Terrier B, Fakhouri F, Berezne A, Bouldouyre M-A, Guilpain P, Sogni P, Terris B, Noël L-H, Guillevin L, Mouthon L
Department of Internal Medicine and French Reference Center for necrotizing vasculitides and systemic sclerosis, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), France.
Clin Exp Rheumatol. 2008 May-Jun;26(3):467-70.
Systemic sclerosis (SSc) may affect the gastrointestinal tract and cause very rarely malabsorption syndrome related to bacterial overgrowth. Malabsorption syndrome may be responsible for weight loss, diarrhea, osteomalacia, and iron and vitamins deficiency. We report the case of a SSc patient who developed osteomalacia caused by the combination of two exceptional conditions in the setting of SSc: celiac disease (CD) and primary biliary cirrhosis (PBC)-related Fanconi syndrome. Oral prednisone with angiotensin-converting enzyme inhibitors, was initiated because of active lesions of tubulitis, and led to the complete regression of bone pains, and by the improvement of renal function and regression of the features of proximal tubulopathy. Thus, in the presence of vitamin deficiencies in a patient with SSc, together with a search for malabsorption syndrome secondary to bacterial overgrowth, CD and/or PBC-associated Fanconi syndrome should be investigated.
