IgG4-Related Disease

Martín‐Nares, Eduardo; Hernández-Molina, Gabriela; Baenas, Diego Federico; Paira, Sergio

doi:10.1097/rhu.0000000000001787

Cited by 21 publications

(18 citation statements)

References 68 publications

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“…For differential diagnosis, the literature 7,8 showed that mimickers of IgG4-RD were mainly malignancy, vasculitis, Sjögren's syndrome, sarcoidosis, multicentric Castleman's disease, and infections.…”

Section: Discussionmentioning

confidence: 99%

IgG4‐related disease presenting as severely symptomatic hypercalcemia: A case report and review of literature

2023

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Immunoglobulin G4‐related disease (IgG4‐RD)‐associated hypercalcemia has rarely been reported. We report a case of IgG4‐RD that presented as severe symptomatic hypercalcemia. A 50‐year‐old woman with a history of sustained bilateral periorbital swelling and proptosis for more than 5 years presented to our hospital complaining of a 3‐day history of significant and progressive nausea, vomiting, loss of appetite, fatigue, and pruritus. She denied a long history of medication. On admission, laboratory tests showed severe hypercalcemia with serum adjusted calcium elevated to 4.34 mmol/L and renal dysfunction with serum creatinine elevated to 206 μmol/L. Urinary calcium excretion was increased. The serum IgG4 subclass was markedly elevated to 22.4 g/L with polyclonal hypergammaglobulinemia. Tests of autoantibodies were all negative. Bone metabolism markers that reflect the activity of osteoblasts and osteoclasts were all significantly elevated. However, the levels of intact parathyroid hormone and 25(OH) vitamin D3 were decreased. B‐ultrasonography showed chronic inflammation of bilateral submandibular glands. Neither bone marrow biopsy nor positron emission tomography – computed tomography examination showed evidence of neoplastic diseases. The patient was treated with intravenous saline infusion, loop diuretics, salmon calcitonin, glucocorticoids, and hemodialysis with a good response.

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Section: Discussionmentioning

confidence: 99%

IgG4‐related disease presenting as severely symptomatic hypercalcemia: A case report and review of literature

2023

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“…Misdiagnosis may be due to the fact that iMCD patients may have an elevated serum IgG4 level, while some cases of IgG4-RD may show Castleman-like histopathology. Besides, both conditions present with systemic lymphadenopathy with extranodal involvement, and affected organs may overlap between the two conditions [ 2 , 71 ].…”

Section: Inflammatory Conditions That Resemble Clinical or Histopatho...mentioning

confidence: 99%

“…In general, patients with IgG4-RD tend to be older than patients with iMCD [ 2 ]. Clinically, findings suggestive of iMCD are the presence of fever, high CRP, IL-6 and IgA levels and the absence of orbital, salivary gland and pancreatic involvement [ 71 ]. Atopic manifestations history is observed in almost 70% of the IgG4-RD cases but in <30% of patients with iMCD [ 66 ].…”

Section: Inflammatory Conditions That Resemble Clinical or Histopatho...mentioning

confidence: 99%

Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

2022

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Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterised by systemic inflammatory symptoms, generalised lymphadenopathy, polyclonal lymphocyte proliferation, and organ dysfunction caused by a hyperinflammatory state. It accounts for one third to one half of all multicentric Castleman disease (MCD) cases. iMCD is often associated with autoimmune manifestations that may precede the iMCD diagnosis, be identified at the same time or follow it. In addition, iMCD may also coincide with a number of autoimmune diseases (such as psoriasis or myasthenia gravis) or autoinflammatory diseases (such as familial Mediterranean fever). Moreover, diverse inflammatory disorders, such as rheumatoid arthritis, systemic lupus erythematosus, adult-onset Still disease, systemic juvenile idiopathic arthritis, immunoglobulin (IgG4) related disease, or the recently described VEXAS syndrome, can present clinical features or lymphadenopathy with histopathological “Castleman-like” findings compatible with those of iMCD. Given the iMCD clinical heterogeneity and the overlap with other autoimmune or autoinflammatory disorders, iMCD diagnosis can be challenging. In this review, we explore the overlap between iMCD and inflammatory diseases and provide practical guidance on iMCD diagnosis in order to avoid misdiagnosis and confusion with other autoimmune or autoinflammatory conditions.

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“…Despite the presence of heightened level of IgG4-positive plasma cells in the mucous membrane of the intestine during inflammatory bowel disease, this disease is not a condition of IgG4-related disease. The conditions of IBDs have no storiform fibrosis and phlebitis in their histological and clinical features, and therefore do not correspond to the profile of IgG4-related disease (Tanaka et al, 2018;Satou et al, 2020;Martín-Nares et al, 2022).…”

Section: Introductionmentioning

confidence: 99%

Diagnosis of inflammatory bowel disease according to human IgG4 and possibilities of evaluating efficacy of the therapy

Stepanov

Тарасова

Stoykevich

et al. 2022

Regul. Mech. Biosyst.

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Taking into account the progress of understanding diagnosis, course prognosis, evaluation of effectiveness of therapy of inflammatory bowel disease and also differentiation diagnosis between its main forms – ulcerative colitis and Crohn’s disease, the search for efficient non-invasive markers for solving those issues is extremely relevant. The patients were divided into groups depending on nosology and severity of the course of the disease. All the patients had undergone endoscopic study for diagnosis verification and biopsy samples were taken for further detection of tissue IgG4 using the immunohistochemical method. Also, we determined concentration of serum IgG4. Increase in IgG4content in blood serum was determined in 54.0% of the cases of inflammatory bowel disease. Concentration of IgG4 in patients suffering ulcerative colitis was higher (by 2.31 and 2.46 times) compared with its level in the control group and patients with Crohn’s disease, respectively. We found relationships between the concentration of serum IgG4 and the activity of the disease. In patients with ulcerative colitis, increased tissue IgG4 was found more often than in patients with Crohn’s disease (by 2.77 times, Р < 0.05). We determined the relationship between tissue IgG4 and histological activity. Simultaneous increase in serum IgG4 and presence of tissue IgG4 during ulcerative colitis were more frequent than during Crohn’s disease (by 2.66 times). In all examined groups of patients, we determined decrease in serum IgG4 content (by 1.66 times) after treatment. Concentration of serum IgG4 and positive tissue IgG4 in ulcerative colitis patients exceeded such in Crohn’s disease patients, which may be used for differentiation diagnosis between those disease types. We determined dependence of IgG4 concentration on severity and duration of the disease, which could be used as a prognostic marker. Decrease in IgG4 content in blood serum against the background of the therapy shows that this indicator could be used as a marker of treatment efficacy. Perspectives of further studies are as follows: parameters of concentration of serum IgG4 and presence of tissue IgG4 could be used as diagnostic and prognostic biomarkers and be introduced to practice for differentiation diagnosis between ulcerative colitis and Crohn’s disease, and could be used as prognostic marker of severity of the disease and therapy efficacy.

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IgG4-Related Disease

Cited by 21 publications

References 68 publications

IgG4‐related disease presenting as severely symptomatic hypercalcemia: A case report and review of literature

IgG4‐related disease presenting as severely symptomatic hypercalcemia: A case report and review of literature

Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

Diagnosis of inflammatory bowel disease according to human IgG4 and possibilities of evaluating efficacy of the therapy

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