Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone


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

In This Article

Indications for Glucocorticoid Therapy

Some patients with AIP go into remission spontaneously with no treatment. Wakabayashi et al.[8] reported that pancreatic swelling improved in nine of 37 AIP patients (24%) with conservative therapy alone. Kubota et al.[9] compared the clinicopathological presentations of eight AIP patients with spontaneous remission to those of 12 patients who entered remission after glucocorticoid therapy. Patients who achieved spontaneous remissions without requiring glucocorticoid treatment had relatively low serum IgG4 concentrations at baseline, did not have either obstructive jaundice or diabetes mellitus, and showed focal rather than diffuse pancreatic swelling. Neither the study by Wakabayashi nor that of Kubota distinguished between Type 1 and Type 2 AIP,[10•] because the Type 1 versus Type 2 paradigm did not exist at the time of those studies or had only recently been formulated. In future studies of AIP, it is essential to define as carefully as possible how many patients have Type 1 disease and how many have Type 2.

The tendency of some AIP patients to improve spontaneously led to the debate about whether glucocorticoids are indicated for the manifestation of IgG4-RSD. Multiple reports from small series[11] and a nationwide survey by the Research Committee of Intractable Pancreatic Diseases from Japan[12,13] provided evidence of a significantly higher remission rate among steroid-treated AIP patients compared with those who did not receive glucocorticoids. Glucocorticoids have also been reported to shorten the time to remission[12] and to improve pancreatic exocrine function.[14] Based on these data, glucocorticoid treatment has become a standard treatment for AIP over the past several years. Any patient with AIP or another manifestation of IgG4-RSD is a candidate for therapy to prevent short-term or long-term organ damage and to prevent the involvement of other organ systems.

Kamisawa et al. [15••] conducted a large, retrospective, multicenter study at 17 referral centers in Japan to evaluate the efficacy of glucocorticoid treatment in AIP. The remission rate among glucocorticoid-treated patients was significantly higher (98%) than that of patients who did not receive steroids (74%) (P < 0.001). The investigators also suggested a benefit of maintenance doses of glucocorticoids (between 2.5 and 10 mg/day of prednisone) with regard to relapse prevention.

A set of Japanese consensus guidelines for the management of AIP has defined certain indications for glucocorticoid treatment.[16] These include obstructive jaundice, abdominal pain, and back pain, and the presence of symptomatic extrapancreatic lesions.


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