An uncommon presentation of Sjögren's syndrome and brucellosis

• Published in: Transfusion and apheresis science Vol. 51; no. 1; pp. 77 - 80
• Main Authors: Celik, Gulperi, Ozturk, Ercument, Ipekci, Suleyman Hilmi, Yilmaz, Sema, Colkesen, Fatih, Baldane, Suleyman, Kebapcilar, Levent
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.08.2014
• Subjects: Acidosis, Renal Tubular - diagnosis; Acidosis, Renal Tubular - etiology; Adult; Brucellosis - complications; Brucellosis - diagnosis; Diabetes Insipidus, Nephrogenic - diagnosis; Diabetes Insipidus, Nephrogenic - etiology; Disseminated intravascular coagulation; Disseminated Intravascular Coagulation - diagnosis; Disseminated Intravascular Coagulation - etiology; Fanconi Syndrome - diagnosis; Fanconi Syndrome - etiology; Fanconi's syndrome; Female; Health technology assessment; Hematology, Oncology and Palliative Medicine; Humans; Hypokalemia; Hypokalemia - diagnosis; Hypokalemia - etiology; Nephrogenic diabetes insipidus; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis; Sjögren's syndrome; Hypokalemia; Disseminated intravascular coagulation; Sjögren's syndrome; Nephrogenic diabetes insipidus; Fanconi's syndrome
• ISSN: 1473-0502; 1878-1683
• DOI: 10.1016/j.transci.2014.03.011

Abstract We describe herein a case of hypokalemia due to proximal renal tubular acidosis (RTA) and Fanconi's syndrome (FS) and nephrogenic diabetes insipidus with DIC – a rare complication of Sjögren's syndrome (SS) and brucellosis. The interesting feature of this case was the presentation with severe hypokalemia, causing acute flaccid quadriparesis with cardiac arrest which is extremely rare. The patient was a 48-year-old woman who suffered cardiopulmonary arrest an hour after hospitalization. Analysis of a blood sample obtained before her cardiopulmonary arrest yielded surprising results: laboratory investigations showed profound hypokalemia (1.1 mEq/L) with renal K wasting, hyperchloremic metabolic acidosis with normal anion gap, hypophosphatemia with hypouricemia, glucosuria, and proteinuria. A diagnosis of RTA and FS were made. On the seventh day, she looked acutely ill, temperature 38.8 °C and pale, and her physical examination revealed purpuric skin lesions on both legs. The serum antibrucella titration agglutination test was found to be 1 of 160 positive with a nosocomial infection. The clinical and laboratory findings were consistent with disseminated intravascular coagulation (DIC). She was unable to concentrate her urine and so a diagnosis of nephrogenic diabetes insipidus (NDI) was reached. A thorough survey for the cause of FS, RTA and NDI revealed that she had xerophthalmia and xerostomia accompanied by high anti-Ro antibody, positive Schirmer test, confirming the diagnosis of SS.