Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone


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

In This Article

Consequences of Failing to Diagnose and Treat IgG4-related Systemic Disease Effectively

Much remains unknown about the natural history of IgG4-RSD and its individual organ system manifestations. The treatment series reported to date – all retrospective in nature – have been limited generally by short follow-up periods. On one hand, spontaneous improvement of uncertain duration occurs in a significant number of patients with AIP and has been reported in other organ systems, as well. A substantial percentage of such patients probably relapse over time; however, many go on to develop other organ system manifestations of IgG4-RSD. On the other hand, this fibroinflammatory illness leads to permanent organ system dysfunction and death in some patients. Cirrhosis and portal hypertension result in a significant minority of patients with AIP,[3] and these complications apparently develop within months to years of symptom onset. Potentially irreversible consequences in other organ systems include aortic aneurysm and dissection,[4,5] irreversible fibrosis within the retroperitoneum[6] or thyroid gland,[7] chronic salivary and lacrimal gland dysfunction, and others. Thus, there appears to be a limited window of time when the currently available therapies can be effective. The natural history of IgG4-RSD in some organ systems (e.g. the kidney and lung) remains poorly described.


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