Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria.

OBJECTIVE: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. METHODS: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. RESULTS: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. CONCLUSION: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.

Surgical Treatment of Chronic Parotitis

Abstract Introduction chronic parotitis (CP) is a hindering, recurring inflammatory ailment that eventually leads to the destruction of the parotid gland. When conservative measures and sialendoscopy fail, parotidectomy can be indicated. Objective to evaluate the efficacy and safety of parotidectomy as a treatment for CP unresponsive to conservative therapy, and to compare superficial and near-total parotidectomy (SP and NTP). Methods retrospective consecutive case series of patients who underwent parotidectomy for CP between January 1999 and May 2012. The primary outcome variables were recurrence, patient contentment, transient and permanent facial nerve palsy and Frey syndrome. The categorical variables were analyzed using the two-sided Fisher exact test. Alongside, an elaborate review of the current literature was conducted. Results a total of 46 parotidectomies were performed on 37 patients with CP. Neartotal parotidectomy was performed in 41 and SP in 5 cases. Eighty-four percent of patients was available for the telephone questionnaire (31 patients, 40 parotidectomies) with a mean follow-up period of 6,2 years. Treatment was successful in 40/46 parotidectomies (87%) and 95% of the patients were content with the result. The incidence of permanent and transient facial nerve palsy was 0 (0%) and 12 (26.1%), respectively. Frey syndrome manifested in 20 (43.5%) patients. Neither this study nor careful review of the current literature resulted in evident difference between SP and NTP regarding the primary outcome variables. Conclusion parotidectomy is a safe and effective treatment for CP in case conservative therapy fails. There is no evidence of a distinct difference between SP and NTP regarding efficiency, facial nerve palsy or Frey syndrome. (AU)

Role of Salivary Duct Morphology in the Etiology of Chronic Obstructive Parotitis: Statistical Analysis of Sialographic Features and Computational Fluid Dynamics Analysis of Salivary Flow.

PURPOSE: The causes of some cases of chronic obstructive parotitis (COP) without obstructive factors are still unclear. The authors hypothesized that some morphologic features of salivary ducts might contribute to the development of COP. This study investigated the role of salivary duct morphology in the etiology of COP. MATERIALS AND METHODS: The authors designed and implemented a case-and-control study. Cases were defined as patients with COP, diagnosed from September 2014 to May 2017 at the Affiliated Hospital of Stomatology of the Sun Yat-sen University (Guangzhou, China), and controls were healthy participants. The primary predictor variables were the occurrence of an accessory duct (AD), the number of branches uniting to form the Stensen duct (SD), the angle between the AD and the SD, and the angle between branches identified on sialographic computed tomograms. Data from the 2 groups were compared to investigate the association between these variables and COP. The χ2 test, Student t test, and logistic regression were computed, with significance set at a P value less than .05. Fluid dynamics analysis was used to analyze salivary flow field in models of salivary ducts with different morphologic features reconstructed from sialographic computed tomograms. RESULTS: The sample was composed of 39 patients with COP and 18 controls without COP. The 2 groups were not similar for incidences of an AD (71.8 vs 38.9%) and the angle between branches (96.5 ± 26.0° vs 71.5 ± 21.2°). There was no relevant difference between groups in the number of branches and the angle between the AD and the SD. The area of low velocity was larger in the model with the wider angle between branches. CONCLUSIONS: The results suggest that the presence of an AD and a wider angle between duct branches are associated with COP.

Pneumoparotitis as a complication of long-term oronasal positive airway pressure for sleep apnea.

BACKGROUND: Parotid swelling is rarely caused by pneumoparotitis from retrograde insufflation of air into Stensen's duct. Previous reports have identified occupational exposures, self-induced habits, exercise, spirometry, and short-term positive pressure airway ventilation as causes of salivary duct insufflation. METHODS: We present 2 cases of pneumoparotitis in patients on long-term oronasal continuous positive airway pressure (CPAP) for obstructive sleep apnea. RESULTS: A diagnosis of pneumoparotitis was made by CT scan in case 1 and sialography in case 2. Patients were advised to transition from oronasal to nasal-only CPAP. One patient was successfully transferred and had good symptomatic improvement, whereas the second patient did not tolerate nasal CPAP and had persistent symptoms on oronasal CPAP. CONCLUSION: Long-term use of oronasal CPAP is a potential cause of pneumoparotitis.

