IgG4–related paratesticular pseudotumor in a patient with autoimmune pancreatitis and retroperitoneal fibrosis: an extrapancreatic manifestation of IgG4–related disease

The number of patients with autoimmune pancreatitis who visited hospitals in Japan in 2007 was approximately 2709 (95% confidence interval; range 2540–3040). Because IgG4-related disease is a new clinical entity, there are no data with regard to its prevalence. To estimate the number of patients with IgG4-related disease in Japan, we randomly selected hospitals using stratification and asked them how many patients they had with IgG4-related disease in 2009. The number of patients with Mikulicz’s disease, IgG4-related retroperitoneal fibrosis, IgG4-related renal disease, IgG4-related pulmonary disease, and IgG4-related lymphadenopathy who visited hospitals in Japan in 2009 was approximately 4304 (95% confidence interval; range 3360–5048), 272 (95% confidence interval; range 264–306), 57 (95% confidence interval; range 47–66), 354 (95% confidence interval; range 283–424), and 203 (95% confidence interval; range 187–240), respectively. The total number of patients with IgG4-related disease without autoimmune pancreatitis in Japan was approximately 5190 (95% confidence interval; range 4141–6084). The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years. The total number of patients with IgG4-related disease in Japan in 2009, including autoimmune pancreatitis, was approximately 8000.

Download Full-text

A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

SAGE Open Medical Case Reports ◽

10.1177/2050313x211016993 ◽

2021 ◽

Vol 9 ◽

pp. 2050313X2110169

Author(s):

Kim Rouven Liedtke ◽

Christoph Käding ◽

Paula Döring ◽

Sander Bekeschus ◽

Anne Susann Glitsch

Keyword(s):

Retroperitoneal Fibrosis ◽

Inflammatory Diseases ◽

Histological Change ◽

Iliac Vessel ◽

Related Disease ◽

Igg4 Related Disease ◽

Cystic Tumors ◽

Retroperitoneal Cyst ◽

Retroperitoneal Lymphangioma ◽

Severe Fibrosis

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

Download Full-text

IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report

Annals of Medicine and Surgery ◽

10.1016/j.amsu.2020.12.028 ◽

2021 ◽

Vol 61 ◽

pp. 69-72

Author(s):

Almurtada Razok ◽

Rubab Malik ◽

Priyanka Cackamvalli ◽

Muhammad Zahid

Keyword(s):

Case Report ◽

Retroperitoneal Fibrosis ◽

Organ Involvement ◽

Related Disease ◽

Igg4 Related Disease

Download Full-text

Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease

Journal of Gastroenterology ◽

10.1007/s00535-011-0386-x ◽

2011 ◽

Vol 46 (3) ◽

pp. 277-288 ◽

Cited By ~ 185

Author(s):

Kazuichi Okazaki ◽

Kazushige Uchida ◽

Masanori Koyabu ◽

Hideaki Miyoshi ◽

Makoto Takaoka

Keyword(s):

Autoimmune Pancreatitis ◽

Related Disease ◽

Igg4 Related Disease ◽

Recent Advances

Download Full-text

Erdheim-Chester Disease: The Importance of Information Integration

Case Reports in Oncology ◽

10.1159/000477658 ◽

2017 ◽

Vol 10 (2) ◽

pp. 613-619 ◽

Cited By ~ 5

Author(s):

Anna Nikonova ◽

Khashayar Esfahani ◽

Guillaume Chausse ◽

Stephan Probst ◽

Tina Petrogiannis-Haliotis ◽

...

Keyword(s):

Retroperitoneal Fibrosis ◽

Braf V600e Mutation ◽

Braf V600e ◽

High Dose ◽

Related Disease ◽

Igg4 Related Disease ◽

Erdheim Chester Disease ◽

History Of ◽

Erdheim Chester ◽

Chester Disease

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. Case Presentation: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease. However, the patient was refractory to high-dose steroids and progressed further, developing an epicardial soft tissue mass and recurrent neurological symptoms. Integration of the above findings with new information at another hospital about a radiological history of symmetrical lower extremities long bone lesions raised the differential diagnosis of ECD. Molecular analysis of formalin-fixed paraffin-embedded tissue of both of the patient’s retroperitoneal biopsies (the second one of which had shown a small focus of foamy histiocytes, CD68+/CD1a–) was positive for BRAF mutation, confirming the diagnosis of ECD. The patient demonstrated a dramatic and sustained metabolic response to vemurafenib on follow-up positron emission tomography scans. Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis that could represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.

Download Full-text