Primary Angiitis of the CNS Medication

Updated: Nov 10, 2021
  • Author: Mazen Noufal, MD; Chief Editor: Helmi L Lutsep, MD  more...
  • Print

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.



Class Summary

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

Prednisone (Sterapred)

Used as an immunosuppressant in the treatment of vasculitis and autoimmune disorders. It decreases inflammation by suppression of migration of polymorphonuclear leukocytes and reversal of increased capillary permeability, suppresses the immune system by reducing activity and volume of the lymphatic system, and suppresses adrenal function at high doses.


Immunosuppressant agents

Class Summary

These agents interfere with processes that promote immune reactions resulting from diverse stimuli.

Cyclophosphamide (Cytoxan, Neosar)

Alkylating agent. Activated in the liver to its active metabolite, 4-hydroxycyclophosphamide, which alkylates the target sites in susceptible cells. The mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

When used in autoimmune diseases, mechanism of action is thought to involve immunosuppression due to destruction of immune cells via DNA cross-linking.

In high doses, affects B cells by inhibiting clonal expansion and suppression of production of immunoglobulins. With long-term, low-dose therapy, affects T cell functions.

Azathioprine (Imuran)

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. Mechanism whereby azathioprine affects autoimmune diseases unknown. Works primarily on T cells. Suppresses hypersensitivities of cell-mediated type and causes variable alterations in antibody production. Immunosuppressive, delayed hypersensitivity, and cellular cytotoxicity tests are suppressed to a greater degree than antibody responses. Works very slowly; may require 6-12 mo of trial prior to effect. Up to 10% of patients may have idiosyncratic reaction disallowing use. Do not allow WBC count to drop below 3000/mL or lymphocyte count to drop below 1000/mL.


Calcium channel blockers

Class Summary

Inhibits calcium ions from entering slow channels or select voltage-sensitive areas of vascular smooth muscle.

Nifedipine (Adalat, Procardia)

Inhibits calcium ion from entering the slow channels or select voltage-sensitive areas of vascular smooth muscle producing vasodilation.

Nimodipine (Nimotop)

For improvement of neurologic impairments resulting from cerebrovascular spasms.

Nimodipine shares the pharmacology of other calcium channel blockers; animal studies indicate that nimodipine has a greater effect on cerebral arteries than other arteries; this increased specificity may be due to the drug's increased lipophilicity.

Verapamil (Calan, Calan SR, Covera-HS, Verelan)

Inhibits calcium ion from entering the slow channels or select voltage-sensitive areas of vascular smooth muscle during depolarization; produces relaxation of vascular smooth muscle and vasodilation; slows automaticity and conduction of AV node.