SummaryOsteonecrosis of the jaw (ONJ) has been recently described after intravenous administration of amino-bisphosphonates and -less frequently -in association with the use of oral bisphosphonates. Bisphosphonate-Related Osteonecrosis of the Jaw (BRONJ) may affect mandible bone (65%), maxilla bone (26%) and rarely (9%) both sites simultaneously. Although causality may never be proven, emerging experimental data have established a strong association between monthly intravenous bisphosphonate administration and ONJ. Current level of evidence does not fully support a cause and effect relationship between the use of oral BPs and ONJ. In this paper, we report a clinical case of BRONJ in a 73 years old woman affected by rheumatoid arthritis (RA) and periodontitis, after three years of treatment with alendronate 70 mg one a week, plus daily calcium and vitamin D. The patient developed a tooth abscess at the lower jaw, accompanied by increased inflammatory markers, that never returned to normal range despite antibiotic therapy, inducing deterioration of joint synovium. The worsening of joint status after the onset of ONJ was reflected by the progressive increase in the number of swollen (SJ) and tender (TJ) joints, by the deterioration of the score DAS 28 (which passed from 5.46 to 7.07), pain (with VAS increasing from 60 to 90), and by a progressively impaired quality of life, as reported using the HAQ score (from 1,25 to 2,5). The patient was switched to antifracture therapy with strontium ranelate and the osteonecrosis was successfully treated with antibiotics, surgical curettage and local ultrasounds.

SummaryThe Ambulatory of Biella is the only service of Rheumatology of the Biella area. The Paget's disease is the 0.5% and appears to be representative of the territory of Biella. We evaluated the pagetics locations, disease activity and quality of life, the presence of gene mutation and frequency of comorbidities and therapeutic efficacy. Patients with active disease were treated with intravenous bisphosphonate therapy and investigated in relation to the side effects: flu-like syndrome and therapeutic efficacy by ALP and scintigraphy. 15 out of 20 patients analyzed resulted in the active disease with pain in the upper-middle pagetic location. Treatment with bisphosphonates has led to remission of disease: normalization of ALP values in 90%, documented by the scintigraphy of control. In the 86% of cases it was not necessary to make a second infusion, even 3 years after the first administration. In only two cases was carried out another infusion for the persistence of pain, despite levels of ALP prove the rule, in such cases, the scintigraphy can showed uptake of the sites involved. Bone scintigraphy is more sensitive and specific of ALP in determining disease activity and should be used in cases of doubt of retreatment. 53% of cases showed as side effect a flu-like syndrome; 6 months after infusion secondary hyperparathyroidism in 46% of cases. SQSTM1 mutation was positive in two patients originating from Veneto and Sardinia, but in no patients in Biella. The presence of two aggregations family suggesting other mutations in the Biella population.