Nephrotic syndrome presenting in a patient with primary Sjögren’s syndrome: questions

Abstract

Case summary A 17 year-old African American woman with a history of Sjogren’s syndrome presented with several days of periorbital and pedal edema. She also had fatigue, headache, abdominal pain, and diffuse arthralgias. She had no gross hematuria or oliguria. There were no recent sore throats or upper respiratory tract infections. She had no weight loss, night sweats, joint swelling, oral ulcerations, or rash. She had taken several doses of ibuprofen for arthralgias but was not taking any other medications. She was diagnosed with primary Sjogren’s syndrome at 11 years of age after presenting with bilateral parotitis. The diagnosis of Sjogren’s syndrome was based on the parotitis and a history of xerostomia and xerophthalmia. Schirmer’s test was abnormal (prolonged wetting time of filter paper placed in the lower eyelid), indicating abnormal tear production. Sialometry showed inadequate saliva production. Serologic markers at the time of diagnosis of Sjogren’s syndrome included normal serum C3 and C4, elevated antinuclear antibodies (ANA) of 1:2560, elevated anti-Ro/SSA antibody, negative anti-double-stranded DNA antibody, and elevated rheumatoid factor. She had a normal serum creatinine concentration of 0.7 mg/dl and a normal urinalysis with no blood or protein. She was treated with prednisone for 1 year and remained symptom free aside from intermittent mild xerostomia and xerophthalmia. Her father has systemic lupus erythematosus (SLE) and lupus nephritis.