Dermatologic Manifestations of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) Medication

Updated: Dec 15, 2017
  • Author: Claudia Hernandez, MD, FAAD; Chief Editor: William D James, MD  more...
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Medication

Medication Summary

Corticosteroids are the mainstay of treatment in eosinophilic granulomatosis with polyangiitis (EGPA). The addition of other medications may be necessary in cases of life- or organ-threatening vasculitis.

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Corticosteroids

Class Summary

These drugs have anti-inflammatory and immunosuppressive properties.

Methylprednisolone (Medrol, Solu-Medrol)

Methylprednisolone is used to treat inflammatory and immune reactions. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Prednisolone (Econopred, Articulose-50, Delta-Cortef)

Prednisolone decreases autoimmune reactions, possibly by suppressing key components of the immune system.

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Immunomodulators

Class Summary

These agents modulate immune responses to various stimuli.

Methotrexate (Folex PFS, Rheumatrex)

Methotrexate has an unknown mechanism of action in the treatment of inflammatory reactions; it may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).

Azathioprine (Imuran)

Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, lowering autoimmune activity.

Cyclosporine (Sandimmune, Neoral)

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft vs host disease) in many organs. Base dosing on ideal body weight.

Interferon alfa 2b (Interferon alfa-2b, Intron A)

Interferon alfa 2a and 2b are recombinant DNA products. Their mechanism of antitumor activity is not clearly understood; they have direct antiproliferative effects against malignant cells and modulation of host immune response may be important.

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Alkylating agents

Class Summary

These agents are recommended as initial therapy of severe, life-threatening Churg-Strauss syndrome (allergic granulomatosis) and for patients who are not responsive to corticosteroids alone.

Cyclophosphamide (Cytoxan, Neosar)

Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with the growth of normal and neoplastic cells.

Chlorambucil (Leukeran)

Chlorambucil alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

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Anti-TNF agents

Class Summary

These agents are recommended in severe cases when corticosteroids and cyclophosphamide may be insufficient to induce remission.

Infliximab (Remicade)

Infliximab is a monoclonal antibody with human constant and murine variable regions; it neutralizes the biologic activity of TNF-alpha with high binding affinity to the soluble transmembrane forms of TNF-alpha and it inhibits binding of TNF with receptors.

Etanercept (Enbrel)

Etanercept is a fusion protein of the TNF receptor and Fc portion of human IgG-1; it binds TNF and blocks the interaction of TNF-alpha and TNF-beta with cell-surface receptors, rendering TNF biologically inactive.

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