Primary Sjogren’s syndrome manifesting with distal renal tubular acidosis and severe metabolic bone disease

Sjogren’s syndrome (SS) is a chronic, autoimmune, inflammatory disorder affecting primarily the salivary and lacrimal glands with potential for systemic involvement. The disease predominantly occurs in women in the age group of 35–45 years and is relatively rare in children. It mainly affects salivary and lacrimal glands with potential for systemic involvement. Children presenting with the severe metabolic bone disease at the very outset has not been reported in the paediatric literature. We report a 13-year-old girl who presented with pain in multiple large joints with predominant involvement of hip joints leading to difficulty in walking for the past 6 months and unintentional weight loss of the same duration. Investigations revealed distal renal tubular acidosis with severe metabolic bone disease as an extra-glandular manifestation of primary SS.