Role of the Accessory Parotid Gland in the Etiology of Parotitis: Statistical Analysis of Sialographic Features.

This retrospective study aimed to identify if the existence of the accessory parotid gland correlated with the etiology of parotitis. This may aid the development of better treatment strategies in the future. Sialographic features of cases with parotitis and healthy subjects were reviewed. The chi-square test was used to compare the incidence of accessory parotid gland between the groups. The Student's t test was used to compare the length of Stensen's duct, the length from the orifice to the confluence of the accessory duct, and the angle between the accessory duct and Stensen's duct between the groups. The incidence of accessory parotid gland in patients with parotitis was 71.8% (28/39), which was significantly higher than that in healthy subjects (P = 0.005). Patients with parotitis had a longer Stensen's duct than healthy subjects (P = 0.003). There was no significant difference in the length from the orifice to the confluence of the accessory duct or the angle between the accessory duct and Stensen's duct (P = 0.136 and 0.511, respectively) between the groups. The accessory parotid gland might play a role in the pathogenesis of parotitis. The existence of an accessory parotid gland is likely to interfere with salivary flow. Computational fluid dynamics analysis of salivary flow in the ductal system would be useful in future etiologic studies on parotitis.

Diagnosis and management of pediatric salivary gland infections.

The incidence of salivary gland infections in the pediatric population is low but not infrequently seen in pediatric oral and maxillofacial surgery practices and hospital environs. With an ever increasing armamentarium of diagnostic tools and medical and surgical therapies, these patients can be managed successfully with minimum morbidity and decreased incidence of recurrences.

Value of apparent diffusion coefficient calculation before and after gustatory stimulation in the diagnosis of acute or chronic parotitis.

The purpose of the study was to investigate the value of diffusion-weighted (DW) echo-planar imaging (EPI) for quantifying physiological changes of the parotid gland before and after gustatory stimulation in patients suffering from acute or chronic recurrent inflammation in comparison with healthy volunteers. Using a DW-EPI sequence at 1.5 T, parotid glands of 19 consecutive patients with acute (n=14) and chronic (n=5) inflammation of parotid glands and 52 healthy volunteers were examined. Magnetic-resonance (MR) images were obtained before and after gustatory stimulation with 5 cc of lemon juice. In volunteers mean ADC values of 1.14 x 10(-3) mm(2)/s before and 1.2 x 10(-3) mm(2)/s after gustatory stimulation were observed. In acute inflammation ADC values were higher before [1.22 x 10(-3) mm(2)/s (p=0.006)] and after stimulation [1.32 x 10(-3) mm(2)/s (p<0.001)]. Before stimulation ADC differences between chronic inflammation (1.05 x 10(-3) mm(2)/s) and healthy volunteers (p=0.04) as well as between acute and chronic inflammation (p=0.005) were statistically significant. No differences were detected after stimulation between chronic inflammation (1.2 x 10(-3) mm(2)/s) and healthy volunteers (p=0.94) and between acute and chronic inflammation (p=0.15), respectively. DW-EPI seems to display the physiological changes of the parotid gland in patients suffering from acute or chronic inflammation and might be useful for discriminating healthy from affected glands.

Is recurrent parotitis in childhood still an enigma? A pilot experience.

AIM: To test the hypothesis that dental malocclusion with mandibular misplacement may be a causative factor for recurrent parotitis (RP) through unbalancing of masticatory muscles. METHODS: Thirteen patients (age 4-14 years) who were referred to a dental clinic for RP and malocclusion were treated by oral appliance positioning for a 6-month period. Monthly visits were scheduled regularly. RESULTS: Symptoms were clearly improved in nine children. No effect was obtained in three patients. One patient was lost at follow-up. CONCLUSION: Occlusal intervention is effective in patients with RP and associated malocclusion. It should be considered an important option for the treatment of such intriguing disorder.

