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Lymphadenopathy

  • Kazushige UchidaEmail author
  • Kazuichi Okazaki
Chapter

Abstract

Lymphadenopathy is well known as a common extrapancreatic lesion in type 1 autoimmune pancreatitis (AIP). Generally, steroid therapy is effective similar to type 1 AIP. Lymphadenopathy appears synchronously or metachronously with type 1 AIP. IgG4-related lymphadenopathy may sometimes mimic or be misdiagnosed as lymphoma, multicentric Castleman disease, or disseminated malignancy (e.g., pancreatic cancer). In IgG4-related lymphadenopathy, five histological patterns are recognized: type I, multicentric Castleman disease-like; type II, follicular hyperplasia; type III, interfollicular expansion; type IV, progressive transformation of germinal center; and type V, nodal inflammatory pseudotumor-like. Generally, IgG4-related disease (RD) (especially type 1 AIP) appears to have 3 major histopathological features, including (1) dense lymphoplasmacytic infiltrate, (2) storiform fibrosis, and (3) obliterative phlebitis. On the other hand, IgG4-related lymphadenopathy showed no unique histological features. Fibrosis is only seen in inflammatory pseudotumor-like type. However, IgG4-RD still remains unclear. Further studies are needed to clarify the pathogenesis of lymphadenopathy, including the role of IgG4.

