Juvenile Primary Sjögren’s Syndrome with Ranula Development

Background: Juvenile primary Sjögren’s syndrome (pSS) is a rare disease and the occurence of ranula within it is extremely uncommon. Only few cases have been described in the literature so far but of course there may be unreported cases. Their best treatment within this condition is also unknown.

Objective: Here is reported a rare case of juvenile pSS in a patient with ranula. It is previously suggested that the extravasation of saliva from damaged ducts with lymphocytic infiltration in Sjögren’s syndrome patients can cause ranulas. The aim of this case report is to discuss the above mentioned claim and possible therapies.

Methods: A patient treated for Sjögren’s syndrome experienced ranula brings to the question whether other similar cases exist and how they are treated? The literature search was done and the personal experience is described.

Results: Very small number of patients with juvenile or adult onset Sjögren syndrome were noted in the literature. Described ranulas were treated surgically or they resolved naturally. This patient is treated with chloroquine and corticosteroids that enhanced her laboratory findings but did not help ranula regression. It is treated surgically in the end.

Conclusion: It seems that corticosteroid and chloroquine therapy does not have an infuence on ranula existence but this is a single patient experience so descriptions of other similar patients are needed to see whether Sjögren syndrome patients really develop ranulas and what is the best choice for their treatment.