Rheumatology International

, Volume 27, Issue 6, pp 593–597 | Cite as

Acquired hypophosphatemia osteomalacia associated with Fanconi’s syndrome in Sjögren’s syndrome

  • Yi-Sun Yang
  • Chiung-Huei Peng
  • Sung-Kien Sia
  • Chien-Ning Huang
Case Report


Sjögren’s syndrome is an autoimmune disorder involving exocrine glands that occurs alone or in association with various autoimmune and connective tissue diseases. The severity of Sjögren’s syndrome ranges from isolated sicca syndrome to severe complications such as vasculitis, lung and renal involvement. Overt or latent renal tubular acidosis caused by autoimmune tubulointerstitial nephritis, is a common extraglandular manifestation in Sjögren’s syndrome. Osteomalacia is a rare complication of renal tubular acidosis, and it was reported to be associated with distal renal tubular acidosis in Sjögren’s syndrome. We report a 60-year-old woman who presented with multiple bone deformity and general muscle weakness. Osteomalacia was secondary to Fanconi’s syndrome, and the Fanconi’s syndrome was a result of renal involvement in Sjögren’s syndrome. Fanconi’s syndrome is a rare kidney manifestation in Sjögren’s syndrome. It may be latent and may precede the subjective sicca symptoms. These findings suggest that evidence for Sjögren’s syndrome should be sought in adult patients with unexplained osteomalacia and renal tubular acidosis, even in the absence of subjective sicca syndrome. Conversely, in patients with Sjögren’s syndrome, early investigation and treatment of renal tubular dysfunction may prevent future complications, such as osteomalacia.