Keywords

Autoimmune Pancreatitis Obliterative Phlebitis Extrapancreatic Lesion Multicentric Castleman Disease Polyclonal Hypergammaglobulinemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Sarles H, Sarles JC, Muratore R, et al. Chronic inflammatory sclerosis of the pancreas–an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–98.PubMedCrossRefGoogle Scholar
  2. 2.
    Kawaguchi K, Koike M, Tsuruta K, et al. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–95.PubMedCrossRefGoogle Scholar
  3. 3.
    Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci. 1995;40:1561–8.PubMedCrossRefGoogle Scholar
  4. 4.
    Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.PubMedCrossRefGoogle Scholar
  5. 5.
    Ito T, Nakano I, Koyanagi S, et al. Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy. Dig Dis Sci. 1997;42:1458–68.PubMedCrossRefGoogle Scholar
  6. 6.
    Horiuchi A, Kawa S, Akamatsu T, et al. Characteristic pancreatic duct appearance in autoimmune chronic pancreatitis: a case report and review of the Japanese literature. Am J Gastroenterol. 1998;93:260–3.PubMedCrossRefGoogle Scholar
  7. 7.
    Uchida K, Okazaki K, Konishi Y, et al. Clinical analysis of autoimmune-related pancreatitis. Am J Gastroenterol. 2000;95:2788–94.PubMedCrossRefGoogle Scholar
  8. 8.
    Okazaki K, Uchida K, Chiba T. Recent concept of autoimmune-related pancreatitis. J Gastroenterol. 2001;36:293–302.PubMedCrossRefGoogle Scholar
  9. 9.
    Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.PubMedCrossRefGoogle Scholar
  10. 10.
    Zamboni G, Luttges J, Capelli P, et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445:552–63.PubMedCrossRefGoogle Scholar
  11. 11.
    Notohara K, Burgart LJ, Yadav D, et al. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003;27:1119–27.PubMedCrossRefGoogle Scholar
  12. 12.
    Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–8.PubMedCrossRefGoogle Scholar
  13. 13.
    Hart PA, Kamisawa T, Brugge WR, et al. Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut. 2013;62:1771–6.PubMedCentralPubMedCrossRefGoogle Scholar
  14. 14.
    Kamisawa T, Funata N, Hayashi Y, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003;52:683–7.PubMedCentralPubMedCrossRefGoogle Scholar
  15. 15.
    Mori S, Tokuda H, Sakai F, et al. Radiological features and therapeutic responses of pulmonary nontuberculous mycobacterial disease in rheumatoid arthritis patients receiving biological agents: a retrospective multicenter study in Japan. Mod Rheumatol. 2012;22:727–37.PubMedCentralPubMedCrossRefGoogle Scholar
  16. 16.
    Erkelens GW, Vleggaar FP, Lesterhuis W, et al. Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet. 1999;354:43–4.PubMedCrossRefGoogle Scholar
  17. 17.
    Nakazawa T, Ohara H, Yamada T, et al. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis. Hepatogastroenterology. 2001;48:625–30.PubMedGoogle Scholar
  18. 18.
    Hamano H, Kawa S, Ochi Y, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359:1403–4.PubMedCrossRefGoogle Scholar
  19. 19.
    Saeki T, Nishi S, Imai N, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2010;78:1016–23.PubMedCrossRefGoogle Scholar
  20. 20.
    Takeda S, Haratake J, Kasai T, et al. IgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis. Nephrol Dial Transplant. 2004;19:474–6.PubMedCrossRefGoogle Scholar
  21. 21.
    Uchiyama-Tanaka Y, Mori Y, Kimura T, et al. Acute tubulointerstitial nephritis associated with autoimmune-related pancreatitis. Am J Kidney Dis. 2004;43:e18–25.PubMedCrossRefGoogle Scholar
  22. 22.
    Shimatsu A, Oki Y, Fujisawa I, et al. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocr J. 2009;56:1033–41.PubMedCrossRefGoogle Scholar
  23. 23.
    Li Y, Nishihara E, Hirokawa M, et al. Distinct clinical, serological, and sonographic characteristics of Hashimoto’s thyroiditis based with and without IgG4-positive plasma cells. J Clin Endocrinol Metab. 2010;95:1309–17.PubMedCrossRefGoogle Scholar
  24. 24.
    Komatsu K, Hamano H, Ochi Y, et al. High prevalence of hypothyroidism in patients with autoimmune pancreatitis. Dig Dis Sci. 2005;50:1052–7.PubMedCrossRefGoogle Scholar
  25. 25.
    Yoshimura Y, Takeda S, Ieki Y, et al. IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med. 2006;45:897–901.PubMedCrossRefGoogle Scholar
  26. 26.
    Kasashima S, Zen Y, Kawashima A, et al. A clinicopathologic study of immunoglobulin G4-related sclerosing disease of the thoracic aorta. J Vasc Surg. 2010;52:1587–95.PubMedCrossRefGoogle Scholar
  27. 27.
    Kamisawa T, Chari ST, Giday SA, et al. Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas. 2011;40:809–14.PubMedCrossRefGoogle Scholar
  28. 28.
    Okazaki K, Uchida K, Fukui T. Recent advances in autoimmune pancreatitis: concept, diagnosis, and pathogenesis. J Gastroenterol. 2008;43:409–18.PubMedCrossRefGoogle Scholar
  29. 29.
    Vlachou PA, Khalili K, Jang HJ, et al. IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations. Radiographics. 2011;31:1379–402.PubMedCrossRefGoogle Scholar
  30. 30.
    Hamano H, Arakura N, Muraki T, et al. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41:1197–205.PubMedCrossRefGoogle Scholar
  31. 31.
    Cheuk W, Yuen HK, Chu SY, et al. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32:671–81.PubMedCrossRefGoogle Scholar
  32. 32.
    Sato Y, Kojima M, Takata K, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman’s disease. Mod Pathol. 2009;22:589–99.PubMedCrossRefGoogle Scholar
  33. 33.
    Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.PubMedCrossRefGoogle Scholar
  34. 34.
    Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.PubMedCrossRefGoogle Scholar
  35. 35.
    Grimm KE, Barry TS, Chizhevsky V, et al. Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells. Mod Pathol. 2012;25:480–91.PubMedCrossRefGoogle Scholar
  36. 36.
    Sato Y, Notohara K, Kojima M, et al. IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int. 2010;60:247–58.PubMedCrossRefGoogle Scholar
  37. 37.
    Sato Y, Yoshino T. IgG4-Related Lymphadenopathy. Int J Rheumatol. 2012;2012:572539.PubMedCentralPubMedCrossRefGoogle Scholar
  38. 38.
    Cheuk W, Chan JK. Lymphadenopathy of IgG4-related disease: an underdiagnosed and overdiagnosed entity. Semin Diagn Pathol. 2012;29:226–34.PubMedCrossRefGoogle Scholar
  39. 39.
    Peterson BA, Frizzera G. Multicentric Castleman’s disease. Semin Oncol. 1993;20:636–47.PubMedGoogle Scholar
  40. 40.
    Sato Y, Kojima M, Takata K, et al. Multicentric Castleman’s disease with abundant IgG4-positive cells: a clinical and pathological analysis of six cases. J Clin Pathol. 2010;63:1084–9.PubMedCrossRefGoogle Scholar
  41. 41.
    Koo CH, Nathwani BN, Winberg CD, et al. Atypical lymphoplasmacytic and immunoblastic proliferation in lymph nodes of patients with autoimmune disease (autoimmune disease- associated lymphadenopathy). Medicine (Baltimore). 1984;63:274–90.Google Scholar
  42. 42.
    Segal GH, Clough JD, Tubbs RR. Autoimmune and iatrogenic causes of lymphadenopathy. Semin Oncol. 1993;20:611–26.PubMedGoogle Scholar
  43. 43.
    Kojima M, Nakamura S, Shimizu K, et al. Clinical implication of idiopathic plasmacytic lymphadenopathy with polyclonal hypergammaglobulinemia: a report of 16 cases. Int J Surg Pathol. 2004;12:25–30.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Division of Gastroenterology and Hepatology, The Third Department of Internal MedicineKansai Medical UniversityOsakaJapan

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