[The fracture risk assessment tool (FRAXTM), where are we in Tunisia?]. / Le score FRAX®, ou on est en Tunisie.

BACKGROUND: The fracture risk assessment tool (FRAXTM), published in February 2008, is developed based on the use of clinical risk factors with or without bone mineral density tests. AIM: To calculate the FRAX tool in a cohort of Tunisian patients in whom bone mineral density (BMD) was assessed by dual X ray absorptiometry (DXA); to correlate this score to osteoporotic fracture and to BMD assessment and to propose a threshold for therapeutic intervention. METHODS: In a cross sectional study of 582 patients older than 40 years, in whom a BMD measurement by DXA has been performed between January 2006 and December 2009, clinical risk factor for osteoporotic fracture and the occurrence of a prior fragility fracture were assessed. The French version of the FRAX tool was used. Threshold for pharmacological intervention was evaluated by ROC curve. RESULTS: Patients were aged 62.3 ± 10.4 years. They were female in 91.2% of cases. BMD measurement was under 2.5 standard deviation in 53.2%. Osteopenia was noted in 29.2% of cases and BMD was normal in 17.4 % of cases. Osteoporotic fractures were observed in 38.2% of cases. Major osteoporotic fractures (FOM) (hip, vertebra, radius occurred in 82% of cases. The FRAX® score calculated with T-score was 8.55 ± 8.54% for the FOM and 3.02 ± 6.37% for femoral neck (FN), while it was 7.81 ± 6.45% for the FOM and 2.58 ± 3.97% for the FN if calculated without T-score with a significant difference (p <10-3). For the patients having T-score under 2.5 SD, FRAX score was 11.39 ± 10.32% for the FOM and 4.74 ± 8.13% for the FN if calculated with T-score and it was 9.18 ± 6.95 % for the FOM and 3.19 ± 4.11 % if calculated without T-score. The score FRAX was correlated to BMD (r=0,53, p <10-3) and to fracture prevalence (p < 10-3). The threshold of therapeutic intervention was fixed to 30% for the FOM and 7% for the FN. CONCLUSION: Our study confirms the usefulness of the FRAX score in the prediction of fracture risk in Tunisian population. The determination of therapeutic threshold intervention requires other prospective and larger studies with medico-economic analyses.

[Safety of rheumatic disease drugs at childbearing age]. / Sécurité des médicaments à usage rhumatologique en âge de procréation.

The aim of this study is to determine the safety of commonly prescribed antirheumatic drugs at childbearing age, in pregnancy and lactation, through systematic literature review. Patients who take cytotoxic drugs should be informed of the risks of impared fertility. During pregnancy, non steroidal anti-inflammatory drugs (NSAIDs) can be safely administered until gestational week 32. Acetaminophen and low to moderate doses of corticosteroids are safe. Among, the disease-modifying agents, antimalarial agents, sulfasalazine, azathioprine and ciclosporin are compatible with pregnancy, and can be administered until birth. Paracetamol, prednison, antimalarial agents, sulfasalazine and most NSAIDs can safely be used by lactating mothers. To ensure a favourable outcome for both the mother and the child, the pregnancy should be planned, started during a period of disease stability, monitored closely and treated as needed.

[Adult-onset of Still's disease with atypical articular and skin manifestations]. / Atteintes articulaire et cutanée atypiques au cours d'une maladie de Still de l'adulte.

BACKGROUND: Adult-onset Still's disease (ASD) is an uncommon clinical entity. It is a diagnosis of exclusion, characterized by a clinical triad of intermittent fever spikes, evanescent rash, and either arthralgia or arthritis. Destructive arthritis more commonly affects the hips, wrists, tarsal joints and cervical spine. AIM: To report an unusual case of ASD with severe distal interphalangeal destructive arthritis and finger skin vesiculopustules. OBSERVATION: A 19 years old girl was followed for 2 year-history of ASD with polycyclic articular involvement. She noted, since 2 months, rapid appearance of painful tumefaction in the distal interphalangeal joints (DIP) with maculopustular eruption distributed exclusively on the hands, in front, only of DIP and few proximal interphalangeal joints (PIP). Further more, she complained of polyarticular active disease. Hands and wrists X-ray showed narrowed distal-interphalangeal joint space of only DIP joints. RMN imaging revealed in addition carpal, metacarpal and PIP articular inflammatory damage. The infectious investigation remained negative. A surgical skin and DIP biopsy specimens showed disseminated micro-abscesses with polynuclear leukocyte dermal infiltration. There was no signs of osteitis. Bacterial and fungal cultures from the pus failed to reveal any causative organisms. Skin lesions gradually disappeared in response to conventional ASD therapy after intensification. Hence, the diagnosis of distal destructive arthritis of ASD associated with atypical neutrophilic dermatosis (Sweet's syndrome) was made. CONCLUSION: ASD is rare, heterogeneous, with unpredictable evolution. The distal destructive arthritis represents a possible complication. The presence of pustules as atypical cutaneous features of Sweet's syndrome may be seen in severe forms of ASD and clinicians must be alert to the possibility of a misdiagnosis in these cases.

HLA-B27 and HLA-B51 determination in Tunisian healthy subjects and patients with suspected ankylosing spondylitis and Behçet's disease.

The aim of this retrospective study is to assess the frequency of HLA-B27 and HLA-B51 in healthy subjects from the center of Tunisia and to investigate their usefulness in the diagnosis of ankylosing spondylitis (AS) and Behçet's disease (BD), respectively. Microlymphocytotoxicity test was used to perform serologic HLA typing in a group of 124 healthy volunteers and a group of 365 patients suffering from clinical manifestations of AS and/or BD. HLA-B27 was found in 3.2% of healthy subjects and in 42.9% of patients with AS (P < 0.00006). HLA-B51 is, however, found in 16.1% of healthy subjects and in 30.0% of patients with BD (P > 0.05). Unlike HLA-B51, which seems to be as frequent in Tunisian patients with BD as in healthy subjects, HLA-B27 is more frequent in patients with AS than in controls. This highlights the usefulness of HLA-B27, rather than that of HLA-B51, in the diagnosis of the respective diseases.

Complete reversal of tumoral calcinosis after subtotal parathyroidectomy in a hemodialysis patient.

INTRODUCTION: Tumoral calcinosis is a rare complication of chronic hemodialysis whose mechanism is incompletely understood. The treatment is challenging and should target the main precipitating factors, most notably secondary hyperparathyroidism and calcium-phosphate (Ca x P) product elevation. CASE REPORT: In this 41-year-old patient, tumoral calcinosis developed in the right hip and subacromial bursa of the right shoulder after 3 years of chronic hemodialysis. Laboratory tests showed hyperparathyroidism with Ca x P elevation. Subtotal parathyroidectomy was performed. Eight months later, there was no evidence of tumoral calcinosis at either of the previously affected sites. CONCLUSION: The effectiveness of parathyroidectomy in our patient underlies the key role played by secondary hyperparathyroidism in the pathogenesis of tumoral calcinosis complicating hemodialysis.