Weber-Christian Disease Medication

Updated: Oct 07, 2019
  • Author: Donald A Person, MD, FAAP, FACR; Chief Editor: Lawrence K Jung, MD  more...
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Medication

Medication Summary

No specific uniformly effective therapy for Weber-Christian disease is recognized. Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, mycophenolate, and clofazimine. Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations. [10, 20, 21, 22, 23]  Nonsteroidal anti-inflammatory agents (eg, indomethacin) may reduce fever, arthralgias, and other signs of malaise. Involvement of specific organs may require specific supportive drugs.

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Corticosteroids

Class Summary

These agents are used for suppression of acute inflammatory exacerbations. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Sterapred)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

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Immunomodulators

Class Summary

These agents inhibit key factors that mediate immune reactions, which, in turn, decrease inflammatory responses. They may have potential long-term therapeutic response.

Azathioprine (Imuran)

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Cyclosporine (Neoral, Sandimmune)

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, graft versus host disease) for a variety of organs. For children and adults, base dosing on ideal body weight. Demonstrated to be helpful in variety of skin disorders.

Cyclophosphamide (Neosar, Cytoxan)

Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Demonstrated to be helpful in various skin disorders.

Mycophenolate (CellCept)

Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.

Hydroxychloroquine (Plaquenil)

Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.

Thalidomide (Thalomid)

Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.

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Antibiotics

Class Summary

Several antibiotics (eg, tetracycline, clofazimine) are used for their anti-inflammatory activity.

Tetracycline (Sumycin)

Treats gram-positive and gram-negative organisms, as well as mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).

Clofazimine (Lamprene)

Clofazimine is a lipophilic rhimophenazine dye that inhibits template function of DNA by binding to it. It is weakly bactericidal and has anti-inflammatory effects. Although the mechanism of action is unclear, it seems to exert its main effect on neutrophils and monocytes in various ways (eg, stimulating phagocytosis and release of lysosomal enzymes). Clofazimine is indicated for panniculitis of mycobacterial infections. It has been used in other inflammatory dermatoses but with unknown efficacy.

Use of clofazimine for the treatment of any disease other than leprosy is discouraged by the WHO and the manufacturer since indiscriminate use may promote emergence of resistant strains of M leprae.

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Nonsteroidal anti-inflammatory drugs

Class Summary

These agents may reduce fever, arthralgia, and pain.

Indomethacin (Indocin)

Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation. Inhibits prostaglandin synthesis.

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