Soad Haj-Yahia1, Ilan Ben-Zvi2, Merav Lidar3, Avi Livneh4. 1. Clinical Immunology, Angioedema and Allergy Unit, The Zabludowicz Center for Autoimmune Diseases, The Chaim Sheba Medical Center, Tel Hashomer, Israel. 2. Heller Institute of Medical Research, The Chaim Sheba Medical Center, Tel Hashomer, Israel; Rheumatology Unit, The Chaim Sheba Medical Center, Tel Hashomer, Israel; Department of Medicine F, The Chaim Sheba Medical Center, Tel Hashomer, Israel; The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; The Talpiot Medical Leadership Program, The Chaim Sheba Medical Center, Tel Hashomer, Israel. 3. Rheumatology Unit, The Chaim Sheba Medical Center, Tel Hashomer, Israel; The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 4. Heller Institute of Medical Research, The Chaim Sheba Medical Center, Tel Hashomer, Israel; Rheumatology Unit, The Chaim Sheba Medical Center, Tel Hashomer, Israel; Department of Medicine F, The Chaim Sheba Medical Center, Tel Hashomer, Israel; The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: alivneh@tauex.tau.ac.il.
Abstract
OBJECTIVE: Familial Mediterranean fever (FMF) is the most common interleukin 1 (IL-1)-driven monogenic autoinflammatory disease. Yet published data also suggest that tumor necrosis factor (TNF) may have a role in the pathogenesis of FMF and may serve as a target for treatment. In the present study we evaluate this hypothesis. METHODS: To this goal, we studied the incidental effect on FMF of TNF-directed treatment, administered to colchicine-refractory FMF patients for the management of a concurrent inflammatory disease. The rates of FMF patients and of treatments with complete or nearly complete FMF response were determined, based on the number of FMF attacks during TNF-blocker exposures. The possible effect of various FMF and non-FMF features on the outcome was determined using comparative analysis. Patients were identified and data were retrieved using electronic files from the FMF clinic. RESULTS: Twenty-six patients were identified, each receiving ≥1 of four TNF-blockers for a mean duration of 27.6±16.4months. The TNF-blockers were found to induce complete or nearly complete FMF response in 10 (38.5%) of the patients, and in 13 of 50 (26%) exposures. No clinical, genetic, demographic, or therapeutic feature could predict which FMF patient would respond favorably to TNF-blocker therapy. CONCLUSION: This study suggests that TNF-blockers may be beneficial for a small proportion of colchicine-resistant FMF patients.
OBJECTIVE:Familial Mediterranean fever (FMF) is the most common interleukin 1 (IL-1)-driven monogenic autoinflammatory disease. Yet published data also suggest that tumor necrosis factor (TNF) may have a role in the pathogenesis of FMF and may serve as a target for treatment. In the present study we evaluate this hypothesis. METHODS: To this goal, we studied the incidental effect on FMF of TNF-directed treatment, administered to colchicine-refractory FMFpatients for the management of a concurrent inflammatory disease. The rates of FMFpatients and of treatments with complete or nearly complete FMF response were determined, based on the number of FMF attacks during TNF-blocker exposures. The possible effect of various FMF and non-FMF features on the outcome was determined using comparative analysis. Patients were identified and data were retrieved using electronic files from the FMF clinic. RESULTS: Twenty-six patients were identified, each receiving ≥1 of four TNF-blockers for a mean duration of 27.6±16.4months. The TNF-blockers were found to induce complete or nearly complete FMF response in 10 (38.5%) of the patients, and in 13 of 50 (26%) exposures. No clinical, genetic, demographic, or therapeutic feature could predict which FMFpatient would respond favorably to TNF-blocker therapy. CONCLUSION: This study suggests that TNF-blockers may be beneficial for a small proportion of colchicine-resistant FMFpatients.