title: Fanconi's syndrome and distal (type 1) renal tubular acidosis in a patient with primary Sjögren's syndrome with monoclonal gammopathy of undetermined significance.
abstract: Tubulointerstitial nephritis is a well-recognized complication in primary Sjögrens syndrome. Fanconi's syndrome is a far less frequent complication compared with distal tubular dysfunction. We here describe a 49-year-old woman with primary Sjögren's syndrome. In 1997, she was diagnosed with primary Sjögren's syndrome with tubulointerstitial nephritis, and was then treated with oral prednisolone for the tubulointerstitial nephritis. In 2002, she was referred to our hospital because of progressive fatigue. At that time, biclonal spike on serum protein (IgG-kappa and IgA-kappa) and Bence-Jones protein in urine were found. Bone marrow aspiration showed 1.0% plasma cell infiltration. Thus, a diagnosis of monoclonal gammopathy of undetermined significance (MGUS) was made. In 2004, she was again admitted to our hospital because of mild renal dysfunction and hypokalemia. Laboratory evaluation showed inappropriate, alkaline urine in hyperchloremic metabolic acidosis and a positive urine anion gap, indicating the presence of distal (Type 1) renal tubular acidosis (RTA). The urine concentration defect was also found. Further studies revealed proximal tubular dysfunction, including renal glycosuria, generalized aminoaciduria, phosphaturia, uricosuria and proximal RTA. The kidney biopsy represented diffuse and severe tubulointerstitial nephritis with dense infiltrates of lymphocytes and IgA and K light chain-positive plasma cells. No findings of multiple myeloma or malignant lymphoma were observed. In conclusion, our patient had Sjögren's syndrome with MGUS and exhibited dysfunction of both proximal tubule (Fanconi's syndrome) and distal tubule, which may be attributed to diffuse tubulointerstitial nephritis.
authors.affiliation: Department of Nephrology, Jichi Medical School, Shimotsuke, Tochigi, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kobayashi
authors.forename: S
authors.initials: S
authors.lastname: Muto
authors.forename: J
authors.initials: J
authors.lastname: Nemoto
authors.forename: Y
authors.initials: Y
authors.lastname: Miyata
authors.forename: S
authors.initials: S
authors.lastname: Ishiharajima
authors.forename: M
authors.initials: M
authors.lastname: Hironaka
authors.forename: Y
authors.initials: Y
authors.lastname: Asano
authors.forename: E
authors.initials: E
authors.lastname: Kusano
mesh_terms: D000141:Acidosis, Renal Tubular / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D005198:Fanconi Syndrome / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000648:ultrastructure; D008875:Middle Aged; D010265:Paraproteinemias / Q000175:diagnosis*; D012859:Sjogren's Syndrome / Q000097:blood* / Q000150:complications*