Effectiveness, safety and drug survival of tumor necrosis factor-α inhibitors in the treatment of spondyloarthritis: A real-life study in Tunisia

Aim of the work: The aim of the present study was to evaluate effectiveness of anti-tumor necrosis factor-α (anti-TNFα) in the treatment of spondyloarthritis (SpA) and to assess their safety and drug survival. Patients and methods: Forty-two SpA patients (33 men, 9 women) were retrospectively studied. The disease was progressive in all patients. Response was assessed after 6 months using Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Functional Index (BASFI) scores and other clinical parameters. A major clinical response was defined as 50% improvement of the initial BASDAI. Patients were grouped into those with ankylosing spondylitis (AS) (24 patients) or psoriatic arthritis (PsA) and enteropathic arthritis (EA) (18 patients) and the response to anti-TNF was compared. Results: The mean age of the patients was 41.3 ± 9.7 years and disease duration 14.6 ± 8.2 years. After 6 months, 74% of patients were BASDAI 50 responders. The mean BASDAI and BASFI scores varied from 56 ± 20 and 61.8 ± 26 to 19 ± 19 and 24 ± 25 respectively (p < .001). The two SpA groups had the same effectiveness profile. The comparison between them showed a greater reduction of morning stiffness duration and erythrocyte sedimentation rate in patients with PsA or EA (p = .04). At least, one adverse event developed by 48% of patients and it was severe in 12%. Bronchopulmonary infections were the most frequent (8 patients). Drug survival rate was estimated at 86% after 1 year of treatment. Conclusion: Anti-TNFα therapy has a good response rate in SpA patients and an acceptable safety profile which explains the high drug survival rates. Keywords: Spondyloarthritis, Anti-TNF, Effectiveness, Safety, Drug surviva

A Rare Case of Hypophosphataemic Osteomalacia in von Recklinghausen Neurofibromatosis

Background: Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease, is a one of the more common hereditary autosomal disorders. However, osteomalacia in neurofibromatosis type 1 is very rare tumour-induced osteomalacia; fibroblast growth factor-23 is usually implicated.Patients and methods: We report the case of a patient with a history of von Recklinghausen neurofibromatosis who presented with hypophosphataemic osteomalacia.Results: The patient was treated with high-dose calcitriol and oral phosphate with clinical improvement. Conclusion: Even though it is a rare entity, we must consider the diagnosis of hypophosphataemic osteomalacia in patients with neurofibromatosis in order to deliver appropriate treatment

Synovitis with pitting edema as the presenting manifestation of antisynthetase syndrome

International audienc

Osteogenesis imperfecta and Ledderhose disease: Is there a link? A report of two Tunisian siblings

Introduction: Osteogenesis imperfecta (OI) or “brittle bone disease” is a rare genetic disorder tissue due to an abnormal production of type I collagen. It can cause hearing loss, dentinogenesis imperfecta, heart failure, spinal cord problems and permanent deformities. Ledderhose’s disease or Plantar fibromatosis (PF) is also a rare condition that may be caused by an abnormal proliferation of collagen tissue. It is a benign fibroblastic proliferative disorder in which fibrous nodules may develop in the plantar aponeurosis, more specifically on the medial plantar side of the foot arch and on the forefoot region. Rare cases of association of polyfibromatosis with keloids or arthritis have been reported. Case report: We report two Tunisian brothers aged 17 and 14 years old, from a first-degree consanguineous marriage and with medical history of multiple bone fractures. The elder brother has medical history of juvenile arthritis treated with methotrexate and etanercept. Clinical examination of both patients showed blue sclera, bone deformities with humeral and femoral curvature and unequal leg length. They were diagnosed with OI and received bisphosphonate to prevent further fractures. One year later, they developed lumps under the sole skin of their feet. They were diagnosed with PF and received conservative treatment including non-steroidal anti-inflammatory drugs, physiotherapy and orthotic support. Conclusion: To the best of our knowledge, the association of PF with OI has never been reported. Both diseases suggest an association with abnormalities involving collagen. A question remains currently with no answer: is it a fortuitous association