Multiple Limb Weakness May Be Sign of Sjögren’s, Case Report Suggests

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by Alice Melao |

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Severe muscle weakness in all four limbs may be a symptom of Sjögren’s syndrome, a case report contends.

Researchers hope their study will increase awareness about the multitude of ways Sjögren’s syndrome may be recognized, and subsequently improving the diagnosis.

The study, “Complete Hypokalemic Quadriparesis as a First Presentation of Sjögren Syndrome,” was published in the Canadian Journal of Kidney Health and Disease.

Sjögren’s syndrome is an autoimmune condition characterized by the infiltration of immune cells into secreting glands. As a result, patients usually have dry eyes and mouth. However, other organs may be affected by the disease.

Kidneys often are affected, with patients having increased inflammation, low potassium levels and, at times, failure to excrete acidic urine.

These problems may somtimes cause paralysis, arrhythmias, weakness in the cranial muscles, and respiratory arrest, all of which have been reported in Sjögren’s patients.

In this report, researchers share the case of a 73-year-old woman who came to the hospital with extreme muscle weakness in all four limbs.

Initial blood analysis suggested this was caused by critically low levels of potassium. But oral and intravenous potassium supplements failed to elevate potassium levels to normal range.

She was then referred to the University of Alberta Hospital, where a clinical history assessment revealed the woman had chronic pulmonary obstructive disease (COPD), and had been experiencing progressive muscle weakness and fatigue for the past two months. She also complained of worsening constipation and dry eyes for the past year.

A new physical examination revealed dry mucosa, which is a membrane that lines the mouth and nose cavities. At that time, the patient still had slightly weakened quadriceps muscles.

This led to a diagnosis of distal renal tubular acidosis (RTA), a medical condition in which the kidneys do not properly remove acids from the blood into the urine, causing acidosis.

Additional analysis to understand the causes of RTA revealed excess antinuclear antibodies (ANA), rheumatoid factor, and anti-Ro/SSA antibodies. Overall, the findings were consistent with a diagnosis of Sjögren’s syndrome.

The patient then was treated with supplements of sodium bicarbonate and amiloride, which reverted the acidic state and increased potassium levels to normal range.

RTA associated with Sjögren syndrome can be “managed by correcting the acidosis with alkali preparations such as sodium bicarbonate and potassium citrate,” the researchers wrote.

While the patient’s blood potassium levels remained stable for the following three weeks, a later biopsy of the kidneys revealed severe inflammation, tissue scarring, atrophy, and small vessel wall thickening.

“Our report emphasizes that although Sjögren syndrome is most often associated with chronic sicca [dryness] symptoms, it may present for the first time with extraglandular manifestations which may be life threatening,” the researchers concluded.