fibrinopeptide-a and Scleroderma--Systemic

In seven patients with systemic sclerosis (SS), we evaluated the clinical effectiveness of oral administration of saprogrelate hydrochloride (SH: 300 mg/day) for 2 months on Raynaud's phenomenon (RP) and respiratory failure estimated by Hugh-Jones classification.. We evaluated laboratory data (arterial blood gas (pH, PaO2 and PaCO2), pulmonary function tests (%VC, FEV1/FVC and %DL(CO)), mean pulmonary arterial pressure (mPAP), white blood cell count, C-reactive protein and the plasma levels of fibrinopeptide A (FPA), beta-thrombogloblin (beta-TG), platelet factor 4 (PF4) and thrombomodulin (TM)) before and after SH administration.. The frequency and duration of RP, as well as the coldness, numbness and pain of RP were significantly decreased after SH administration (P < 0.05, P < 0.01 and P < 0.001). Respiratory failure estimated by Hugh-Jones classification was also significantly decreased after SH administration (P < 0.05), and the %DL(CO) was significantly increased (P < 0.01). The mPAP decreased significantly after SH administration (P < 0.05). Plasma FPA, beta-TG and PF4 significantly decreased after administration (P < 0.05 and P < 0.01).. SH therapy could prevent RP and respiratory failure in patients with SS.

Topics: Adult; Aged; beta-Thromboglobulin; Blood Pressure; C-Reactive Protein; Female; Fibrinopeptide A; Humans; Leukocyte Count; Male; Middle Aged; Platelet Factor 4; Pulmonary Artery; Pulmonary Fibrosis; Pulmonary Ventilation; Raynaud Disease; Respiratory Insufficiency; Scleroderma, Systemic; Serotonin Antagonists; Thrombomodulin

We measured plasma levels of the blood coagulation/fibrinolysis molecular markers, thrombin-antithrombin III complex (TAT), fibrinopeptide A (FPA), alpha 2-plasmin inhibitor plasmin complex (PIC), beta-thromboglobulin (beta-TG), platelet factor 4 (PF4), at 6:00, 12:00, 18:00 and 24:00 in 10 female patients with progressive systemic sclerosis (PSS) (severe and mild sclerosis groups, each n = 5), 3 cases of dermatomyositis (DM) (M:F = 2:1) and 5 female healthy controls (HC). Corticosteroid (predonisolon; 20-25 mg/day) was administered orally in six patients with PSS and dermatomyositis longer than one month. Plasma levels of TAT increased more than 3 ng/ml in 8 out of 10 cases (80%) of PSS, while the levels increased in only 2 of 8 cases (25%) of the non-PSS groups (DM and HC). The severe sclerosis group of PSS showed a peak at 6:00 in the circadian variations of plasma levels of TAT and FPA, while the mild sclerosis group of PSS showed a peak at 12:00 or 24:00, and both DM and HC at 24:00. However, there was no significant peak in circadian variations of the plasma levels of PIC in the severe sclerosis group of PSS, although there was a peak at 24:00 in other diseases. The synchronized peaks of TAT and PIC were seen in 4 of 8 cases (50%) of the non-PSS group. On the other hand, this synchronization was only detected in 1 of 10 cases (10%) of PSS. The plasma levels of beta-TG and PF4 increased in 8 of 10 cases (80%) of PSS, but these levels did not increase in 8 non-PSS cases. Circadian variation of plasma levels of beta-TG showed a peak at 6:00 in the severe sclerosis group of PSS, while the mild sclerosis group of PSS, DM and HC revealed peaks at different times of 18:00, 24:00 and 12:00, respectively. Additionally, the plasma levels of beta-TG increased more than those of PF4 in the treated group with corticosteroid, although both beta-TG and PF4 revealed a statistically significant correlation in the non-treated group. These results may suggest abnormalities of not only platelet activity, but also of blood coagulation/fibrinolysis system in both severe and mild sclerosis groups of PSS.

Topics: Adult; Aged; alpha-2-Antiplasmin; Antifibrinolytic Agents; Antithrombin III; beta-Thromboglobulin; Biomarkers; Blood Coagulation Factors; Case-Control Studies; Circadian Rhythm; Female; Fibrinolysin; Fibrinolysis; Fibrinopeptide A; Glucocorticoids; Humans; Male; Middle Aged; Peptide Hydrolases; Platelet Factor 4; Prednisolone; Scleroderma, Localized; Scleroderma, Systemic