PlasmapheresisRx

Indications: Plasmapheresis can be effective in a number of rheumatologic and immunologic disorders, including Goodpasture syndrome, myasthenia gravis, Guillain-Barré syndrome, hyperviscosity syndrome (e.g.,with Waldenström macroglobulinemia), cryoglobulinemia (particularly with renal involvement), thrombotic thrombocytopenic purpura, catastrophic antiphospholipid antibody syndrome (CAPS), idiopathic demyelinating polyneuropathy, rapidly progressive glomerulonephritis, and refractory autoimmune hemolytic anemia. In addition to thrombotic thrombocytopenic purpura, plasmapheresis may be of benefit in other disorders associated with microangiopathic hemolytic anemia, including hemolytic-uremic syndrome, SLE presenting with thrombotic thrombocytopenic purpura–like features, disseminated intravascular coagulation, and CAPS. Plasmapheresis may be of benefit in other conditions, e.g., SLE with transverse myelitis, alveolar hemorrhage or catastrophic conditions. However, the evidence is anecdotal at best. For SLE nephritis, several studies showed that in unselected groups of patients, plasmapheresis is of no benefit. Plasmapheresis is not indicated in RA or dermatomyositis.

Mechanisms of Action: Plasmapheresis nonspecifically removes plasma constituents, including immunoglobulins and other plasma proteins. Removal of particular antibodies or immune complexes presumably underlies its effect in most diseases. In thrombotic thrombocytopenic purpura, and perhaps other conditions, some of the benefit perhaps also derives from infusion of normal plasma.

Comments: There are few adequate controlled trials in rheumatic diseases. Plasmapheresis should usually be used in conjunction with immunosuppressive therapy to prevent rebound increase in antibody production.

Cost: $$$$$. An important consideration in the use of plasmapheresis is its cost, which runs upward of several thousand dollars for a treatment. Although generally well tolerated, it may also be associated with transient hypocalcemia and bleeding diathesis.

BIBLIOGRAPHY

Nguyen TC, Kiss JE, Goldman JR, Carcillo JA. The role of plasmapheresis in critical illness. Crit Care Clin 2012;28:453-68. PMID: 22713617.

Schwartz J, Winters JL, Padmanabhan A, et al. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: the sixth special issue. J Clin Apher 2013;28:145-284. PMID:23868759.