She had regained 18 kg, and her albumin and chemistry panel had normalized

She had regained 18 kg, and her albumin and chemistry panel had normalized. pericardial effusions.1 PLGE is associated with multiple autoimmune conditions, most commonly systemic lupus erythematosus (SLE).2 PLGE has also been seen with Sj?gren’s syndrome, a systemic autoimmune disease characterized by chronic inflammation of the salivary and lacrimal glands.1-3 Sj?gren’s syndrome-associated PLGE is extremely rare: there are 10 reported cases in Asia, 9 of which were women. Case Report A 58-year-old white woman presented with a history of 43-kg unintentional weight loss over 5 years and 6 months of worsening crampy abdominal pain, diarrhea, nausea, vomiting, and electrolyte abnormalities. She noted non-bloody stools occurring 6-7 times per day with occasional incontinence. She had a history of Sj?gren’s syndrome diagnosed 5 years earlier by positive anti-Sj?gren’s-syndrome-related antigen A (anti-SSA), anti-Sj?gren’s-syndrome-related antigen B Centrinone-B (anti-SSB), and lip biopsy. Antibodies to double-stranded DNA (dsDNA) were negative. On presentation, she appeared cachectic, with exam notable for dry mucous membranes and diffuse mild abdominal tenderness without organomegaly. Laboratory results revealed hemoglobin (Hgb) 8.6 g/dL, bicarbonate 14 mmol/L, potassium 3.3 mmol/L, transferrin saturation 17%, prealbumin 19 mg/dL, erythrocyte sedimentation Centrinone-B rate 78 mm, C-reactive protein 0.5 mg/dL, and INR 1.0. Her renal function was otherwise normal. The total protein and albumin nadir during this hospitalization was 4.9 g/dL and 2.6 g/dL, respectively. Serum levels of fat-soluble vitamins were low except for vitamin E. Stool studies were negative for occult blood, em Clostridium difficile /em , and fecal fat; alpha-1 antitrypsin (A1AT) fecal clearance was 13 mL over 18 h (normal 13 mL/day). Normal labs included thyroid stimulating hormone, Hgb A1c, tissue transglutaminase IgA and IgG antibodies, quantitative immunoglobulins, C3, C4, and urine protein electrophoresis. She had a positive nuclear antibody (1:1280). Urine labs were consistent with a diagnosis of a distal renal tubular acidosis. CT enterography was significant for mild ascites and body wall edema without evidence of cirrhosis, bowel wall thickening, enhancing masses, or mesenteric lymphadenopathy. Echocardiogram was normal. Esophagoduodenography (EGD) with small bowel enteroscopy showed erythematous gastropathy, and Centrinone-B biopsies showed inactive chronic gastritis without em Helicobacter pylori /em . Random duodenal biopsies demonstrated architecturally normal small bowel mucosa with increased intraepithelial lymphocytes ( 30 lymphocytes/100 enterocytes; Figure 1). Colonoscopy with random biopsies was normal. Open in a separate window Figure 1 Duodenal biopsies demonstrating architecturally normal small bowel mucosa with increased intraepithelial lymphocytes ( 30 lymphocytes/100 enterocytes). The patient’s abdominal discomfort, nausea, renal tubular acidosis, and duodenal biopsies supported active Sj?gren’s syndrome with Myh11 systemic involvement. We proceeded with treatment of PLGE secondary to Sj?gren’s syndrome with immunosuppressive treatment of oral prednisone 60 mg daily and intravenous cyclophosphamide 800 mg monthly. Two months after discharge, she had resolution of diarrhea and abdominal pain. She had regained 18 kg, and her albumin and chemistry panel had normalized. Her prednisone was tapered to 10 mg daily and she continued to receive monthly cyclophosphamide. Discussion This is the first reported case of PLGE associated with Sj?gren’s syndrome outside of Asia in a white patient. The prevalence of this condition is unknown in U.S. patients with Sj?gren’s syndrome. PLGE was suspected in our patient after exclusion of other causes of low protein such as malnutrition, nephrotic syndrome, and liver dysfunction. The new diagnosis of a distal renal tubular acidosis supported active systemic Sj?gren’s syndrome and raised suspicion for PLGE as another extraglandular manifestation of her Sj?gren’s syndrome. Given active systemic Sj?gren’s syndrome, diagnosis of PLGE, presence of common gastrointestinal manifestations of Sj?gren’s syndrome, and exclusion of other causes of PLGE, she was diagnosed with PLGE secondary to Sj?gren’s syndrome.4,5 PLGE is usually diagnosed with nuclear radiology testing and/or a fecal clearance of A1AT 13 mL/day.6 No targeted systemic treatment is available for systemic Sj?gren’s syndrome, and there is.