The objective of our study was to investigate the safety and efficacy of IL-1-blocking therapy in colchicine-resistant FMF and in patients with FMF and AA amyloidosis
Lachmann, "Therapeutic blockade of interleukin-6 by tocilizumab in the management of AA amyloidosis
and chronic inflammatory disorders: a case series and review of the literature," Clinical and Experimental Rheumatology, vol.
Systemic AA amyloidosis
(formerly secondary amyloidosis) is due to tissue deposition of serum amyloid A, which is an acute phase reactant produced by the liver.
Cadinanos, "Systemic AA amyloidosis
: epidemiology, diagnosis, and management," Journal of Clinical Epidemiology, vol.
Solomon, "AA amyloidosis
associated with a mutated serum amyloid A4 protein," Amyloid, vol.
Merlini, "Susceptibility to AA amyloidosis
in rheumatic diseases: a critical overview," Arthritis Care and Research, vol.
can complicate a number of chronic inflammatory conditions, including rheumatoid arthritis (RA), juvenile RA, and ankylosing spondylitis1,2.
Ehresmann, "Infliximab treatment of familial Mediterranean fever and its effect on secondary AA amyloidosis
," Journal of Clinical Rheumatology, vol.
(34.) Livneh A, Zemer D, Langevitz P, Laor A, Sohar E, Pras Colchicine treatment of AA amyloidosis
of familial Mediterranean fever.
KIACTA(TM) is being developed for the treatment of AA amyloidosis
, a life-threatening orphan disease that occurs in patients with long-lasting inflammatory conditions, most commonly due to rheumatoid arthritis.
As the definition comprises the term secondary, there must be certain engendering factors involved, and the leading causes of AA amyloidosis
are chronic inflammatory diseases such as rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) that account for almost half of the patients (2).
In conclusion, this case illustrates a phenotypic shift in a renal transplant patient with unrecognized FMF and secondary AA amyloidosis
possibly due to the newly established equilibrium in various cytokines with ongoing immunosuppression.
(3) The authors did not describe clearly whether their classification is suitable for this particular form of renal AA amyloidosis
. As the hilar pattern of glomerular involvement is closely associated with the vascular form, (3) the latter should thus be considered as a minimal amyloid deposition (class I), in spite of the sometimes massive vascular amyloid deposits.
A severe autosomal-dominant periodic inflammatory disorder with renal AA amyloidosis
and colchicine resistance associated to the MEFV H478Y variant in a Spanish kindred: an unusual familial Mediterranean fever phenotype or another MEFV-associated periodic inflammatory disorder?
Turkey, in common with other countries flanking the Mediterranean basin, is an endemic area of familial Mediterranean fever associated with AA amyloidosis
. This scoring system, adapted from the 2004 ISN/RPS systemic lupus erythematosus classification, and first presented at the World Congress of Nephrology held in Singapore, is updated in this issue.