Osteomalacia Sjögren’s syndrome Fanconi’s syndrome 


  1. 1.
    Bloch KH, Buchanan WW, Wohl MJ, Bunim JJ (1965) Sjögren’s syndrome. A clinical, pathological and serological study of sixty-two cases. Medicine 44:187–231PubMedGoogle Scholar
  2. 2.
    Manthorpe R, Asmussen K, Oxholm P (1997) Primary Sjögren’s syndrome: diagnostic criteria, clinical features and disease activity. J Rheumatol 24(Suppl 50):8–11Google Scholar
  3. 3.
    Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, Cavazzana I, Viola BF, Valzorio B, Mazzucchelli C, Cattaneo R, Scolari F, Maiorca R (2001) Clinical and morphological features of kidney involvement in primary Sjögren’s syndrome. Nephol Dial Transplant 16:2328–2336CrossRefGoogle Scholar
  4. 4.
    Goules A, Masouridi S, Tzioufas AG, Ioannidis JPA, Skopoudi FN, Moutsopoulos HM (2000) Clinically significant and biopsy-documented renal involvement in primary Sjögren’s syndrome. Medicine (Baltimore) 79:241–249CrossRefGoogle Scholar
  5. 5.
    Shiozawa S, Shiozawa K, Shimizu S, Nakada M, Isobe T, Fujita T (1987) Clinical studies of renal disease in Sjögren’ss syndrome. Ann Rheum Dis 46:768–772PubMedCrossRefGoogle Scholar
  6. 6.
    Bridoux F, Kyndt X, Abou-Ayache R, Mougenot B, Baillet S, Bauwens M, Lemaitre V, Goujon JM, Touchard G, Vanhille P (2004) Proximal tubular dysfunction in primary Sjögren’s syndrome: a clinicopathological study of 2 cases. Clin Nephrol 61:434–439PubMedGoogle Scholar
  7. 7.
    Eriksson P, Denneberg T, Enestrom S, Johanson B, Lindstrom F, Skogh T (1996) Urolithiasis and distal tubular acidosis preceding primary Sjögren’s syndrome: a retrospective study 5–53 years after the presentation of urolithiasis. J Intern Med 239:483–488PubMedCrossRefGoogle Scholar
  8. 8.
    Moutsopoulos HM, Cledes J, Skopoulli FN, Elisaf M, Youinou P (1991) Nephrocalcinosis in Sjögren’s syndrome: a late sequela of renal tubular acidosis. J Intern Med 230:187–191PubMedCrossRefGoogle Scholar
  9. 9.
    Shearn MA, Tu WH (1965) Nephrogenic diabetes insipidus and other defect of renal tubular function in Sjögren’s syndrome. Am J Med 39:312–318PubMedCrossRefGoogle Scholar
  10. 10.
    Dowd JE, Lipsky PE (1993) Sjögren’s syndrome presenting as hypokalemic periodic paralysis. Arthritis Rheum 36:1735–1738PubMedCrossRefGoogle Scholar
  11. 11.
    Zimhony O, Sthoeger Z, Ben David D, Bar Khayim Y, Geltner D (1995) Sjögren’s syndrome presenting as hypokalemic paralysis due to distal tubular acidosis. J Rheumatol 22:2366–2368PubMedGoogle Scholar
  12. 12.
    Monte Neto JT, Sesso R, Kirsztajn GM, Da Silva LC, De Carvalho AB, Pereira AB (1991) Osteomalacia secondary to renal tubular acidosis in a patient with primary Sjögren’s syndrome. Clin Exp Rheumatol 9:625–627PubMedGoogle Scholar
  13. 13.
    Clarke BL, Wynne AG, Wilson DM, Fitzpatrick LA (1995) Osteomalacia associated with adult Fanconi’s syndrome: clinical and diagnostic features. Clin Endocrinol (Oxf) 43:479–490Google Scholar
  14. 14.
    Narvaez J, Domingo-Domenech E, Narvaez JA, Nolla JM, Valverde J (2004) Acquired hypophosphatemic osteomalacia associated with multiple myeloma. Joint Bone Spine 72:424–426CrossRefGoogle Scholar
  15. 15.
    Pal B, Griffiths ID (1998) Primary Sjögren’s syndrome presenting as osteomalacia secondary to renal tubular acidosis. Br J Clin Pract 42:436–438Google Scholar
  16. 16.
    Hajjaj-Hassouni N, Guedira N, Lazrak N, Hassouni F, Filali A, Mansouri A, Balafrej L (1995) Osteomalacia as a presenting manifestation of Sjögren’s syndrome. Rev Rhum Engl Ed 62:529–532PubMedGoogle Scholar
  17. 17.
    Okazaki H, Muto S, Kanai N, Shimizu H, Masuyama J, Minato N, Sumiya M, Asano Y, Kano S (1991) A case of primary Sjögren’s syndrome presenting as osteomalacia secondary to renal tubular acidosis. Ryumachi 31:45–53PubMedGoogle Scholar
  18. 18.
    Jovelic A, Stefanovic D (2005) Distal renal tubular acidosis as a cause of osteomalacia in a patient with primary Sjögren’s syndrome [abstract]. Vojnosanit Pregl 62:769–773PubMedGoogle Scholar
  19. 19.
    Fulop M, Mackay M (2004) Renal tubular acidosis, Sjögren’s syndrome, and bone disease. Arch Intern Med 164:905–909PubMedCrossRefGoogle Scholar
  20. 20.
    Morris RC, Sebastian A, Morris E, Ueki I (1968) Hypergammaglobulinemic renal tubular acidosis: a spectrum of physiological disturbances. J Clin Invest 47:70Google Scholar
  21. 21.
    Kamn DE, Fischer MS (1972) Proximal renal tubular acidosis and the Fanconi’s syndrome in a patient with hypergammaglobulinemia. Nephron 9:208–219CrossRefGoogle Scholar
  22. 22.
    Walker BR, Alexander F, Tannenbaum PJ (1971) Fanconi’s syndrome with renal tubular acidosis and light chain proteinuria. Nephron 8:103–107PubMedCrossRefGoogle Scholar
  23. 23.
    Delplace M (1983) Les manifestation renales du syndrome de Sjögren. Revue de la literature a partir d’un cas [abstract]. Sem Hosp 59:1693–1698Google Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Yi-Sun Yang
    • 1
  • Chiung-Huei Peng
    • 2
  • Sung-Kien Sia
    • 1
  • Chien-Ning Huang
    • 1
  1. 1.Division of Endocrinology and Metabolism, Department of Internal Medicine, Chung-Shan Medical University HospitalChung Shan Medical UniversityTaichung Taiwan
  2. 2.Department of NursingHungkuang UniversityTaichungTaiwan

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