iloprost and Antiphospholipid-Syndrome

More than a decade has gone by since the detailed clinical description of the Antiphospholipid (Hughes) Syndrome. Because of the wide spectrum of manifestations, virtually any physician may encounter patients with this potentially treatable condition. Because of limited controlled, prospective data, current therapy remains empirical and directed at coagulation mechanisms, immune mechanisms, or both. There is now good evidence that patients with antiphospholipid-associated thrombosis will be subject to recurrences and require prophylactic therapy. Although most authorities agree about the efficacy of warfarin alone or warfarin plus low-dose aspirin in preventing recurrences of venous and arterial thrombosis, there is still doubt regarding the intensity and duration of warfarin therapy. Steroids and immunosuppressive drugs have not provided long-term benefit. Controlled clinical trials of the treatment of pregnant women with antiphospholipid antibody demonstrated that prednisolone is ineffective, and possibly detrimental, in treatment of recurrent pregnancy loss and that heparin plus low-dose aspirin is beneficial.

Topics: Abortion, Habitual; Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Aspirin; Autoimmune Diseases; Contraindications; Drug Therapy, Combination; Female; Heparin; Humans; Iloprost; Immunoglobulins, Intravenous; Immunosuppressive Agents; Male; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Prospective Studies; Recurrence; Thrombophilia; Thrombosis; Warfarin

To determine the effects of short-term, maximum-tolerated-dose and long-term, optimum-dose iloprost treatment of severe pulmonary hypertension associated with systemic sclerosis (SSc) and the primary antiphospholipid syndrome (APS).. Three patients with SSc and 2 with APS who had failed to respond to oral vasodilator therapy for pulmonary hypertension were enrolled in a 32-week, open, prospective trial. Short-term infusion of maximum-tolerated doses and continuous infusion of optimum doses of iloprost were carried out following baseline cardiac catheterization. Catheterization was repeated at 2 and 32 weeks. All 5 patients completed the study and continued therapy for an average of 82 weeks (range 58-103).. Acute infusion of maximum tolerated doses significantly ameliorated the cardiac index (0.92 liters/minute/m2; P < 0.01), pulmonary artery O2 saturation (10.6%; P < 0.05), and pulmonary resistance (-6.7 units; P < 0.05). After 2 weeks of continuous infusion of optimum doses, there was improvement in pulmonary resistance (> or = 16%) and pulmonary artery O2 saturation (> 30%) in the 2 patients with primary APS. After 2 and 32 weeks, the 3 SSc patients showed variable hemodynamic responses. New York Heart Association functional class and exercise tolerance improved in all patients. There was 1 episode of bacteremia, and 1 patient died after 72 weeks of study.. Continuous iloprost infusion may improve exercise tolerance and quality of life in patients with severe pulmonary hypertension associated with SSc and primary APS.

Topics: Adolescent; Adult; Antiphospholipid Syndrome; Connective Tissue Diseases; Exercise Tolerance; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Iloprost; Infusions, Intravenous; Male; Middle Aged; Prospective Studies; Time Factors

Thrombotic antiphospholipid syndrome (t-PAPS) is characterized by arterial, venous, or microvascular occlusions, which are explained, in part, by the presence of antiphospholipid (aPL) antibodies. Although there is much evidence indicating that isolated aPL antibodies increase the activity of platelets obtained from healthy volunteers, platelet function in t-PAPS has not been as widely studied.. To evaluate platelet reactivity in t-PAPS patients.. Platelet aggregation, protein expression, and cyclic nucleotide levels were carried out in platelet rich plasma (PRP) or washed platelets (WPs) obtained from t-PAPS or healthy volunteers.. ADP-induced aggregation was significantly higher in PRP obtained from t-PAPS than obtained from the control. The protein expression of P2Y. The overexpression of P2Y

Topics: Adenosine Diphosphate; Antiphospholipid Syndrome; Blood Platelets; Cyclic AMP; Cyclic GMP; Humans; Iloprost; Nitroprusside; Platelet Aggregation; Platelet Aggregation Inhibitors; Signal Transduction

Topics: Adolescent; Age of Onset; Antiphospholipid Syndrome; Child; Female; Foot; Foot Ulcer; Humans; Iloprost; Ischemia; Raynaud Disease; Regional Blood Flow; Treatment Outcome; Vasoconstriction; Vasodilator Agents; Wound Healing

Topics: Acute Kidney Injury; Antiphospholipid Syndrome; Cardiomyopathies; Child; Hemolytic-Uremic Syndrome; Humans; Iloprost; Male; Peripheral Vascular Diseases; Peritoneal Dialysis, Continuous Ambulatory; Plasmapheresis; Skin

Undifferentiated connective tissue disease with secondary antiphospholipid syndrome was diagnosed in a 33 year old woman after recurrent arterial thromboses, two miscarriages and myocarditis. Despite effective immunosuppression and anticoagulation her initially mild precapillary pulmonary hypertension progressed in the absence of thromboembolic events, cardiopulmonary disease or other systemic disorders. With continuous i.v. iloprost in a dosage of up to 4.5 ng/kg/min for a total of 15 months, her 6 min walking-distance improved from 210 to 315 m, the pulmonary vascular resistance decreased from 1710 to 1111 dyn x s x cm-5 and the mean pulmonary arterial pressure decreased from 64 to 54 mmHg. This partial success of conservative treatment enabled a reassessment of the necessity for heart and lung transplantation.

Topics: Adult; Antiphospholipid Syndrome; Catheterization, Central Venous; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Iloprost; Infusions, Intravenous; Long-Term Care; Treatment Outcome

Topics: Adult; Antiphospholipid Syndrome; Female; Follow-Up Studies; Humans; Iloprost; Ischemia; Male; Middle Aged; Necrosis; Pain; Toes