Page 73 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 49

rarely necessitate permanent withdrawal of the medication. However, pancreatitis and
gastritis severe enough to warrant hospitalization can occur. Second, bone marrow
suppression occurs in nearly all patients, usually manifested by mild pancytopenia. Third,
there is typically a long delay in the onset of the therapeutic effect of 3 months or more.
Taking all of these factors into account, most clinicians concur with the slow advancement of
the dose over weeks, commencing with 50 mg/day and achieving maintenance levels of 2 to 3
mg/kg/day with careful monitoring of liver and marrow function.

Mycophenolate mofetil is an inhibitor of purine synthesis. It has been traditionally
employed to prevent organ transplant rejection. Initial enthusiasm for mycophenolate in
refractory autoimmune disorders was tempered by recognition of its predisposition to
systemic tumor formation as a rare side effect as well as the inability to demonstrate
superiority over corticosteroids and other immunosuppressants. Nevertheless, most experts
agree that patients with systemic vasculitides who are poor candidates for corticosteroids,
cyclophosphamide, and are intolerant of azathioprine may be given effective, safe and long-
term treatment with mycophenolate [223, 224].

While azathioprine and methotrexate appeared to be equally effective in maintaining
remission in GPA [225], mycophenolate mofetil was less effective than azathioprine [226]; it
can be used as an alternative to cyclophosphamide, azathioprine and methotrexate in patients
AAV with renal impairment where is carries less risk [227].

High dose IVIg therapy is the most widely employed immunomodulating agent for
autoimmune neurological disorders [228]. It is alternative therapy for CNS and PNS
vasculitis and diverse connective tissue disorders. Among 22 patients with relapsing AAV
including 19 with GPA and 3 MPA, IVIg was administered at the dose of 0.5 grams/kg/day
for four days as additional therapy monthly for six months in conjunction with corticosteroids
and immunosuppressants (21 patients) [229]. IVIg induced complete remissions of relapsed
AAV in 13 of 22 patients at nine months. The immunomodulating and anti-inflammatory
actions of IVIg are provided by monthly doses of 2000 mg/kg/ body weight given 400 to 500
mg/kg per day respectively over four to five days each month at a slow drip with
acetaminophen and diphenhydramine pretreatment to prevent the commonest side effects
including headache, fever, chills, rash, erythema, flushing, nausea, myalgia, arthralgia,
abdominal cramps, chest and back pain. True anaphylactic reactions to IVIg can occur in
recipients with documented prior allergies to immune globulins or antibodies, especially IgA
type. Transient reversible renal insufficiency occurs in individuals with preexisting renal
disease. Susceptible individuals can be identified by less than normal expected 24-hour
creatinine clearance rates for age and abnormal vascular perfusion on radionuclide scans.
Aseptic meningitis rarely occurs several hours after treatment and resolves over several days
with discontinuation of therapy.

References

[1] Jennette, J. C., Falk, R. J., Bacon, P. A., et al. 2012 Revised International Chapel Hill
Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65:1-11.

[2] Ruperto, N., Ozen, S., Pistorio, A., et al. EULAR/PINTO/PRES criteria for Henoch-
Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis

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