Session Information
Session Type: ACR Concurrent Abstract Session
Session Time: 2:30PM-4:00PM
Background/Purpose: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by self-limiting febrile attacks associated with serosal or synovial inflammation as well as increased risk for AA-amyloidosis. There are limited data about kidney transplantation (KT) in FMF patients with amyloidosis regarding the post-transplant course of FMF and amyloidosis. This study aims to investigate the efficacy, safety and long term outcome of IL-1 receptor antagonist (IL-1RA, anakinra) treatment in FMF patients with amyloidosis who underwent KT. Methods: We screened our database for FMF patients diagnosed with amyloidosis between 1978-2016, and reviewed charts of those who underwent kidney transplantation (KT). Seventeen FMF patients with KT and receiving anakinra constituted the study group. All patients were also receiving colchicine treatment. All patients were on maintenance immunosuppressive therapy. Partial response was defined as ≥50% decrease in proteinuria whereas complete response was defined as <0.3 gr/d proteinuria.
Results: Demographic features of the patients are shown in Table. The percentage of irregular/inadequate colchicine use in patients who developed amyloidosis was 41%. Mean age of transplant recipients at the time of transplantation was 32±7 (22-51). The reasons to start anakinra treatment in the post-transplant FMF group included inadequate response to colchicine treatment (n=13), persistently elevated CRP (n=8), and persistence of proteinuria (n=3). Recurrence of renal amyloidosis in transplant kidney was noted in four patients. Mean GFR at the last follow-up was 62±30(15-142) mL/min. Attack frequency was significantly decreased in patients after anakinra treatment Table2. After initiation of anakinra treatment, CRP decreased to normal values in 50% of the patients, and partial and complete response were achieved in only two patients with proteinuria. The proteinuria levels remained stable in three patients under colchicine and anakinra treatment. Serious infection required hospitalization was observed in two patients (pneumonia and pyelonephritis) treated with anakinra on top of other immunosuppressives, and the patient with pneumonia died due to septic shock. Severe neutropenia (<500/mm3) developed in one patient after three years of anakinra therapy without serious complications. Table-1. Demographic and Clinical Parameters of FMF Patients Who Underwent KT.
| Male/Female ratio (n) |
12/5 |
| Age (mean±SD, range) |
39±10 (24-59) |
| Age of onset (mean±SD, range) |
10±7 (2-26) |
| Age of diagnosis (mean±SD, range) |
21±11 (8-48) |
| Family history of FMF, n (%) |
10 (58%) |
| Family history of amyloidosis, n (%) |
1 (6%) |
| Disease follow-up duration (mo) |
32±50 (3-216) |
| Time to diagnosis (mo) |
140±137 (6-504) |
| Arthritis at presentation n(%) |
9 (53%) |
| MEFV mutation, n (%) |
M694V, 11 (100%)
|
| Duration of IL-1RA (mo) |
21±16 (4-51) |
| High CRP rate during attack free periods n (%) |
9 (53%) |
| Mean colchicine doses after KT (mg) |
1.1±0.4 (0.5-2) |
Abbreviations: FMF, Familial Mediterranean Fever; mo, months; CRP, C-reactive protein; KT, kidney transplantation Table-2. Attack frequency under anakinra treatment
| Patients | Anakinra Duration (months) | Before anakinra attack frequency /month | After anakinra attack frequency / /month |
|
1 |
3 |
1 |
0 |
|
2 |
3 |
2 |
0 |
|
3 |
4 |
0 |
0 |
|
4 |
4 |
0,5 |
0 |
|
5 |
4 |
0 |
0 |
|
6 |
7 |
1 |
0 |
|
7 |
11 |
0 |
0 |
|
8 |
14 |
1 |
0 |
|
9 |
15 |
1 |
0 |
|
10 |
24 |
1 |
0 |
|
11 |
27 |
0 |
0 |
|
12 |
31 |
3 |
1 |
|
13 |
36 |
2 |
0 |
|
14 |
36 |
1 |
0 |
|
15 |
37 |
0,7 |
0 |
|
16 |
48 |
1 |
0 |
|
17 |
51 |
1,3 |
0 |
Conclusion: Anakinra treatment seems to be effective and safe in refractory FMF patients with amyloidosis who underwent KT
To cite this abstract in AMA style:
Toz B, Çalışkan YK, Erer B, Ocal L, Gul A. Efficacy and Safety of IL-1 Inhibitors in Amyloidosis Associated with Familial Mediterranean Fever Who Underwent Kidney Transplantation [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/efficacy-and-safety-of-il-1-inhibitors-in-amyloidosis-associated-with-familial-mediterranean-fever-who-underwent-kidney-transplantation/. Accessed .« Back to 2016 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-and-safety-of-il-1-inhibitors-in-amyloidosis-associated-with-familial-mediterranean-fever-who-underwent-kidney-transplantation/