Lesiones bucales en niños VIH/Sida y su relación con linfocitos CD4 y carga viral: Valencia / Mouth injuries in children with HIV/AIDS and their relation with CD4 limphocytes and viral load

El 90 por ciento de los pacientes VIH-SIDA presentan lesiones bucales durante el curso de la enfermedad, observándose en la mayoría de los niños en las etapas iniciales, lesiones muchas veces no imputables al VIH. Determinar las manifestaciones bucales y su relación con el porcentaje de linfocitos CD4 y la carga viral, en 40 niños VIH/SIDA que acudieron a la consulta de infectología y odontología pediátrica de la Ciudad Hospitalaria “Dr. Enrique Tejera”. A través de un estudio descriptivo y de campo, se analizaron las variables, edad, género, categoría clínico-inmunológica, manifestaciones en tejidos blandos y duros, porcentaje de linfocitos CD4 y carga viral. El grupo de niños más afectado fue el de 1 a 6 años y el género femenino. La categoría clínica inmunológica más frecuente fue la B2 y la C3. En 65 por ciento hubo caries y en 80 por ciento lesión de tejidos blandos. La lesión bucal más común fue: adenopatías 57.5 por ciento, seguida de candidiasis 30 por ciento, y en igual proporción (17,5 por ciento) afta-úlceras y gingivitis; parotiditis y xerostomía 12.5 por ciento, herpes oral y petequias 10 por ciento, queilitis 7.5 por ciento, leucoplasia vellosa y eritema gingival lineal 2.5 por ciento. Independientemente del porcentaje de CD4 y carga viral, se evidenciaron manifestaciones bucales en tejidos duros y blandos, en una frecuencia relativamente alta, de allí la imperiosa necesidad de institucionalizar los programas de atención odontológica, así como la instrucción, sensibilización y motivación a los padres, representantes y personal de salud en el área pediátrica, sobre la importancia de la salud bucal en los niños VIH/SIDA.

90 percent of the childrens infected with VIH-AIDS may be at increased risk of experiencing oral lesions during the course of the illness, principally at the initial stage, the most of these lesions were not associated to HIV. To determine the oral manifestations and their relation with the percentage of lymphocytes CD4 and the viral load, in 40 children being treated for HIV-infection of the Paediatric Infection and Dentistry Service at the “Ciudad Hospitalaria Dr. Enrique Tejera.”. By means of a descriptive study of field, the variables age, gender, immunological clinical category, soft and hard tissue manifestations, percentage of lymphocytes CD4, and viral load were analyzed. The childrens more affected were the group of 1 to 6 years old and in feminine gender. The most frequent immunological clinical category was the B2 and the C3. In 65 percent there was dental caries, and in 80 percent soft oral lesion. The most common oral manifestation was adenopaty 57,5 percent, followed of candidacies 30 percent, and in equal proportion 17.5 percent aphthous ulcerations, and gingival erythema, parotid en largement and xerostomia 12,5 percent, herpes oral and petechiae 10 percent, cheilitis 7,5 percent, hairy leukoplakia and gingival erythema linear 2.5 percent. Independently of percentage CD4 and the viral load, oral manifestations on hard tissue were recorded as much as in soft tissue. The hard and soft weave injuries were relatively high, consequently, the urgent necessity to institutionalize preventive and therapeutic dental program, and also the education, sensibilization and motivation to parents, representatives and health personnel in the paediatric area; about the importance of oral health in HIV/AIDS children.

Differential responses of rostral subnucleus caudalis and upper cervical dorsal horn neurons to mechanical and chemical stimulation of the parotid gland in rats.

