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03 April 2018 : Case report  Ecuador

Sjögren’s Syndrome Associated with Fanconi’s Syndrome and Osteomalacia

Rare coexistence of disease or pathology

Jorge O. Gutierrez1EF*, Maria F. Zurita1F, Luis A. Zurita2ADEF

DOI: 10.12659/AJCR.907503

Am J Case Rep 2018; 19:392-396

Abstract

BACKGROUND: Sjögren’s syndrome is a chronic inflammatory autoimmune disease, which is also known as sicca syndrome, due to the symptoms of dry eyes and dry mouth, and is associated with other connective tissue diseases and autoimmune diseases. Sjögren’s syndrome can also be associated with renal involvement. Fanconi’s syndrome is associated with impaired reabsorption in the proximal renal tubule associated with tubulointerstitial nephritis and is associated with renal tubular acidosis and hypophosphatemia. Osteomalacia is a rare association with Sjögren’s syndrome, which may result from renal disease.

CASE REPORT: We report the case of a 34-year-old woman who presented with xerostomia, xerophthalmia, bone fractures, and osteomuscular pain. A Schirmer test showed reduced tear production, and a biopsy of a minor salivary gland of the lip, with high titers of antinuclear antibodies (ANA), and positive anti-SSA/Ro and anti-SSB/La antibodies confirmed the diagnosis of Sjögren’s syndrome. Serum and urinary laboratories tests and clinical manifestations confirmed Fanconi’s syndrome associated with osteomalacia. The patient was treated with potassium supplements, 25-hydroxyvitamin D (25(OH)D), hydroxychloroquine, mycophenolate mofetil, and prednisone, with a favorable response.

CONCLUSIONS: This case is of a rare association between Sjögren’s syndrome, Fanconi’s syndrome, and osteomalacia. Even though these are rare clinical associations, early detection can improve the quality of life and prevent further complications.

Keywords: Fanconi Syndrome, Osteomalacia, Sjogren's Syndrome

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923