title: Osteomalacia as a presenting manifestation of Sjögren's syndrome.
abstract: Osteomalacia is still common in Morocco, where the leading causes are nutritional deficiencies followed by intestinal diseases. Osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to a connective tissue disease such as Sjögren's syndrome. The case of a 40-year-old woman who presented with a five-year history of generalized bone pain, severe weight loss and a waddling gait is reported. She had low levels of serum phosphate (0.74 mmol/L), serum calcium (1.97 mmol/L), and urinary calcium (1.22 mmol/24 h). Serum alkaline phosphatase was 210 IU/L. Roentgenograms showed Looser's zones (right femoral neck, sixth and seventh right ribs). There was bilateral parotid gland enlargement, dryness of the mouth, nose and eyes, and bilateral punctate keratitis. A lip biopsy showed changes corresponding to stage II of the Chisholm and Mason classification. Tests for rheumatoid factor (latex and Waaler-Rose) and antinuclear factor were negative. The alkaline reserve was 18 mmol/L, serum potassium was 3.5 mmol/L, serum chloride was 112 mmol/L and urinary pH was 6.5. A renal biopsy showed tubulointerstitial lesions, lymphoplasmocytic infiltrates and interstitial sclerosis with patchy tubular atrophy. The patient was given bicarbonates, high-dose vitamin D followed by 1-alpha-hydroxycholecalciferol (0.3 microgram/d), and calcium (1 g/d). Follow-up was 42 months at the time of this writing. The role of tubular disorders in the genesis of osteomalacia is discussed, and the renal manifestations of Sjögren's syndrome are reviewed.
authors.affiliation: Department of Rheumatology B, El Ayachi Hospital, Rabat Teaching Hospital, Morocco.
authors.forename: N
authors.initials: N
authors.lastname: Hajjaj-Hassouni
authors.forename: N
authors.initials: N
authors.lastname: Guedira
authors.forename: N
authors.initials: N
authors.lastname: Lazrak
authors.forename: F
authors.initials: F
authors.lastname: Hassouni
authors.forename: A
authors.initials: A
authors.lastname: Filali
authors.forename: A
authors.initials: A
authors.lastname: Mansouri
authors.forename: L
authors.initials: L
authors.lastname: Balafrej
mesh_terms: D000141:Acidosis, Renal Tubular / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D010018:Osteomalacia / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism