Respiratory arrest following severe muscle weakness as initial presentation of Sjögren’s syndrome: diagnostic and therapeutic strategies: a case report

Abstract

Background: SjÖgren’s syndrome refers to an autoimmune disorder defined by chronic inflammation as a consequence of the exocrine gland’s lymphocytic infiltration. Patients may present with heterogeneous clinical symptoms involving impairment in the lungs, kidneys, and nervous system. The most common renal involvement is tubulointerstitial nephritis (TIN) responsible for 20% of renal tubular acidosis. We report a case of respiratory failure resulting from severe hypokalemia in a patient with SjÖgren syndrome. Case presentation: A 46-year-old female who experienced progressive quadriplegia leading to respiratory distress caused by severe hypokalemia. Laboratory findings showed: sodium 128 mmol/L, potassium 1.1 mmol/L, Cloride 93 mmol/L, Calcium 7.3 mg/dL (corrected 7.5 mg/dL), albumin 3.3 g/dL, Magnesium 1.8 mg/dL with amylase 244 U/L, lipase 4168 U/L, The ANA test increased slightly and Ro-52, PM-Scl100 showed strong positive results, finally the patient was diagnosed with SjÖgren's syndrome with renal tubular acidosis (RTA). She was later diagnosed with renal tubular acidosis secondary to SjÖgren’s syndrome and treated using methylprednisolone, potassium, and alkali administration. Later, her potassium levels reached normal, muscle weakness improved, and she was extubated. Conclusion: This case emphasizes the concept of an uncommon form of SjÖgren's syndrome in the form of severe refractory hypokalemia due to renal tubular acidosis accompanied by acute pancreatitis disguised in patients without SICCA complaints. Renal tubular acidosis is a rare case, but SjÖgren's syndrome is a cause that must be considered in all patients who present with paralysis due to refractory hypokalemia so that there is no misinterpretation and management.