Update in ethiopathogeny, diagnosis and treatment of the IgG4 related disease

Martínez‐Valle, Fernando; Orozco-Gálvez, Olimpia; Fernández‐Codina, Andreu

doi:10.1016/j.medcle.2018.05.005

Cited by 11 publications

(7 citation statements)

References 52 publications

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“…The typical histological features of IgG4-RD include dense lymphoplasmacytic infiltrates, storiform-type fibrosis, and obliterative phlebitis[ 6 ]. The diagnosis of at least two of the above three criteria is needed, usually diffuse lymphoplasmic cell infiltration and storiform-type fibrosis[ 7 , 8 ]. However, lymph nodes, the lung, and other organs and tissues often do not have the characteristic manifestations of storiform-type fibrosis and phlebitis obliterans.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Hao¹,

Yang²,

Bian³

et al. 2021

WJCC

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BACKGROUND Immunoglobulin (Ig) G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases. IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions, such as Castleman disease, lymphatic follicular reactive hyperplasia, and lymphoma. CASE SUMMARY A 65-year-old male patient, with Guillain-Barre syndrome for 5 years, presented to our hospital complaining of bilateral orbital mass for 2 years. After hospitalization, the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L, but serum interleukin-6 was normal. The pathological morphology of orbital mass and cervical lymph node were consistent, which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles, and the interstitial fibrous tissue was proliferative. Immunohistochemistry showed that CD20 (B cells) (+), CD3 (T cells) (+), CD38 (+), IgG (+), IgG4 positive cells > 100/high powered field, and IgG4/IgG > 40%. Combined with clinical and immunohistochemical results, lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease. Prednisone acetate treatment was given at 40 mg/d. After 2 wk, the superficial lymph nodes and orbital masses shrank, and the IgG4 level decreased. As prednisone acetate was regularly used at a reduced dosage, no recurrence of the disease has been observed. CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients, and immunoglobulin, complement, interleukin-6, C-reactive protein, and other examinations should be performed to confirm the diagnosis.

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Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Hao¹,

Yang²,

Bian³

et al. 2021

WJCC

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“…IgG4-RD can affect a single organ or multiple organ systems and can be found bilaterally in organs, and an increase in the size of the affected organs or impairment of organ functions occurs (13). Among the serological findings, the serum IgG4 concentration is expected to be >135 mg/dl; however, the serum IgG4 level did not increase in half of the reported cases (14). Besides, the serum IgG4 level may also be high in patients with chronic diseases and certain malignancies (15).…”

Section: Case Reportmentioning

confidence: 93%

Igg4-related disease of the ovary

Akyol

Atalay²,

Hasdemir³

et al. 2020

TJPATH

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Immunoglobulin G4-related disease is characterized by dense fibrosis, obliterative phlebitis, and lymphoplasmacytic infiltration that contains abundant IgG4 positive plasma cells. It causes tumefactive lesions in the involved organs and is most commonly seen in the salivary glands, pancreas, and retroperitoneum. Ovarian involvement has been reported in only two cases. In our case, a 58-year-old female patient presented with abdominal distention and pain. Pelvic computed tomography revealed a soft tissue lesion compatible with the omental cake, several intraabdominal implants, and bilateral adnexal fullness. A laparotomy was performed under suspicion of peritoneal carcinomatosis secondary to bilateral adnexal mass. In the histopathologic examination, abundant lymphoplasmacytic infiltration and dense fibrosis were observed in both ovaries and the peritoneum. In the areas of greatest density, the density of IgG4-positive plasma cells was found to range from 40 to 50 per high-power field. The patient was accepted as suffering from probable IgG4-related disease because of the bilateral involvement of the ovaries and the histopathological findings. In conclusion, we present this case to draw attention to the fact that IgG4-related disease can also be seen in the ovary.

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“…According to these criteria, an autoimmune disease must be associated to a specific autoantigen against of which specific autoantibodies are produced. Investigation on this regard has not yet recorded a definite description of a specific autoantigen-autoantibody axis in the pathophysiology of IgG4-RD [22]. Nevertheless, there are other antibody-mediated syndromes in internal medicine, which are described as autoimmune and include a distinctive cluster of seronegative phenotype (Antiphospholipid Syndrome-APS) [23].…”

Section: Igg4-rd: An Autoimmune Disease?mentioning

confidence: 99%

IgG4 related disease: Current prospectives.

Sarantopoulou¹,

Sarantopoulos²,

Farmaki³

2018

immunology-case-reports.

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IgG4, a subclass of IgG, has been the subject of intense scientific research, resulting in the classification of heterogeneous clinical medical conditions, defined as IgG4 Related Disease (IgG4-RD). The ongoing investigation on refining the diagnosis, classification and pathophysiology of IgG4-RD, generated further questions on the role of IgG4 in inflammation, autoimmunty and induction of diseases and syndromes. On this regard, emerging reports indicate that this subclass of immunoglobulin G, may participate on the pathophysiology of autoimmune diseases, like Rheumatoid Arthritis. These reports provide new aspects of well-defined diseases of Internal Medicine, and support the fundamental knowledge immunologists rigorously support, that IgGs' role exceeds the boundaries of infections and actively participate in regulating the host's general homeostasis.

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Update in ethiopathogeny, diagnosis and treatment of the IgG4 related disease

Cited by 11 publications

References 52 publications

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

Igg4-related disease of the ovary

IgG4 related disease: Current prospectives.

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