Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease

Laco, Ján; Podhola, Miroslav; Kamarádová, Kateřina; Novák, Ivo; Dobeš, Daniel; Broďák, Miloš; Hácová, Mária; Ryška, Aleš

doi:10.1007/s00428-013-1480-7

Cited by 22 publications

(14 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The pathognomonic significance of the antibodies produced has not yet been sufficiently determined. Published studies suggest that only a portion of patients with Ormond's disease meet this criterion (Zen et al, 2009;Takahashi et al, 2010;Laco et al, 2013). In our cohort, we found increased concentrations of IgG4 in the serum in eight patients, whereas measurements were taken from 18 patients before starting immunotherapy.…”

Section: Discussionmentioning

confidence: 56%

Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

2016

View full text Add to dashboard Cite

We present a retrospective analysis of patients treated in our Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, during 1997-2013 for Ormond's disease. We analyse the clinical history, diagnostic approaches, surgical, and immunosuppressive therapies and their subsequent effect on our patients. 28 patients treated for Ormond's disease were included. Patients with established disease activity (26 patients) were given immunosuppressive treatment, using corticosteroids in combination with azathioprine. Treatment response was evaluated using clinical symptomatology, inflammatory parameters and imaging methods. In the cohort as a whole, immunosuppressive therapy was applied in 26 patients; in two patients it was not used as no inflammatory activity was found with the disease. In all 26 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of inflammatory infiltrate. Out of the total number of 26 patients, two patients experienced disease exacerbation 7 and 16 months after the immunosuppressive treatment was discontinued. The longest follow-up period was 16 years; the shortest one was 21 months. Idiopathic retroperitoneal fibrosis--Ormond's disease--is a disease with serious complications. Standard treatment involves a combination of surgery and immunosuppressive treatment. The combination of corticosteroids and azathioprine represents a potentially safe and useful method of treatment.

show abstract

Section: Discussionmentioning

confidence: 56%

Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

2016

View full text Add to dashboard Cite

show abstract

“…Most pediatric patients had orbital involvement, followed by sinonasal disease, lacrimal gland disease, and sialadenitis. [7] Other manifestations included disease of the mediastinum, retroperitoneum [8] , pancreas, lung, and mesenterium.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease in an adolescent with radiologic-pathologic correlation

Silva

Hoffman

Policeni

et al. 2017

Radiology Case Reports

View full text Add to dashboard Cite

Immunoglobulin G4–related disease (IgG4RD) is an immune-mediated condition characterized by lymphoplasmacytic infiltrates and fibrosis of affected organs. IgG4RD may affect many different organs either individually or together in a multiorgan condition and, thus, incorporates a wide range of fibroinflammatory phenotypes with shared pathologic features. Although IgG4RD most commonly occurs in late adulthood, it may affect children and adolescents. Only one case of IgG4RD presenting as isolated submandibular gland involvement has been reported in the pediatric population. Radiographic features of IgG4RD are often nonspecific making diagnosis challenging, but it is important for radiologists to be familiar with this diseased as its inclusion the differential for diffuse salivary enlargement may be the first step in making an accurate diagnosis. Here, we report a case of a child presenting with bilateral submandibular gland swelling to increase awareness of this condition in the pediatric population.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

show abstract

“…The differential diagnosis is idiopathic and secondary RF caused by drugs, malignancy, Erdheim-Chester disease, infection, radiotherapy, and surgery (40). Although glucocorticoids are a mainstay of treatment of idiopathic and of IgG4-related RF, it is crucial to confirm IgG4-related RF by biopsy for further appropriate follow-up of the extent of the disease and the specific treatment.…”

Section: Retroperitoneummentioning

confidence: 99%

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

105

129

View full text Add to dashboard Cite

IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy.The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression.About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD.This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. 3Highlights Ø IgG4-related disease is a chronic fibrotic inflammation.Ø The disease affects multiple organs, is progressive, and impairs function.Ø The disorder has an allergic background and is immune-mediated.Ø Compatible clinical signs and typical pathology are the basis for the diagnosis.Ø Glucocorticoids and/or B-cell depletion induces prompt clinical response.Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrotic inflammation, characterized by tissue infiltration by lymphocytes, IgG4-positive plasma cells, simultaneous development of fibrosis, and often, but not always, elevated serum levels of IgG4 (1, 2). The disease can affect multiple organs. Early diagnosis of IgG4-RD is not easy because of the indolent course of the disease with vague, nonspecific symptoms. It may first come to clinical attention as visible organ swelling or detected organ dysfunction due to fibrosis, or is identified incidentally by imaging and specific biopsy when the patient undergoes investigation of suspected tumor disease. Most forms of organ involvement in IgG4-RD, even in the advanced fibrotic stages, respond well to glucocorticoids (3, 4). 4A comprehensive search for all articles in E...

show abstract

Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases, with emphasis to possible relationship to IgG4-related disease

Cited by 22 publications

References 34 publications

Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

IgG4-related disease in an adolescent with radiologic-pathologic correlation

IgG4-related disease: A relatively new concept for clinicians

Contact Info

Product

Resources

About