Introduction:

the Syndrome of Sjögren is an exocrinopatía autoinmune characterized by an infiltrated inflammatory, with linfocitos prevalence in glands exocrinas and knitted extraglandulares. The glandular lesion is characterized by the hiposecreción in mucous oral and ocular.

Clinical case:

a 13-year-old female adolescent with Sjögren's Syndrome is presented, whose initial clinical manifestation was extra-glandular, with the kidney as the main affected organ in type-I distal renal tubular acidosis.

Conclusions:

it is demonstrated that the realization of a meticulous anamnesis, clinical exploration and the use of different diagnostic tools, they make possible a precocious diagnosis of the illness and of their manifestations extraglandulares.

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