Tocilizumab in the treatment of twelve cases with aa amyloidosis secondary to familial mediterranean fever

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Etanercept 1 endocrinologydiseasesdrugs
amyloidosis 37 endocrinologydiseases
cyclophosphamide 1 endocrinologydiseasesdrugs

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Select Drug Character Offset Drug Term Instance
Etanercept 10132 homozygote924F02034Anakinra0.40.36166.6225.896018470769.73.56961M694V homozygote1022F08120-0.390.5175.02133182780400.42893MEFV −/−1145FAS836Anakinra, Infliximab, Etanercept , Canakinumab0.80.9380.754.37061.7656804.41.65691M680Iheterozygote-M694Vheterozygote1221M0734Anakinra0.830.85143.6132.2211700167401.311.422329M694V/N
cyclophosphamide 10817 amyloidosis or associated diseases. Anakinra was given to five, canakinumab to three, infliximab to three, cyclophosphamide to two, etanercept to one, sulfasalazine to two and azathioprine to one patient(Table 3). The reasons
Select Disease Character Offset Disease Term Instance
amyloidosis 93 Title: Orphanet Journal of Rare DiseasesTocilizumab in the treatment of twelve cases with aa amyloidosis secondary to familial mediterranean feverSerdal UgurluAysa HaciogluYasaman AdibniaVedat HamuryudanHuri
amyloidosis 378 5/2017Publication date (collection): /2017AbstractBackgroundThere is no established treatment of AA amyloidosis , a long-term complication of various chronic inflammatory diseases associated with increased mortality,
amyloidosis 647 there are few reports pointing out that tocilizumab(TCZ), an anti IL-6 agent may be effective in AA amyloidosis resistant to conventional treatments. We report our data on the effect of TCZ in patients with FMF complicated
amyloidosis 778 conventional treatments. We report our data on the effect of TCZ in patients with FMF complicated with AA amyloidosis .MethodsFMF patients with histologically proven AA amyloidosis, treated with TCZ (8 mg/kg per month)
amyloidosis 840 patients with FMF complicated with AA amyloidosis.MethodsFMF patients with histologically proven AA amyloidosis , treated with TCZ (8 mg/kg per month) were followed monthly and the changes in creatinine, creatinine
amyloidosis 2529 recurrence of FMF attacks too. Further studies are warrented to test the efficacy and safety of TCZ in AA amyloidosis secondary to FMF as well as other inflammatory conditions.BackgroundFamilial Mediterranean Fever (FMF)
amyloidosis 2922 colchicine treatment prevents the recurrence of inflammatory attacks and also the development of AA amyloidosis , which is the most devastating complication of the disease related with increased mortality [[1]]. Development
amyloidosis 3051 most devastating complication of the disease related with increased mortality [[1]]. Development of AA amyloidosis in a compliant patient on regular prophylactic dose of colchicine is extremely rare. However poor compliance
amyloidosis 3295 intolerance due to side effects may render the patient from receiving the proper dose that will protect from amyloidosis [[2]].FMF is the most common cause of AA amyloidosis in Turkey with an overall frequency of 1-2/1000
amyloidosis 3348 receiving the proper dose that will protect from amyloidosis [[2]].FMF is the most common cause of AA amyloidosis in Turkey with an overall frequency of 1-2/1000 and amyloidosis is diagnosed in about one tenth of this
amyloidosis 3412 [[2]].FMF is the most common cause of AA amyloidosis in Turkey with an overall frequency of 1-2/1000 and amyloidosis is diagnosed in about one tenth of this population [[3], [4]].Although a number of agents have been
amyloidosis 3576 [[3], [4]].Although a number of agents have been considered, there is no established treatment of AA amyloidosis . IL-6 is one of the pro-inflammatory cytokines playing a critical role in the induction of SAA genes,
amyloidosis 3933 tocilizumab (TCZ), a humanized monoclonal anti IL-6 receptor antibody, was effective in the treatment of amyloidosis secondary to various rheumatic diseases. It binds to soluble and membrane-bound IL-6 receptors and down
amyloidosis 4227 [8]].Here we report our experience with TCZ in the treatment of 12 FMF patients complicated with AA amyloidosis .MethodsIn this case series, 14 patients recieved TCZ with the diagnosis of FMF related AA amyloidosis.
amyloidosis 4329 amyloidosis.MethodsIn this case series, 14 patients recieved TCZ with the diagnosis of FMF related AA amyloidosis . Only the results of 12 are given here because of suspect diagnosis of FMF in one, and the discontinuation
amyloidosis 4581 observed right after the first infusionin the other patient. All 12 patients with biopsy-proven FMF amyloidosis were regular attendees of the dedicated FMF clinic in Cerrahpasa Medical Faculty. They fullfilled the
