TY - JOUR
T1 - Serum amyloid protein A concentration in cryopyrin-associated periodic syndrome patients treated with interleukin-1 beta antagonist
AU - Pastore, Serena
AU - Paloni, Giulia
AU - Caorsi, Roberta
AU - Ronfani, Luca
AU - Taddio, Andrea
AU - Lepore, L.
AU - Alessio, Maria
AU - Cantarini, Luca
AU - Cattalini, Marco
AU - Consolini, Rita
AU - Gattorno, Marco
AU - Gerloni, Valeria
AU - Insalaco, Antonella
AU - Martini, Giorgia
AU - Martino, Silvana
AU - Obici, Laura
AU - Rigante, Donato
PY - 2014
Y1 - 2014
N2 - Objective: 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-1 beta (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). Conclusion: 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.
AB - Objective: 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-1 beta (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). Conclusion: 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.
KW - Amyloidosis
KW - Cryopyrin-associated periodic syndromes
KW - IL-1β inhibitor
KW - Inflammation
KW - Serum amyloid protein A
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M3 - Article
C2 - 25069027
AN - SCOPUS:84907509450
VL - 32
SP - S63-S66
JO - Clinical and Experimental Rheumatology
JF - Clinical and Experimental Rheumatology
SN - 0392-856X
ER -