Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone


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

In This Article

Efficacy of Glucocorticoids: The Frequency of Relapse

A sizeable subset of AIP patients is refractory to glucocorticoid tapering and suffers frequent relapses. The estimates of disease relapse over the long term are probably low, because most studies to date have had rather short follow-up periods. The risk of disease flare appears to increase substantially with time. In the study by Kamisawa et al.,[15••] the relapse rates in AIP patients treated with steroid was reported as 32% within 6 months after treatment, 56% within 1 year, and 92% within 3 years.

A retrospective study by Hirano et al.[19] suggested that the maintenance of glucocorticoids was associated with a lower relapse rate compared with a regimen leading to complete steroid cessation. The relapse rates in the two unrandomized groups over a mean observation time of 24 months were 32 and 70%, respectively (P = 0.01). Both the high relapse rate despite maintenance glucocorticoids and the higher rate among patients without ongoing therapy are noteworthy here. Another Japanese study by the Research Committee of Intractable Pancreatic Diseases[13] showed a significantly lower relapse rate in AIP patients who were maintained on low-dose steroid therapy compared with patients whose steroid treatment was stopped (26 versus 40%, P < 0.05).

Larger studies of this question with long-term follow-up and careful assessment of adverse events are desirable. Larger studies would also help define the clinical characteristics of patients at risk for relapse, thereby permitting more rational decisions with regard to the use of maintenance glucocorticoids. A variety of risk factors for disease relapse have been suggested (Table 1).[3,9,19–22] Unfortunately, none of these has been confirmed in a prospective study and there is no consensus about patients at high risk for relapse. Significant elevation of serum IgG4 concentration and the presence of IgG4-associated cholangitis have been reported as predictive factors for relapse in AIP.[3,9] Furthermore, all of the data relating to disease relapse in IgG4-RSD pertain to AIP. Data about the likelihood of treatment response and disease relapse in patients with extrapancreatic disease are either sparse or nonexistent.

In addition to disease relapse, it also seems clear that a significant minority of patients fail to achieve optimal disease control despite treatment responses that are regarded as good. In a Mayo Clinic series, resolution of biliary tract strictures and normalization of liver enzyme levels was achieved in only 18 of 30 patients (60%) with glucocorticoids.[3] Improvement but not complete resolution was observed in an additional 11 patients (37%). These data suggest an important potential role for immunosuppressive agents or biologic approaches to therapy.


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