SAT0629 Igg4 related castleman’s disease or “secondary” igg4 related disease of multicenter castleman’s disease?
Authors: X. Zhang, P. zhang, L. peng, Y. fei, W. zhang, R. feng, W. zhang
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> It's widely accepted that a diagnosis of IgG4-related disease (IgG4-RD) can be made under a premise of rule out a series of disease including Castleman's disease (CD). Clinically, the two diseases may share similar clinical manifestations, such as multiple organ involvement. Pathologically, in some cases with CD, IgG4 positive plasma cell infiltrations are also evident. Otherwise, IgG4-RD may pathologically exhibits CD-like feature[.<sup>1–3</sup> <h3>Objectives</h3> When the histopathology coincides with the diagnosis of both IgG4-RD and CD, it is hard to depart the two disease entity utterly. It's unknown weather it is IgG4 related CD or "secondary" IgG4-RD of multicentric CD, here we call it IgG4-CD provisionally. To our knowledge, no comparative study of IgG4-CD and IgG4-RD has yet been published. In this study, we aim to review the clinical feature of IgG4-CD. <h3>Methods</h3> This study is based on a retrospective analysis of a prospectively acquired database. IgG4-CD were defined histopathologically in patients who fulfilled the diagnosis of both IgG4-RD and CD. Forty five definate IgG4-RD patients were recruited as control. Clinical features including organ involvement, serum IgG4, IgG, IgE, ESR and CRP levels of all the participants were collected and analysed statistically. <h3>Results</h3> Fifteen patients (2.8%) out of 534 patients with IgG4-RD in China's largest prospective IgG4-RD and Mimicry cohort fulfilled the definition of IgG4-CD. There were 14 males and 1 female, mean age was 47±18 years, and the median disease duration before diagnosis was 12 (1–132) months. Eight patients have allergic history. IgG4-CD patients had more lymph node involvement (100% vs 57.8%, p&lt;0.01), while IgG4-RD patients had more submandibular (33.3% vs 77.1%, p&lt;0.01) and parotid gland (13.3% vs 40.9%, p&lt;0.05) affected. IgG4-CD patients had significantly higher levels of ESR (mm/h), CRP (mg/L), IgG (g/L), IgG1 (g/L), IgG3(g/L), IgG4(mg/dL), and IgE (g/L) [76.50 (5.00–129.00) vs 17.00 (2.00–89.00), 5.39 (0.54–134.00) vs 1.6 (0.08–113.74), 35.28 (9.76–69.00) vs 19.36 (7.46–61.00), 11.2 (6.84–41.00) vs 8.07 (4.08–16.70), 1.55 (0.19–3.20) vs 0.53 (0.09–2.00), 1590.00 (133.00–6420.00) vs 1070.00 (152.0–6100.00),807.5 (215.00–2967.00) vs 214.00 (20.40–2437.00), respectively. p&lt;0.05). Except for one patient refused to receive drug therapy untreated, the remaining patients with IgG4-CD all received glucocorticoid (GC) treatment. Patients with multi-organ involvement or in severe inflammatory condition were treated with both GC and immunosuppressive agents, those who with active disease but resistant to above regimen accepted biologics such as rituximab. All patients with IgG4-CD exhibited favourable outcomes. <h3>Conclusions</h3> Both IgG4-RD and CD can involve multiple organs. There are a small group of patients who had clinical and pathological characteristics of both CD and IgG4-RD showed better clinical outcome. Long-term prognosis of these patients, the relationship of CD and IgG4-RD are waiting to be further elucidated. <h3>References</h3> [1] Ishida F, Kitano K, Kobayashi H, et al. Elevated IgG4 levels in a case with multicentric Castleman's disease. Br J Haematol1997;99:981–982. [2] Sasaki T, Akiyama M, Kaneko Y, et al. Distinct features distinguishing IgG4-related disease from multicentric Castleman's disease. RMD Open2017;3(1):e000432. <h3>Acknowledgements</h3> None. <h3>Disclosure of Interest</h3> None declared