Hypokalaemic paralysis revealing Sjögren syndrome in an elderly man.

Abstract

A 73 year old white man presented with life threatening hypokalaemic paralysis requiring admission to an intensive care unit. Biochemical investigations showed severe hypokalaemia with hyperchloraemic metabolic acidosis, a spot urine pH of 6.5, and a positive urinary anion gap, establishing the diagnosis of distal renal tubular acidosis. Autoimmune tests revealed Sjögren syndrome as the underlying cause of the distal renal tubular acidosis. Full recovery followed potassium and alkali replacement. This dramatic presentation of Sjögren syndrome has not previously been reported in an elderly man. (J Clin Pathol 1999;52:157-158) Keywords: hypokalaemic paralysis; renal tubular acidosis; Sjögren syndrome Sjögren syndrome, an autoimmune exocrinopathy, can occur as an entity on its own (primary Sjögren syndrome) or in association with other autoimmune rheumatic disorders, most often rheumatoid arthritis, systemic lupus erythematosus, and scleroderma (secondary Sjö-gren syndrome). Overt or latent renal tubular acidosis, caused by autoimmune tubulointerstitial nephropathy, is a common extraglandular manifestation occurring in 33% of Sjögren syndrome patients. 1 Hypokalaemic paralysis is a well known, albeit rare, complication of severe distal renal tubular acidosis from any cause.2 3 Cases of Sjögren syndrome manifesting for the first time as hypokalaemic paralysis caused by severe distal renal tubular acidosis have been reported from various parts of the world. [4][5][6] As far as we are aware, this presentation of Sjögren syndrome has not been reported from the United Kingdom. We therefore report such a case to increase awareness of this dramatic presentation of the syndrome. Case reportA 73 year old white man presented with weakness of both legs of five days' duration. He had suVered previously from joint pains, but was usually mobile and self dependant. He gave a history of polyuria and polydipsia for the last four months. Five days before admission, he was unable to get up and felt worse in the morning, with improvement by the end of the day. On the day of admission, he was found lying on the floor by his sister.There was no history of dizziness, chest pain, cough, or dyspnoea. He smoked 10 cigarettes a day and drank 14 units of alcohol a week.On examination, he was conscious, oriented, and did not smell of alcohol, though he looked very frail and dehydrated. Recumbent blood pressure was 165/70 mm Hg, pulse rate was 98 beats/min and regular, jugular venous pressure was not raised, lung fields were clear, and heart sounds were normal. Abdominal examination showed no abnormality.On CNS examination, the cranial nerves were intact. There was proximal muscle weakness and wasting, more prominent in the legs than in the arms, with no evident muscle tenderness. Reflexes were normal apart from bilateral diminished ankle jerks. A straight leg raising test showed no abnormality, and there were no sensory deficits. An ECG showed sinus rhythm with obvious flattening of the T waves in all leads. A chest radiograph showe...

27-Year-Old Woman With Numbness and Weakness of the Extremities

“…There are numerous case reports of primary Sjögren syndrome presenting as hypokalemic paralysis, [7][8][9] showing that overt distal RTA can precede the development of sicca complex symptoms as it did in our patient. In a retrospective study of 10 women who presented with distal RTA and urolithiasis, 8 had positive anti-SS-A titers, 7 of whom developed Sjögren syndrome at an average of 15 years of follow-up.…”

Subacute cutaneous lupus erythematosus and life-threatening hypokalaemic tetraparesis: a rare complication

“…This case illustrates life‐threatening hypokalaemia caused by dRTA in a patient with SCLE. There are similar cases in the world literature, 2–4 some of which showed SLE/SS overlap. It has been suggested that the papulosquamous variant of SCLE, leucopenia and a high titre of autoantibodies are risk factors for the development of full‐blown SLE in those with SCLE, as illustrated by our patient.…”

Hypokalaemic paralysis precipitated by distal renal tubular acidosis secondary to Sjögren's syndrome

“…20 Histopathology from renal biopsy specimens from these patients show evidence of an interstitial lymphocytic infiltrate with plasma cells. Although previous authors have regarded this as contributory to the tubular dysfunction, 20,25 there is not universal agreement with regards to the significance attributed to this. 22 Hypergammaglobulinaemia is often present and has been suggested as a possible pathological means of precipitating distal tubular dysfunction.…”