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2017 ; 12
(1
): 105
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Tocilizumab in the treatment of twelve cases with aa amyloidosis secondary to
familial mediterranean fever
#MMPMID28558744
Ugurlu S
; Hacioglu A
; Adibnia Y
; Hamuryudan V
; Ozdogan H
Orphanet J Rare Dis
2017[May]; 12
(1
): 105
PMID28558744
show ga
BACKGROUND: There is no established treatment of AA amyloidosis, a long-term
complication of various chronic inflammatory diseases associated with increased
mortality, such as familial Mediterranian fever (FMF). Recently 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 with AA amyloidosis. METHODS: FMF
patients with histologically proven AA amyloidosis, treated with TCZ (8 mg/kg per
month) were followed monthly and the changes in creatinine, creatinine clearance,
the amount of 24-hour urinary protein, erythrocyte sedimentation rate (ESR) and
C-reactive protein (CRP) were noted throughout the treatment period. Adverse
effects of the treatment were closely monitored. RESULTS: TCZ was given to 12
patients (6 F, 6 M) who also continued to receive colchicine (1.9?±?0.4 mg/day).
Coexisting diseases were ankylosing spondylitis(4) and Crohn's disease(1). The
mean age was 35.2?±?10.0 years and the mean follow-up on TCZ was
17.5?±?14.7 months. The renal functions remained stable (mean creatinine from
1.1?±?0.9 mg/dl to 1.0?±?0.6 mg/dl), while a significant decrease in acute phase
response (the mean CRP from 18.1?±?19.5 mg/L to 5.8?±?7.1 mg/L and ESR from
48.7?±?31.0 mm/h to 28.7?±?28.3 mm/h) was observed and the mean 24-hour urinary
protein excretion reduced from 6537.6?±?6526.0 mg/dl to 4745.5?±?5462.7 mg/dl.
Two patients whose renal functions were impaired prior to TCZ therapy improved
significantly on this regimen. No infusion reaction was observed. None of the
patients experienced any FMF attack under TCZ treatment with the exception of 2,
one of whom had less frequent attacks while the other had episodes of
erysipelas-like erythema. CONCLUS?ON: Tocilizumab improved the acute phase
response and the renal function in this group of patients and was generally well
tolerated. Besides improving the renal function TCZ seemed to control the
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.