What therapies have been used successfully for the non thrombotic manifestations of APS?
• Immune-mediated thrombocytopenia (<20,000/mm 3 ): glucocorticoids with or without IVIG. Second-line therapy: traditional immunosuppressives, rituximab. Try to avoid splenectomy due to operative clot risk.
• Immune-mediated hemolytic anemia: same as thrombocytopenia.
• Acute renal failure due to thrombotic microangiopathy: plasma exchange.
• Chronic ischemic nephropathy due to vaso-occlusive lesions: sirolimus especially post renal transplant.
• Heart valve lesions: nothing effective. Anticoagulation to prevent emboli.
• Neurologic manifestations: seizures (antiepileptic agents); chorea (antidopaminergic medications, glucocorticoids, immunosuppressives); longitudinal transverse myelitis (R/O anti-neuromyelitis optica disease, treat with anticoagulation/glucocorticoids/immunosuppressives).
• Migraine headaches: 2-week trial of LMWH. If effective, continue anticoagulation.
• Livedoid vasculopathy: usually not responsive to glucocorticoids. Antiplatelet agents, pentoxifylline, sildenafil, IVIG, hyperbaric oxygen, and anticoagulation often times in combination.