IgG4-related disease and hypereosinophilic syndrome: Overlapping phenotypes

Moussiegt, Aurore; Müller, R.; Ebbo, Mikaël; Grados, A.; Graveleau, J.; Ackermann, Félix; Bachmeyer, Claude; Bernit, E.; Blanche, P.; Canouï, Etienne; Chaix, Fabrice; Costédoat-Chalumeau, Nathalie; Groote, P. De; Etienne, Nicolas; Fior, R; Flamarion, Edouard; Kennel, Titouan; Launay, David; Lerolle, Nathalie; Limal, Nicolas; Loustau, Valentine; Menage, Elodie; Moulis, Guillaume; Papo, Thomas; Pouchot, J.; Quéméneur, T.; Roumier, Mathilde; Tchérakian, Colas; Lefèvre, Guillaume; Rebours, Vinciane; Kahn, Jean‐Emmanuel; Schleinitz, N.; Groh, Matthieu; Cereo,

doi:10.1016/j.autrev.2021.102889

Cited by 11 publications

(7 citation statements)

References 8 publications

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“…Examples include episodic angioedema with eosinophilia (Gleich Syndrome), eosinophilic granulomatosis with polyangiitis (eGPA), IgG4‐related disease (IgG4‐RD), and the eosinophilia‐myalgia syndrome (EMS) (Table 3 , Table S8 ). 71 , 72 Gleich syndrome is characterized by angioedema, the presence of phenotypically aberrant clonal T cells, and increased polyclonal IgM. 72 eGPA is a systemic eosinophilic vasculitis characterized by peripheral eosinophilia, asthma, and chronic rhinosinusitis with polyps.…”

Section: Specific Syndromes and Organ‐specific Pat...mentioning

confidence: 99%

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Valent

Klion

Roufosse

et al. 2022

Allergy

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Eosinophilia and eosinophil activation are recurrent features in various reactive states and certain hematologic malignancies. In patients with hypereosinophilia (HE), HE-induced organ damage is often encountered and may lead to the diagnosis of a hypereosinophilic syndrome (HES). A number of known mechanisms and etiologies contribute to the development of HE and HES. Based on these etiologies and the origin of eosinophils, HE and HES are divided into primary forms where eosinophils are clonal cells, reactive forms where an underlying reactive or neoplastic condition is detected and eosinophils are considered to be "non-clonal" cells, and idiopathic HE and HES in which neither a clonal nor a reactive underlying pathology is detected.Since 2012, this classification and the related criteria have been widely accepted and regarded as standard. However, during the past few years, new developments in the field and an increasing number of markers and targets have created a need to update these criteria and the classification of HE and HES. To address this challenge, a Working Conference on eosinophil disorders was organized in 2021. In this conference, a panel of experts representing the relevant fields, including allergy, dermatology, hematology, immunology, laboratory medicine, and pathology, met and discussed new markers and concepts as well as refinements in definitions, criteria and classifications of HE and HES. The outcomes of this conference are presented in this article and should assist in the diagnosis and management of patients with HE and HES in daily practice and in the preparation and conduct of clinical trials.

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Section: Specific Syndromes and Organ‐specific Pat...mentioning

confidence: 99%

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Valent

Klion

Roufosse

et al. 2022

Allergy

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“…But here again, histology is susceptible to abuse: some conditions such as Castleman's disease or Rosai–Dorfman's disease may be responsible for IgG4‐RD‐like features 12,47 . Furthermore, HES and eosinophilic granulomatosis and polyangiitis (mostly ANCA‐negative phenotype) may be responsible for genuine overlapping syndromes with IgG4‐RD, defining a spectrum of eosinophil‐mediated diseases that is still poorly characterized 28,80,81 …”

Section: Diagnostic Of Thoracic Igg4‐rdmentioning

confidence: 99%

“…12,47 Furthermore, HES and eosinophilic granulomatosis and polyangiitis (mostly ANCA-negative phenotype) may be responsible for genuine overlapping syndromes with IgG4-RD, defining a spectrum of eosinophil-mediated diseases that is still poorly characterized. 28,80,81 The diagnostic approach (Figure 3) therefore consists of collecting all the clinical, biological, imaging and histological elements in favour of the diagnosis, while keeping in mind…”

Section: Diagnostic Of Thoracic Igg4-rdmentioning

confidence: 99%

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Thoracic manifestations of IgG4‐related disease

et al. 2022

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Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon. It can be detected in up to 30% of patients with systemic IgG4-RD and is the exclusive manifestation of the disease in about 10% of cases. Clinical symptoms are nonspecific and may include dyspnoea, cough or chest pain. Chest CT findings are heterogeneous and primarily include peribronchovascular thickening, nodules, ground-glass opacities and lymphadenopathy. There is no specific diagnostic test for IgG4-RD thoracic involvement, which may mimic malignancy or vasculitis. Therefore, a cautious approach is needed to make an accurate diagnosis: a search for extra-thoracic manifestations, elevated serum IgG4 levels, circulating levels of plasmablasts and pathologic evidence of disease is warranted. Although very suggestive, neither the presence of a polyclonal IgG4 lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis are sufficient to confirm the histological diagnosis. Steroids are recommended as first-line therapy. Rituximab or disease-modifying antirheumatic drugs may be used in relapsed or rare cases of steroid-refractory disease. In this review, we summarize current knowledge regarding the pathophysiology, epidemiology, diagnostic modalities (clinical-biological-imaging-histopathology) and treatment of IgG4-RD thoracic involvement.

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“…Similarly, high levels of serum IgE and absolute eosinophil count at baseline have been associated with a higher risk of relapse during followup (70). The concomitant elevation of serum IgG4, IgE and eosinophils is uncommon, underlying the importance of measuring all of them at baseline for a proper prognostic profiling (72). A single study (71) reported serum IgA as additional biomarker for relapse risk assessment.…”

Section: Biomarkers Related To Prognosis and Predictionmentioning

confidence: 99%

Blood biomarkers recommended for diagnosing and monitoring IgG4-related disease. Considerations from the ERN ReCONNET and collaborating partners

Iaccarino

Talarico²,

Bozzalla-Cassione

et al. 2022

Clinical and Experimental Rheumatology

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Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic, clinically heterogenous fibroinflammatory condition, characterised by an accumulation of IgG4 secreting plasma cells in affected tissues and associated with increased serum IgG4 concentrations. Despite a growing recognition of the disease among clinicians from different specialties worldwide, its indolent nature, lack of a single diagnostic test and ability to mimic other malignant, infective and inflammatory conditions, makes the diagnosis challenging. As treatment options evolve, biomarkers correlating with disease activity, predicting prognosis and response to treatment are deemed required. A multidisciplinary panel of experts from the European Reference Network for Rare and Complex Connective tissue diseases (ERN ReCONNET) and affiliated international partners have performed a narrative literature search and reviewed the current evidence of biomarkers in IgG4-RD, including immunoglobulins, cytokines, chemokines and other soluble immune mediators, and cellular components of the immune system. The aim of this paper is to provide useful information for clinicians as to the utility of biomarkers for diagnosing and monitoring IgG4-RD in clinical routine and sets out recommendations for clinical decision making.

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IgG4-related disease and hypereosinophilic syndrome: Overlapping phenotypes

Cited by 11 publications

References 8 publications

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Thoracic manifestations of IgG4‐related disease

Blood biomarkers recommended for diagnosing and monitoring IgG4-related disease. Considerations from the ERN ReCONNET and collaborating partners

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