amyloidosis 5218 to the modified New York criteria [[10]] and one of them also had Crohn’s disease.The diagnosis of amyloidosis was confirmed by detecting amyloid deposits in the tissues obtained either from rectum (two patients)
amyloidosis 5630 response during attack-free periods and deterioration of renal and/or gastrointestinal functions due to amyloidosis on maximum tolerated dose of colchicine. Patients with end stage renal disease (ESRD) on dialysis were
amyloidosis 7432 up No attacks1Unknown8110 Decrease number of attacks11 No response1 Proteinuria in patients with amyloidosis decreaseddecreasedDecreased in 79Side effectsNoneUnknownUnknownElevation of liver enzymes, mild thrombocytopeniaNot
amyloidosis 7964 characteristics and treatment details of 12 patients with the definite diagnosis of FMF and biopsy proven AA amyloidosis who received TCZ are given in Tables 2 and 3. The mean age of the patients was 35.2 ± 10.0, the
amyloidosis 8126 mean age of the patients was 35.2 ± 10.0, the mean duration of FMF was 15.0 ± 9.2 and of amyloidosis was 3.9 ± 4.8 years. The mean maximum dose of colchicine before TCZ therapy was 1.9 ± 0.4 mg/day.Table
amyloidosis 8482 mean ± SD34.2 ± 10.3Sex, M(%)/F (%)6 (%50.0)/6 (%50.0)Disease duration (years), mean ± SD6.43 ± 6.90Duration of amyloidosis (years), mean ± SD3.9 ± 4.8TCZ therapy duration (months), mean ± SD17.5 ± 14.7Number
amyloidosis 10710 (16.7%).Eight patients had received several DMARDs or other biological agents before TCZ either for amyloidosis or associated diseases. Anakinra was given to five, canakinumab to three, infliximab to three, cyclophosphamide
amyloidosis 17684 agents, eprodisate, anti IL-1 antagonists have been considered, there is no established treatment of AA amyloidosis by today [[11]–[14]]. Recently beneficial effect of TCZ, an anti IL-6 agent in the treatment of amyloidosis
amyloidosis 17794 amyloidosis by today [[11]–[14]]. Recently beneficial effect of TCZ, an anti IL-6 agent in the treatment of amyloidosis secondary to JIA has been reported [[5]]. This was followed by other cases of RA, Behcet’s Disease
amyloidosis 17935 reported [[5]]. This was followed by other cases of RA, Behcet’s Disease and FMF complicated with AA amyloidosis and a case series of 11 patients with FMF amyloidosis treated with TCZ [[8], [15]–[19]]. All reported
amyloidosis 17989 Behcet’s Disease and FMF complicated with AA amyloidosis and a case series of 11 patients with FMF amyloidosis treated with TCZ [[8], [15]–[19]]. All reported an overall improvement of renal function, decrease
amyloidosis 18387 response to various cytokines [[20]] and which is the precursor of AA fibrils leading to secondary amyloidosis [[21]]. Supression of SAA protein production by treatment of the underlying inflammatory disease resulted
amyloidosis 18820 amyloid A (SAA) in hepatocytes and its inhibition is postulated to be effective in the treatment of AA amyloidosis .SAA, ESR and CRP are good indicators of disease activity and response to treatment of the underlying
amyloidosis 18952 good indicators of disease activity and response to treatment of the underlying disease that cause amyloidosis [[26]]. It has been shown by Lachmann that elevated levels of SAA was related with an increased risk
amyloidosis 19068 [[26]]. It has been shown by Lachmann that elevated levels of SAA was related with an increased risk of amyloidosis [[27]]. However the data is scarce, a positive correlation between these markers has been reported [[28]].
amyloidosis 20044 clearance and proteinuria are good parameters used in the follow up of renal function in patients with amyloidosis . Overall the mean creatinine clearance remained stable while the mean 24-hour proteinuria and acute
amyloidosis 21782 previously.In a trial published in Germany, TCZ was given to five colchicine resistant FMF patients without amyloidosis ; three of them improved while one was stable and the other one had infusion reactions [[32]]. We observed
amyloidosis 22385 limitation of this study.ConclusionTCZ may be an alternative in the treatment of FMF patients with AA amyloidosis who are resistant/intolerant to colchicine. It is well tolerated and has an acceptable adverse effect
amyloidosis 22571 an acceptable adverse effect profile. TCZ was effective in controlling not only the signs related to amyloidosis but also the FMF attacks. Thus TCZ may be another treatment option besides anti-IL-1 approach even for
amyloidosis 22727 another treatment option besides anti-IL-1 approach even for colchicum resistant FMF patients without amyloidosis . One important point is that the patients should be followed closely for a rapid worsening of renal
amyloidosis 22996 interval of TCZ treatment. To conclude, TCZ seems to be an effective treatment option in patients with AA amyloidosis with few side effects

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