Table 1.
Therapeutic Agent | Mechanism of Action | Clinical Studies in DAH |
---|---|---|
Glucocorticoids | Suppression of peripheral T helper responses Regulation of immunoglobulin production and B cell differentiation Suppression of proinflammatory mediators |
Barile et al (11) Kazzaz et al (1) Santos-Ocampo et al (30) Zamora et al (10) Andrade et al (31) |
Cyclophosphamide | Regulation of B cell activation, differentiation and suppression of B cell function Immunoregulatory effects on T cell subsets and activation markers (increased effector T cells and plasmacytoid dendritic cells) |
Kazzaz et al (1) Santos-Ocampo et al (30) Zamora et al (10) Badsha et al (32) Canas et al (33) Koh et al (34) |
Rituximab | Elimination of CD20+ B cells Modulation of costimulatory molecules that mediate B and T cell interactions |
Narshi et al (17) Tse et al (19) Machado et al (35) Aakjaer et al (36) |
Plasmapheresis | Direct removal of circulating immune complexes, antibodies and cytokines | Jones John et al (18) Claridge et al (37) |
Azathioprine and mycophenolate mofetil | Reduction of de novo purine synthesis results in decreased T and B cell proliferation | Rashidi et al (24) Andrade et al (31) Zamora et al (10) Santos-Ocampo et al (30) |
Immunoglobulin | Immunoregulatory effects including inhibition of B-cell proliferation, antibody production and growth factors and cytokines Anti-inflammatory effect through complement system inhibition and Fc receptor blockade on macrophages |
Andrade et al (31) Shen et al (38) Kwok et al (4) |
Mesenchymal stem cells | Immunomodulatory effects through inhibition of T, B, NK and dendritic cell proliferation and activation | Liang et al (27) Shi et al (39) |
Recombinant factor VIIa | Alveolar hemostasis through formation of FVIIa-tissue factor complex, which activates Factor X, ultimately leading to thrombin generation and clot formation | Heslet et al (13) Esper et al (40) |
Extracorporeal membrane oxygenation | Management of refractory hypoxemia through increased mixed venous oxygen content | Patel et al (14) |