Type 1 Renal Tubular Acidosis with Hypokalemic Quadriparesis in Sjogren Syndrome.

Unlabelled: Sjogren syndrome is an autoimmune disease characterised by lymphocytic infiltration and inflammation of the exocrine glands resulting in decreased secretion of involved glands which manifests mostly as dry eye and dry mouth. The prevalence of the disease is reported to be about 10.3 per 10,000 population. It is more common in females with a male: female ratio of 16:1. Extra glandular manifestations are seen in up to 1/3rd of the cases. Renal involvement is seen in 4.9% of patients with Sjogren syndrome.

Material: Here we present three cases of Sjogren Syndrome who presented to our hospital with hypokalaemic quadriparesis.

Observation: On evaluation all three of the patients were found to have renal tubular acidosis type 1. None of these patients had any symptom of Sjogren syndrome before the onset of quadriparesis. All of these patients had acute onset progressive areflexic quadriparesis with involvement of facial muscles and drooping of eyelids without sensory or bladder bowel involvement. One of these patients had respiratory muscle paralysis severe enough to mandate mechanical ventilation. Arterial Blood Gas analysis and urine electrolyte analysis were suggestive of type 1 renal tubular acidosis. ANA positive in 2 of the 3 patients. Anti-SSA & anti-SSB antibodies were positive in all three patients. Supportive measures and IV fluid and electrolyte correction was done. There was complete recovery of power in all three patients and were discharged on oral medications.Renal Tubular Acidosis is characterised by inability of the nephrons to maintain physiologic acid base balance. This usually results from a defect in the tubular transport mechanisms. Distal Renal tubular acidosis (as in these patients) is further defined by an alkalotic urinary pH(>5.5) and profound hypokalemia due to impairment in H+ secretion in ditstal tubular alpha-intercalated cells. Owing to this imbalance of ionic transport in distal tubules there can be nephrocalcinosis, nephrolithiasis, rickets and severe muscle weakness. Sjogren syndrome is one of the etiologies leading to development of T1RTA.T1RTA can be the presenting feature of Sjogren Syndrome.

Conclusion: Though a rare manifestation of the disease if can be the presenting symptom. Work up for RTA (ABG, urine electrolytes, Urine PH and osmolarity etc) in patients with hypokalaemic paresis can help establish the etiological diagnosis(ANA, anti-SSA,anti-SSB) and help prevent future relapses of the disease.