Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone

Disclosures

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

In This Article

Responses to Glucocorticoid Treatment

Symptomatic improvement in AIP is usually obvious within 2 weeks of initiating glucocorticoids. The literature refers to biochemical remissions (normalization of aberrant liver function tests), serologic remissions (normalization of serum IgG4 concentrations), and radiologic remissions [resolution of computed tomography (CT) findings]. Clinical remissions have been defined differently from study to study because the various parameters of treatment response correlate imperfectly with each other. As an example, substantial declines in serum IgG4 concentrations are typical, but complete normalization occurs in only a minority of patients treated with glucocorticoids. In the study by Kamisawa et al.,[15••] 115 of 182 patients (63%) whose serum IgG4 concentrations were measured normalized completely with treatment.

Serum IgG4 concentrations also appear to be an imperfect predictor of disease relapse. Among the patients reported by Kamisawa et al.,[15••,17•] only 30% with persistently elevated IgG4 experienced relapses. In contrast, 10% of those with normal IgG4 concentrations in remission ultimately relapsed. Thus, stability of serum IgG4 concentrations during a clinical remission is reassuring with regard to continuation of that remission, but most patients have elevated IgG4 concentrations after the withdrawal of glucocorticoids and only a minority of those experience relapses. The significance of isolated serological relapse with no clinical disease recurrence and whether these patients need early treatment to prevent clinical relapse are unknown. Our experience suggests that significant increases in serum IgG4 concentrations should prompt closer follow-up of patients for other clinical evidence of active disease. Longer-term follow-up studies and additional biomarker investigations are needed in this disease.

Patients with extrapancreatic manifestations of IgG4-RSD also appear to respond well to glucocorticoids. For example, the glucocorticoid response of the salivary and lacrimal glands in IgG4-related sialadenitis and IgG4-related dacryoadenitis contrast starkly with the failure of glucocorticoids in the glandular swelling associated with Sjögren's syndrome. However, the durability of such treatment responses after prednisone tapering is not clear in these extrapancreatic manifestations of IgG4-RSD. In addition, some organs affected by this condition (e.g. the thyroid gland and retroperitoneum) are prone to the development of extensive fibrosis, following which treatment response is less predictable.

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