J Rheum Dis.  2012 Aug;19(4):220-224. 10.4078/jrd.2012.19.4.220.

A Case of Type 1 Renal Tubular Acidosis and Osteomalacia in a Patient with Sjogren's Syndrome

• Lee HJ
• Lee SJ
• Park SC
• Park DJ
• Kim TJ
• Lee SS
• Park YW

• KMID: 2223075
• DOI: http://doi.org/10.4078/jrd.2012.19.4.220

Abstract

Sjogren'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 Sjogren's syndrome. Overt or latent renal tubular acidosis is an uncommon extraglandular manifestation in Sjogren'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 Sjogren'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 Sjogren's syndrome complicated by osteomalacia and renal tubular acidosis

Keyword

Sjogren's syndrome; Renal tubular acidosis; Osteomalacia

MeSH Terms

Acidosis, Renal Tubular
Adult
Autoimmune Diseases
Female
Humans
Lower Extremity
Muscle Weakness
Osteomalacia
Salivary Glands
Sjogren's Syndrome

Figure

• 

  Figure 1 Kidney-ureter-bladder (KUB) radiograph demonstrating multiple calculi in both kidney.

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  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.

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  Figure 3 Salivary gland scan shows decreased radioisotope uptakes in both parotid and submandibular glands.

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  Figure 4 Salivary gland biopsy shows focal lymphocytic infiltration in minor salivary glands of lower lip (H & E stain, ×100).

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