Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone


Curr Opin Rheumatol. 2011;23(1):67-71. 

In This Article

Glucocorticoid Regimens

Multiple glucocorticoid regimens for AIP have been reported in the literature.[17•] The essential questions in designing a glucocorticoid treatment plan are the quantity of glucocorticoids required to control the disease and induce remission; the appropriateness of maintenance therapy; and achieving a balance between the beneficial effects of glucocorticoids and their potential for adverse effects.

Long-term glucocorticoid therapy is potentially problematic in patients with IgG4-RSD, for several reasons. First, the disorder often occurs in the elderly, who are already at heightened risk for osteoporosis and complications of glucose intolerance. Second, patients with AIP have a predilection for glucose intolerance because of their underlying pancreatic disease. Although glucocorticoids may improve glucose intolerance initially, the long-term maintenance of euglycemia is complicated by ongoing glucocorticoid use. Third, the data for maintenance glucocorticoid use are slim. In one study from Japan,[15••] 60% of patients who were never treated with glucocorticoids (i.e. who underwent spontaneous remissions) did not relapse during the follow-up period. Such data call into question the wisdom of maintaining patients on long-term glucocorticoids, unless the doses are very low and patients have demonstrated a propensity to flare.

Two main schools of thought with regard to glucocorticoids have emerged. A consensus statement from 17 referral centers in Japan suggested treating patients initially with prednisolone, 0.6 mg/kg/day for 2–4 weeks.[16] This starting dose equates to 42 mg/day of prednisone for a 70-kg individual. The authors suggested further that the prednisolone be tapered over a period of 3–6 months to a dose of 5 mg/day, and then continued at a dose between 2.5–5 mg/day for up to 3 years. Approximately 25% of patients in the long-term retrospective study reported by Kamisawa et al. [15••] experienced disease flares despite receiving maintenance glucocorticoids.

A contrasting approach has been established at the Mayo Clinic,[3,18] wherein the practice is to initiate treatment with prednisone 40 mg/day and to maintain this dose for 4 weeks. A 7-week prednisone taper is then begun, tapering prednisone by 5 mg/week, such that the treatment course is completed and the patient is off all therapies by the end of 11 weeks. With this regimen, more than 50% of AIP patients relapsed within a median of 3 months (range: 0–14 months) after discontinuing glucocorticoids.[3] Most of the patients who relapsed had IgG4-associated cholangitis as well as AIP.

In addition to initial treatment with prednisone, many patients with AIP also undergo biliary stenting procedures. The placement of biliary stents is believed to hasten symptomatic improvement. Treatment with glucocorticoids, in turn, is believed to shorten the time that biliary stents are necessary.[3]


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