Sir,
A 65-year-old normotensive, nondiabetic female presented to the emergency department with sudden onset quadriparesis of one day duration. She had history of multiple tooth extractions over the last 10 years. She also had dryness of mouth and eyes of same duration. On examination her pulse was 84/min, BP 130/70 mmHg and poor oral dental hygiene. Systemic examination was normal. The hemoglobin was 7.5 gm/dL, TLC: 6.5 × 109/L, N 76% L 14% M 5% E 5 %, PLT 121 × 109/L. Her random blood sugar was 132 mg/dL, urea 36 mg/dL, creatinine 1.1 mg/dL, bilirubin 0.52 mg/dL, AST 30 U/L, ALT 34 U/L, alkaline phosphatase 288 U/L, total protein 8.5 mg/dL, albumin 3.5 mg/dL. The urinealysis ECG and chest skiagram were normal.
Arterial blood gas analysis showed ABG-pH of 7.32, HCO3 11.3 mmol /L, pO2 63 mm Hg pCO2 29.4 mm Hg, Na 134 mmol/L, K 1.5 mmol/L. and urinary pH 6.5. The Schirmer's test indicated severely dry eyes (<4 mm). Histopathology of salivary gland revealed chronic mononuclear cell infiltration with mild atrophy of glandular component consistent with Sjogrens syndrome. The patient did not afford antibody testing.
The diagnosis of Sjogren syndrome was established as our patient fulfilled four out of the six criteria of the revised international classification criteria for Sjogren syndrome. The patient's symptoms improved dramatically after correcting serum potassium and patient was discharged on potassium supplements and oral sodium bicarbonate tablets.
The overall prevalence of Sjogren syndrome in India is low. Porkodi et al.[1] reported 36 patient of Sjogren syndrome over a period of 4 1/2 year out of 8000 patients seen with musculoskeletal symptoms amounting to 0.0045%. In another report from Lucknow, only 26 cases of Sjogren’ syndrome were described over a period of 10 years.[2] The low prevalence can be due to lack of awareness.
Type 1 renal tubular acidosis presenting as hypokalemic paralysis in Sjogren syndrome has been reported globally.[3] It may be the first presentation of Sjogren syndrome despite long duration of exocrine gland involvement or it may precede the sicca symptoms. Chen et al.[4] found that renal tubular acidosis was the initial manifestation of primary Sjogren's syndrome in 75% out of a series of eight cases. The disease duration was shorter in patients with renal involvement than in those with normal acidification results.[5] Therefore, in patients presenting as hypokalemic paralysis a clinical suspicion can unmask a sub clinical Sjogren syndrome.
References
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