Clinical presentation and salivary gland histopathology of paediatric primary Sjogren's syndrome

ObjectiveExplore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjogren's syndrome (PPSjS).MethodsA case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded.ResultsAge at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 318 fulfilled AECG-2002 diagnostic criteria, throughout the disease course.ConclusionPPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group.

ASPECTOS ETIOLOGICOS DA HIPERNASALIDADE CONSEQUENTE A PARALISIA DE PALATO MOLE - RELATO DE 6 CASOS

The authors studied the main aspects of etiology of the palate paralysis in six children suffering from soft palate paralysis. They emphasized the importance of multidisciplinary approach in the management of patients with this disease. In the author's cases the most likely etiologies were: neuropathy post-viral epidemic parotitis, tumors localized in the posterior cerebral fossa and idiopathic. They concluded that it is extremely important in these patients a detailed otorhinolaryngologic, neurologic and fonoaudiologic examinations.

Parotidite aguda em adulto jovem sistemicamente saudável: relato de caso clínico

This study reports an unusual case of acute parotitis in a healthy young adult with good oral hygiene. Case Report: The patient sought the Department of Stomatology, at Ponta Grossa State University due to a swollen on the right parotid gland region. The patient underwent clinical and radiological treatments and was prescribed anti-inflammatory drugs and antibiotics. On the fifth day the signs and symptoms had disappeared. Conclusion: The development of acute parotitis in healthy young adults with good oral hygiene is possible, but this makes the diagnosis more difficult. Anti-inflammatory, antibiotic and heat physiotherapy can contribute to the recovery of the signs and symptoms after 5 days of treatment.