Multicentric Reticulohistiocytosis Medication

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Aug 16, 2011
 

Medication Summary

No drug of choice is known. Most patients with multicentric reticulohistiocytosis (MRH) are treated with oral prednisone, with or without a cytotoxic/immunosuppressive agent such as methotrexate, cyclophosphamide, or chlorambucil.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

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Prednisone (Deltasone, Meticorten, Orasone)

 

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Regularly used but not proven to be effective.

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Cytotoxic/immunosuppressive agents

Class Summary

These are steroid sparing. None have been documented to be effective, except in anecdotal reports.

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Methotrexate (Folex PFS, Rheumatrex)

 

Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).

Adjust dose gradually to attain satisfactory response.

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Cyclophosphamide (Cytoxan, Neosar)

 

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

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Antimalarial

Class Summary

Derivatives of 4-aminoquinoline are active against various autoimmune disorders.

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Hydroxychloroquine (Plaquenil)

 

Although not demonstrated to be effective in studies, anecdotal reports suggest possible effect. Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.

Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

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TNF antagonists

Class Summary

Individual case reports suggest a benefit.

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Adalimumab (Humira)

 

Recombinant human IgG1 monoclonal antibody specific for human TNF. Indicated to reduce inflammation and inhibit progression of structural damage in moderate-to-severe rheumatoid arthritis. Reserved for those who experience inadequate response to one or more disease-modifying antirheumatic drugs (DMARDs). Can be used alone or in combination with methotrexate (MTX) or other DMARDs. Binds specifically to TNF-alpha and blocks interaction with p55 and p75 cell-surface TNF receptors.

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Infliximab (Remicade)

 

Neutralizes cytokine TNF-alpha and inhibits it from binding to TNF-alpha receptor. Consult rheumatologist for use.

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Etanercept (Enbrel)

 

Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses.

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Bisphosphonates

Class Summary

These agents are analogs of pyrophosphate and act by binding to hydroxyapatite in bone matrix, thereby inhibiting the dissolution of crystals. These drugs prevent osteoclast attachment to the bone matrix and osteoclast recruitment and viability.

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Alendronate (Fosamax)

 

Inhibits bone resorption via actions on osteoclasts or osteoclast precursors. Used to treat osteoporosis in both men and women. May reduce bone resorption and incidence of fracture at spine, hip, and wrist by approximately 50%. Should be taken with large glass of water at least 30 min before eating and drinking to maximize absorption. Because of possible esophageal irritation, patients must remain upright after taking the medication. Since it is renally excreted, it is not recommended in patients with moderate-to-severe renal insufficiency, ie, CrCl < 30 mL/min or CrCl >3 mg/dL, and, thus, its use in perirenal transplantation is limited.

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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Specialty Editor Board

Bryan L Martin, DO  Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marcel E Conrad, MD  Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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Multiple erythematous nodules are present on the dorsal hands of this adolescent with an inflammatory arthropathy.
Multiple erythematous to brown nodules on the fingers.
Erythematous, poikilodermatous mamillated plaque on the anterior chest.
Histopathology of multicentric reticulohistiocytosis (MRH) skin lesions.
Histopathology of multicentric reticulohistiocytosis (MRH) skin lesions. Higher power demonstrating the ground glass cytoplasm and multinucleated giant cells.
 
 
 
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