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44. Kongress der Deutschen Gesellschaft für Rheumatologie, 30. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie, 26. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie

31.08. - 03.09.2016, Frankfurt am Main

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Anti-Il 1 Therapy in patients with FMF-related amyloidosis living in Germany

Meeting Abstract

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  • Birgit Maria Buhl - Uniklinikum Heidelberg, Rheumatologie, Heidelberg
  • Norbert Blank - Universitätsklinikum Heidelberg, Medizinische Klinik V, Sektion Rheumatologie, Heidelberg
  • Hanns-Martin Lorenz - Universitätsklinikum Heidelberg, Medizinische Klinik V, Sektion Rheumatologie, Heidelberg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. 44. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh); 30. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh); 26. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Frankfurt am Main, 31.08.-03.09.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. DocVS.10

doi: 10.3205/16dgrh243, urn:nbn:de:0183-16dgrh2435

Veröffentlicht: 29. August 2016

© 2016 Buhl et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

Gliederung

Text

Background: Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease characterized by recurrent, self-limiting attacks of fever, and serositis. Renal amyloidosis is a serious complication of FMF that determines the prognosis. Patients with AA-Amyloidosis can be treated with Interleukin-1 (IL-1)-inhibiting drugs.We report our single center experience in adult patients with colchicine-resistant FMF-related amyloidosis who were treated with colchicine and anakinra.

Methods: Demographic data, clinical and laboratory parameters, MEFV mutations, patient reported outcomes and physician global health were extracted from our GARROD (German Anti-IL1beta medication RegistRy in Orphan Diseases) registry.

Results: Within our cohort of 205 adult patients with FMF, we identified 36 patients (17.6%) with FMF-related amyloidosis. All patients received colchicine treatment. A subgroup of these patients continued to have colchicine-resistant FMF attacks and elevated acute phase reactants in the serum. Nine patients (2 female and 7 male) were treated with a combination of colchicine (1.5 +/- 0.65 mg per day) and Anakinra. All patients were of turkish-armenian ancestry. Homozygosity for high penetrance mutation was present in 7 patients, heterozygosity in 2 patients. Three patients (33%) presented with nephrotic syndrome, 3 patients (33%) with chronic kidney disease (CKD), one with end-stage renal disease (ESRD), and 2 patients were 5 and 7 years after renal transplantation.

Before initiation of anakinra the 24h urine protein excretion (mean +/-SD) was 5.0g +/-3.5g /24h. The proteinuria decreased with anakinra to 0.4g +/-1g /24h in the last visit after 24 +/- 18 months. The creatinine serum levels of all patients with CKD decreased or remained stable.

The patient reported health (VAS 70 +/- 21mm) and the physician reported global health (VAS 80 +/- 16mm) both improved significantly (VAS 10 +/- 20mm, VAS 7.5 +/- 20mm) (p 0.001, p 0.0009) after 6 months of treatment.

Conclusion: Anakinra is well tolerated and effective in patients with FMF-related amyloidosis.