Journal List > J Rheum Dis > v.19(4) > 1063978

Lee, Lee, Park, Park, Kim, Lee, and Park: A Case of Type 1 Renal Tubular Acidosis and Osteomalacia in a Patient with Sjögren's Syndrome


Sjögren's syndrome is a chronic autoimmune disease characterized by decreased function of the lacrimal and salivary glands. In addition, many other organs can be involved in patients with Sjögren's syndrome. Overt or latent renal tubular acidosis is an uncommon extraglandular manifestation in Sjögren's syndrome, and osteomalacia is a rare complication of renal tubular acidosis. It has been rarely reported that osteomalacia is associated with distal renal tubular acidosis in patients with Sjögren's syndrome. We report a case of a 34-year-old female patient who was initially presented with muscle weakness in both lower extremities. The patient was then diagnosed with Sjögren's syndrome complicated by osteomalacia and renal tubular acidosis

Figures and Tables

Figure 1
Kidney-ureter-bladder (KUB) radiograph demonstrating multiple calculi in both kidney.
Figure 2
Pseudofracture and decreased bone mineral density in a patient with Sjögren's syndrome and type 1 renal tubular acidosis. (A) Hip X-ray shows insufficiency fracture or pseudofracture of the right femoral neck (black arrows). (B) Bone mineral density examination shows markedly decreased T-score and Z-score in both femoral bones.
Figure 3
Salivary gland scan shows decreased radioisotope uptakes in both parotid and submandibular glands.
Figure 4
Salivary gland biopsy shows focal lymphocytic infiltration in minor salivary glands of lower lip (H & E stain, ×100).


1. Asmussen K, Andersen V, Bendixen G, Schiødt M, Oxholm P. A new model for classification of disease manifestations in primary Sjögren's syndrome: evaluation in a retrospective long-term study. J Intern Med. 1996. 239:475–482.
2. Aasarød K, Haga HJ, Berg KJ, Hammerstrøm J, Jørstad S. Renal involvement in primary Sjögren's syndrome. QJM. 2000. 93:297–304.
3. Poux JM, Peyronnet P, Le Meur Y, Favereau JP, Charmes JP, Leroux-Robert C. Hypokalemic quadriplegia and respiratory arrest revealing primary Sjögren's syndrome. Clin Nephrol. 1992. 37:189–191.
4. Fulop M, Mackay M. Renal tubular acidosis, Sjögren syndrome, and bone disease. Arch Intern Med. 2004. 164:905–909.
5. Yamada A. Tubulointerstitial nephropathy secondary to collagen-vascular diseases. Nihon Rinsho. 1995. 53:1969–1973.
6. Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, Cavazzana I, et al. Clinical and morphological features of kidney involvement in primary Sjögren's syndrome. Nephrol Dial Transplant. 2001. 16:2328–2336.
7. Dafnis E, Spohn M, Lonis B, Kurtzman NA, Sabatini S. Vanadate causes hypokalemic distal renal tubular acidosis. Am J Physiol. 1992. 262:F449–F453.
8. Buckalew VM Jr. Nephrolithiasis in renal tubular acidosis. J Urol. 1989. 141:731–737.
9. Rodríguez Soriano J. Renal tubular acidosis: the clinical entity. J Am Soc Nephrol. 2002. 13:2160–2170.
10. Eriksson P, Denneberg T, Larsson L, Lindström F. Biochemical markers of renal disease in primary Sjögren's syndrome. Scand J Urol Nephrol. 1995. 29:383–392.
11. Wrong OM, Feest TG, MacIver AG. Immune-related potassium-losing interstitial nephritis: a comparison with distal renal tubular acidosis. Q J Med. 1993. 86:513–534.
12. Soy M, Pamuk ON, Gerenli M, Celik Y. A primary Sjögren's syndrome patient with distal renal tubular acidosis, who presented with symptoms of hypokalemic periodic paralysis: Report of a case study and review of the literature. Rheumatol Int. 2005. 26:86–89.
13. Caciotti A, Morrone A, Domenici R, Donati MA, Zammarchi E. Severe prognosis in a large family with hypokalemic periodic paralysis. Muscle Nerve. 2003. 27:165–169.
14. Bingham CT, Fitzpatrick LA. Noninvasive testing in the diagnosis of osteomalacia. Am J Med. 1993. 95:519–523.
15. Sauveur B, Garabedian M, Fellot C, Mongin P, Balsan S. The effect of induced metabolic acidosis on vitamin D3 metabolism in rachitic chicks. Calcif Tissue Res. 1977. 23:121–124.
Similar articles