3 and Q10 amounted, respectively, to 5.23 min (AE2.3 min) and 5.65 min (AE2.64 min) for patients and to 3.94 min (AE3.94 min) and 3.75 min (AE2.11 min) for healthy volunteers. The difference between Q3 and Q10 was not significant.Conclusion: We present a novel self-administered questionnaire quantifying xerostomia and non-tumoral salivary gland pathologies. We recommend the use of the Q10 version, as its scale type is well known in the literature and it translation for international use will be more accurate.
Objectives/Hypothesis
To compare the results of magnetic resonance imaging with magnetic resonance sialography (MRSIAL) and the clinical and laboratory characteristics in a well‐characterized cohort of patients with primary or secondary Sjögren's syndrome (SS) meeting the American–European Consensus Group criteria.
Study Design: Retrospective, observational, monocentric study.
Methods
Thirty‐six patients (81% female, mean age = 48 ± 35 years) with primary or secondary SS who underwent MRSIAL were included in the study.
Results
MRSIAL revealed characteristic radiological signs in the parotid, sublingual, and submandibular salivary glands in 35/36 patients (97%). Patients presenting with anti‐Sjögren's syndrome–related antigen A (SSA) autoantibodies showed more often fatty infiltration, a “pepper‐and‐salt” appearance, ductal stenosis, and/or ductal dilation of the parotid gland (88%, 88%, and 72% respectively) than patients negative for anti‐SSA (12%, 4%, and 28% respectively). MRSIAL demonstrated signs characteristic of SS in all 11 patients with negative minor salivary gland biopsy. For 15 patients undergoing ultrasound examination only, 11 (73%) had SS findings, but all 15 had SS findings on MRSIAL. Two cases of parotid lymphoma were detected by MRSIAL (6%).
Conclusions
MRSIAL is a reliable technique to detect glandular anomalies in patients with SS, and seems to provide a valuable aid in the diagnosis of SS.
Level of Evidence
4 Laryngoscope, 131:E83–E89, 2021
Background
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis with slow progression over the years that is particularly difficult to diagnose.
Cases
Here we report three cases of ECD without BRAF mutation presenting with a renal mass, hairy kidney appearance, and a rather benign course, for which the diagnosis of ECD was delayed, characterized by multiple investigations and unsuccessful treatments attempts. In two cases the distinction from IgG4-related disease required multiple investigations and reevaluation of the clinical, radiological, histological, and immunological characteristics.
Conclusion
A correct diagnosis of ECD may take several years and often requires revisiting previous hypotheses. Reassessment of histological slides and more modern complementary exams such as PET-CT or BRAF and MAPK-ERK mutation analysis can help to confirm the diagnosis of ECD and to select effective therapy.
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