@article {3577, title = {Serum amyloid protein A concentration in cryopyrin-associated periodic syndromes patients treated with interleukin-1 beta antagonist.}, journal = {Clin Exp Rheumatol}, volume = {32}, year = {2014}, month = {2014 Jul-Aug}, pages = {S63-6}, abstract = {

OBJECTIVES: Cryopyrin-associated periodic syndromes (CAPS) are a group of chronic, relapsing autoinflammatory disorders which may be complicated by systemic AA amyloidosis. The aim of our study was to evaluate serum amyloid protein A (SAA) level in CAPS patients treated with Interleukin-1beta (IL-1β) antagonist and to correlate its level with treatment response.

METHODS: All patients of CAPS Italian Register treated with IL-1β inhibitor were enrolled. SAA levels before starting therapy, and at last visit were evaluated. Patients were then divided in complete responders and partial responders.

RESULTS: Twenty-five patients were enrolled. SAA level before starting therapy was increased (median 118.5 mg/L, IQR 96.4-252.8; normal value <6.4 mg/L), while at last visit SAA was significantly reduced (median 4.3 mg/L, IQR 2.3-12.7) (p<0.001). However 12 patients still presented SAA levels beyond normal range, 10/25 patients (40\%) showed a complete response to treatment. Conversely, 15 patients presented only a partial response, of which 12 for increased SAA value and 3 for increased CRP value. Patients with partial response had SAA values significantly higher than patients with complete response (median 12.6 mg/L; IQR 8.3-20.0 vs. 2.7 mg/L; IQR 1.6-4.1, p<0.001).

CONCLUSIONS: Our results confirm the long term efficacy of anti IL-1β treatment in CAPS and the decrease of SAA levels; however 48\% of patients still presented SAA elevation despite treatment. The real risk of these patients in developing amyloidosis is not clear but the persistent increase of SAA needs a close follow-up.

}, keywords = {Adolescent, Adult, Amyloidosis, Child, Child, Preschool, Cryopyrin-Associated Periodic Syndromes, Drug Monitoring, Female, Humans, Immunosuppressive Agents, Interleukin-1beta, Male, Middle Aged, Serum Amyloid A Protein, Treatment Outcome, Young Adult}, issn = {0392-856X}, author = {Pastore, Serena and Paloni, Giulia and Caorsi, Roberta and Ronfani, Luca and Taddio, Andrea and Lepore, Loredana} }