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IgG4-staining plasma cells within a pathologic sample and the numbers and percentages of IgG4-positive cells probably vary from tissue to tissue and stage of the disease. In general, however, one can feel comfortable about the diagnosis in the setting of ³30 IgG4-positive plasma cells per high-power field and an IgG4:total IgG ratio of >50%.
18.5.6Diagnostic Criteria
There are no widely accepted criteria for the diagnosis of IgG4-RD. Three key features are required for an unequivocal diagnosis of IgG4-RD, although it is not essential for all three are not present in all cases. These are either tumefactive organ enlargement or a destructive inflammatory infiltrate; an elevated serum IgG4 concentration; and pathologic features that include elevated numbers of IgG4-positive plasma cells and elevated IgG4:IgG ratios. A careful evaluation to exclude the diagnosis of lymphoma is required prior to rendering a diagnosis of IgG4-RD.
18.5.7Pathogenesis of IgG4-RD
Despite the remarkable advances achieved in the clinical realm, much remains to be understood about the pathogenesis of IgG4-RD. Several lines of evidence are consistent with an autoimmune hypothesis, but an equally plausible alternate is that IgG4-RD is a chronic allergic reaction. Several human leukocyte antigen (HLA) (DRB1_0405 and DQB1_0401) and non-HLA haplotypes/genotypes have been associated with susceptibility to IgG4-related disease or to disease relapse after glucocorticoid therapy [70, 71].
The disease is typically associated with a T-helper 2 immune reaction, which may explain the robust IgG4 response in these individuals [72, 73]. But it is not certain that IgG4 antibody plays a direct pathogenetic role. Specific, target-directed autoantibodies of the IgG4 subclass have not been detected in IgG4-RD. Some studies have suggested that IgG4 suppresses the immune response, thereby fulfilling a protective role [74].
18.6Conclusions
Pancreatic injury and exocrine dysfunction is a feature of SjS, but the true incidence of pancreatic dysfunction in this condition is difficult to assess. In contrast to AIP, a condition in which the exocrine dysfunction is often profound, the pancreatic
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involvement in SjS is relatively mild. IgG4-RD affects virtually the same organs as SjS including the salivary and lacrimal glands, pancreas, biliary tree, kidney, the lung, and skin. It is thus not surprising that many reports on SjS published prior to the recognition of IgG4-RD almost certainly represent the latter entity. The presence of elevated serum IgG4 and characteristic histological features further aided by IgG4 immunoperoxidase stain clinches diagnosis of IgG4-RD and excludes SjS.
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Chapter 19
Nephro-Urological Involvement
Andreas V. Goules and Haralampos M. Moutsopoulos
Contents |
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19.1 Introduction................................................................................................................. |
266 |
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19.2 Interstitial Nephritis in Primary Sjögren’s Syndrome ............................................ |
266 |
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19.2.1 |
Historical Aspects ........................................................................................... |
266 |
19.2.2 |
Clinical Features ............................................................................................. |
267 |
19.2.3 |
Histology......................................................................................................... |
269 |
19.2.4 |
Pathogenesis.................................................................................................... |
269 |
19.2.5 |
Differential Diagnosis ..................................................................................... |
270 |
19.2.6 |
Treatment ........................................................................................................ |
270 |
19.3 Glomerulonephritis in Primary Sjögren’s Syndrome.............................................. |
271 |
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19.3.1 |
Historical Aspects ........................................................................................... |
271 |
19.3.2 |
Clinical Features ............................................................................................. |
271 |
19.3.3 |
Histology......................................................................................................... |
272 |
19.3.4 |
Pathogenesis.................................................................................................... |
273 |
19.3.5 |
Differential Diagnosis ..................................................................................... |
274 |
19.3.6 |
Treatment ........................................................................................................ |
274 |
19.4Painful Bladder Syndrome/Interstitial Cystitis and
Primary Sjögren’s Syndrome..................................................................................... |
275 |
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19.4.1 |
Historical Aspects ........................................................................................... |
275 |
19.4.2 Clinical, Cytoscopic, and Histologic Features ................................................ |
275 |
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19.4.3 Pathogenesis and Association with Sjögren’s Syndrome................................ |
276 |
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19.4.4 |
Differential Diagnosis ..................................................................................... |
276 |
19.4.5 |
Treatment ........................................................................................................ |
277 |
References.............................................................................................................................. |
. |
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A.V. Goules (*) • H.M. Moutsopoulos
Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece
M. Ramos-Casals et al. (eds.), Sjögren’s Syndrome, |
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DOI 10.1007/978-0-85729-947-5_19, © Springer-Verlag London Limited 2012 |
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