Table 2. Learning points.

Learning points in primary Sjögren’s syndrome (pSS)
pSS is a complex, multisystem disease.
Presence of systemic disease in the setting of pSS is an important prognostic determinant.
Kidney involvement accounts for ~ 5% of pSS patients.
The most common renal manifestation is tubulointerstitial nephritis, which can manifest as RTA.
In the setting of unexplained dRTA, consider pSS.
CPM is a rare neurological manifestation of pSS.
CPM is potentially fatal but can have favorable outcome if managed timeously.
Treatment with aggressive intravenous sodium bicarbonate requires careful monitoring due to the risk of CPM with rapid serum sodium changes.

pSS = primary Sjögren’s syndrome; RTA = renal tubular acidosis; dRTA = distal renal tubular acidosis; CPM = central pontine myelinolysis.