Sections

Medication

Medication Summary

Various immunosuppressive agents are used in the treatment of microscopic polyangiitis (MPA). The choice of agents depends on the treatment phase (induction versus maintenance of remission) and disease severity.

Class Summary

First line of treatment for induction of remission and usually for maintenance. For induction of remission, use IV methylprednisolone. For maintenance, use prednisone.

Methylprednisolone (Adlone, Medrol, Solu-Medrol)

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Steroids ameliorate effects immune reactions and may limit biphasic anaphylaxis.

Prednisone (Deltasone, Orasone, Sterapred)

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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.

Class Summary

These agents inhibit immune reactions that result from diverse stimuli.

Cyclophosphamide (Cytoxan, Neosar)

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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 growth of normal and neoplastic cells.

Rituximab (Rituxan)

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Chimeric murine/human monoclonal antibody directed against the CD20 antigen found on surface of B-lymphocytes. It is indicated in combination with glucocorticoids for treatment of adult and pediatric patients aged 2 years or older with granulomatosis with polyangiitis (GPA; Wegener granulomatosis) or microscopic polyangiitis (MPA).

Methotrexate (Folex PFS, Rheumatrex)

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Should not be used if serum creatinine clearance is >2.0 mg/dL.

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. An alternative form of less toxic therapy only for localized and nonaggressive disease is combination of MTX and prednisone.

Azathioprine (Imuran)

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

Avacopan (Tavneos)

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Complement 5a receptor (C5aR) antagonist that inhibits the interaction between C5aR and the anaphylatoxin C5a. Indicated as adjunctive treatment of severe active antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis) in combination with glucocorticoids.

Questions

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Media Gallery

Pulmonary alveolar capillaritis.
Histopathology of alveolar hemorrhage in alveolar capillaritis.
Crescentic glomerulonephritis.
Focal segmental glomerulonephritis.
Histopathology of leukocytoclastic angiitis.
Leukocytoclastic angiitis.

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Author

Mehran Farid-Moayer, MD Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD Rheumatologist, Mills-Peninsula Medical Center, Sutter Health

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Lewis Katz School of Medicine at Temple University

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FASN Professor of Medicine, Section of Nephrology-Hypertension, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

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, American Osteopathic Association

Disclosure: Nothing to disclose.

Microscopic Polyangiitis Medication

Medication Summary

Corticosteroids

Class Summary

Methylprednisolone (Adlone, Medrol, Solu-Medrol)

Methylprednisolone (Adlone, Medrol, Solu-Medrol)

Prednisone (Deltasone, Orasone, Sterapred)

Prednisone (Deltasone, Orasone, Sterapred)

Immunosuppressive agents