Advertisement

Rheumatology International

, Volume 35, Issue 10, pp 1749–1752 | Cite as

Immunoglobulin G4-related disease mimicking lymphoma in a Chinese patient

  • Yanhui LiuEmail author
  • Fei Xue
  • Jing Yang
  • Yin Zhang
Case Based Review - Cases with a Message

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. IgG4-RD tends to form tumefactive lesions. As a result, patients are often suspected of having a malignancy such as lymphoma. In this article, a patient with IgG4-RD and the deep vein thrombosis who was initially suspected of lymphoma is reported. The 63-year-old man presented with painless salivary swelling and multi-lymphadenopathy, progressively swelling and pain in the left leg. Salivary biopsy showed IgG4+ plasma cells >50 per high-power field and IgG4+/IgG+ plasma cell ratio >40 %. The serum IgG4 level was 4.28 g/L (range 0.03–2.01 mg/dL). Ultrasonography showed that the inferior vena cava was partially occluded, and thrombosis in the left iliac vein. Computed tomography scan revealed plaque-like tissue surrounding the inferior vena cava and abdominal aortic, which is typical for the diagnosis of retroperitoneal fibrosis. The patient was effectively treated with corticosteroids, interventional therapy, and anticoagulant therapy which resulted in a reduction in the swelling of the lymph nodes and left leg. Patient with IgG4-RD and deep vein thrombosis is rare and could be misdiagnosed easily as malignant disease. Accurate diagnosis is critical for disease management.

Keywords

IgG4-related disease Sialadenitis Fibrosis Retroperitoneal Deep vein thrombosis Treatment 

Notes

Acknowledgments

This work was supported by the Nature Science Foundation Committee (NO. 81273260) and the Project of science and technology of Zhengzhou City (131PPTGG379-1).

Conflict of interest

The authors declare that they have no competing interests.

References

  1. 1.
    O’Reilly DA, Malde DJ, Duncan T et al (2014) Review of the diagnosis, classification and management of autoimmune pancreatitis. World J Gastrointest Pathophysiol 5:71–81PubMedCentralPubMedGoogle Scholar
  2. 2.
    Palazzo E, Palazzo C, Palazzo M (2014) IgG4-related disease. Joint Bone Spine 81:27–31CrossRefPubMedGoogle Scholar
  3. 3.
    Nambam B, Winter WE, Schatz DA (2014) IgG4 antibodies in autoimmune polyglandular disease and IgG4-related endocrinopathies: pathophysiology and clinical characteristics. Curr Opin Pediatr 26:493–499CrossRefPubMedGoogle Scholar
  4. 4.
    Ferry JA, Deshpande V (2012) IgG4-related disease in the head and neck. Semin Diagn Pathol 29:235–244CrossRefPubMedGoogle Scholar
  5. 5.
    Chen H, Lin W, Wang Q et al (2014) IgG4-related disease in a Chinese cohort: a prospective study. Scand J Rheumatol 43:70–74CrossRefPubMedGoogle Scholar
  6. 6.
    Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192CrossRefPubMedGoogle Scholar
  7. 7.
    Geyer JT, Deshpande V (2011) IgG4-associated sialadenitis. Curr Opin Rheumatol 23:95–101CrossRefPubMedGoogle Scholar
  8. 8.
    Sato Y, Yoshino T (2012) IgG4-related lymphadenopathy. Int J Rheumatol 2012:572539PubMedCentralCrossRefPubMedGoogle Scholar
  9. 9.
    Tzou M, Gazeley DJ, Mason PJ (2014) Retroperitoneal fibrosis. Vasc Med 19:407–414CrossRefPubMedGoogle Scholar
  10. 10.
    Tanuma Y, Yokoo A (2002) Idiopathic retroperitoneal fibrosis with large vessel thrombosis. Hinyokika Kiyo 48:539–543PubMedGoogle Scholar
  11. 11.
    Paetzold S, Gary T, Hafner F et al (2013) Thrombosis of the inferior vena cava related to Ormond’s disease. Clin Rheumatol 32:S67–S70CrossRefPubMedGoogle Scholar
  12. 12.
    Kamisawa T, Okazaki K, Kawa S et al (2010) Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol 45:471–477CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of HematologyHenan Provincial People’s Hospital, The People’s Hospital of Zhengzhou UniversityZhengzhouChina
  2. 2.Department of Hepatobiliary and Pancreatic SurgeryHenan Provincial People’s Hospital, The People’s Hospital of Zhengzhou UniversityZhengzhouChina

Personalised recommendations