Blockage of the salivary duct can produce pain and inflammation from the build up of saliva in the parotid gland. The processing of parotid inflammation-induced pain, however, is poorly understood. The purpose of this study was to clarify the functional involvement of the trigeminal subnucleus interpolaris/caudalis transition region (Vi/Vc) and upper cervical spinal cord (C1/C2) in processing nociceptive input relevant to parotitis. The effect of capsaicin-induced parotitis was examined on a total of 37 nociceptive neurons isolated from the Vi/Vc (n = 23) and C1/C2 (n = 14) regions. Eight of 23 Vi/Vc neurons responded to mechanical distention of the parotid gland, whereas no C1/C2 neurons responded to the parotid distention. Receptive field characteristics in all neurons were examined following capsaicin injections into the parotid gland. Mechanical and cold responses increased significantly in C1/C2 but not Vi/Vc neurons following capsaicin. Receptive field sizes also increased in C1/C2 but not Vi/Vc neurons. At the Vi/Vc transition region, pinch-evoked activity increased in neurons receiving convergent inputs from the parotid gland and facial skin when compared to non-convergent neurons. The present data indicate that the hyperalgesia and referred pain associated with parotitis may result from sensitization of C1/C2, but not Vi/Vc nociceptive neurons.

Parotitis due to organophosphate intoxication.

Acute pancreatitis due to pancreatic exocrine over-secretion induced by organophosphate poisoning has been previously reported but parotid gland involvement has not. This paper describes a case of acute organophosphate-induced parotitis in a patient with pre-existing sialolithiasis. The patient developed bilateral facial swelling in the pre-auricular area extending to the angle of the jaw and also developed elevated serum amylase on the second day of the poisoning. Serum lipase remained normal. Autopsy confirmed parotid gland inflammation and pre-existing ductal lithiasis. This case illustrates that organophosphate-induced parotitis can occur and should be considered in patients with organophosphate poisoning who have hyperamylasemia without elevation in serum lipase.

Chronic parotitis: diagnosis and treatment.

BACKGROUND: Chronic parotitis, or CP, is a nonspecific sialadenitis that often is seen first in the dental office. The cause, although not definitively determined, is most likely multifactorial and includes decreased salivation, stasis and an ascending retrograde duct infection. The authors present a case report to illustrate the symptomatology of CP to facilitate its differentiation from other entities that mimic CP. CASE DESCRIPTION: A 60-year-old woman with a 30-year history of recurrent swellings of her left parotid gland was diagnosed with CP. The diagnosis was based on history, clinical examination, salivary volume and chemistry, computerized tomographic scan and sialography. Treatment was palliative in nature. CLINICAL IMPLICATION: As a member of the health care team, the dentist must be familiar with the various causes of recurrent parotid infections. Early clinical recognition of CP leads to appropriate and successful care.

[Recurrent parotitis in children and HIV infection. Apropos of 4 cases]. / Parotidites récidivantes de l'enfant et infection HIV. A propos de 4 observations.

We report four cases of recurrent infectious parotiditis in children and recall the clinical radiographic and pathogenic features. Particular attention was paid to the possible relationship between sialadenitis and HIV infection in two cases. This would open a large etiological field when HIV contamination is suspected. Treatment if all four cases (spiramycine, diclofenac, soframycin washing, lipiodol instillation and local bucco-dental treatment) was successful leading to longer intervals between relapses.

Gland atrophy following retrograde injection of methyl violet as a treatment in chronic obstructive parotitis.

OBJECTIVE: The purpose of this study was to evaluate the clinical effectiveness of retrograde injection of 1% methyl violet as a treatment for chronic obstructive parotitis. STUDY DESIGN: Sixteen patients with chronic obstructive parotitis were treated with retrograde injection of 1% methyl violet. Pretreatment evaluation and assessment of the treatment effect were performed by clinical and imaging methods, including sialography and sonography of the treated glands. RESULTS: Clinical symptoms following treatment included initial acute swelling and then a decrease in parotid swellings; the final outcome was a clinical cure of the affected glands characterized by disappearance of all symptoms, absence of secretion, and complete obliteration of the main duct orifice. Posttreatment imaging showed total atrophy of the diseased glands. CONCLUSION: Retrograde injection of 1% methyl violet caused total atrophy of the diseased glands and brought about complete relief in all 16 patients, with no detectable side effects.