Rethinking Ormond’s Disease: “Idiopathic” Retroperitoneal Fibrosis in the Era of IgG4-Related Disease

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Rethinking Ormond’s Disease: “Idiopathic” Retroperitoneal Fibrosis in the Era of IgG4-Related Disease

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Title: Rethinking Ormond’s Disease: “Idiopathic” Retroperitoneal Fibrosis in the Era of IgG4-Related Disease
Author: Khosroshahi, Arezou; Carruthers, Mollie N.; Stone, John H.; Shinagare, Shweta; Sainani, Nisha; Hasserjian, Robert P.; Deshpande, Vikram

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Citation: Khosroshahi, Arezou, Mollie N. Carruthers, John H. Stone, Shweta Shinagare, Nisha Sainani, Robert P. Hasserjian, and Vikram Deshpande. 2013. “Rethinking Ormond’s Disease: “Idiopathic” Retroperitoneal Fibrosis in the Era of IgG4-Related Disease.” Medicine 92 (2): 82-91. doi:10.1097/MD.0b013e318289610f.
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Abstract: Abstract Idiopathic retroperitoneal fibrosis (RPF) is a periaortic sclerotic disease that encases adjacent retroperitoneal structures, particularly the ureters. A subset of idiopathic RPF cases can be associated with IgG4-related disease, but the frequency of this association is not clear. We selected 23 cases of idiopathic RPF and identified IgG4-related RPF cases based on the presence of IgG4+ plasma cells in the tissue, using an IgG4/IgG ratio cutoff of >40%. We then compared the IgG4-related RPF patients and the non-IgG4-related RPF patients in terms of both the presence of histopathologic features typical of IgG4-related disease and the simultaneous occurrence (or history) of other organ manifestations typical of IgG4-related disease. The IgG4-related RPF and non-IgG4-related RPF groups were also analyzed in terms of clinical, laboratory, and radiologic features and treatment review. We identified 13 cases of IgG4-related RPF (57% of the total cohort). The distinguishing features of IgG4-related RPF were histopathologic and extra-organ manifestations of IgG4-related disease. The IgG4-related RPF patients were statistically more likely than non-IgG4-related RPF patients to have retroperitoneal biopsies showing lymphoplasmacytic infiltrate (p = 0.006), storiform fibrosis (p = 0.006), or tissue eosinophilia (p = 0.0002). Demographics of the 2 groups, including a middle-aged, male predominance (mean age, 58 yr; 73% male), were similar. IgG4-related disease accounts for a substantial percentage of patients with “idiopathic” RPF. Histopathologic features such as storiform fibrosis, obliterative phlebitis, and tissue eosinophilia are critical to identifying this disease association. Extraretroperitoneal manifestations of IgG4-related disease are also often present among patients with IgG4-related RPF. Elevated IgG4/total IgG ratios in tissue biopsies are more useful than the number of IgG4+ plasma cells per high-power field in cases of RPF that are highly fibrotic.
Published Version: doi:10.1097/MD.0b013e318289610f
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