IgG4-Related Disease of the Appendix

Veerankutty, Fadl H; Saleem, Suhail; Chacko, Sidharth; Sreekumar, Vipin I; Krishnan, Prasad; Varma, Deepak; Kurumboor, Prakash

doi:10.1007/s11605-017-3636-2

Cited by 8 publications

(4 citation statements)

References 3 publications

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“…To the best of our knowledge, this specific presentation has not been previously described. Mucocele formation secondary to trauma or surgery is common, but it is seldom associated with IgG4‐RD 3 . We hypothesize that the inflammation of the IgG4‐RD has caused the formation of the primary mucocele and complicated healing resulted in the multicystic process.…”

Section: Discussionmentioning

confidence: 94%

“…Mucocele formation secondary to trauma or surgery is common, but it is seldom associated with IgG4-RD. 3 We hypothesize that the inflammation of the IgG4-RD has caused the formation of the primary mucocele and complicated healing resulted in the multicystic process. Histopathology of the first biopsy showed features suggestive of IgG4-RD, but did not meet the criteria in accordance with the Boston consensus statement.…”

Section: Discussionmentioning

confidence: 99%

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Vision loss caused by immunoglobulin G4–related disease of the skull base complicated by a mucocele of the sphenoid sinus

Paridaens

Verdijk

Laar

et al. 2022

Int Forum Allergy Rhinol

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder and manifestation in de paranasal and sphenoid sinus is well recognized. In this patient, IgG4-RD presented in an unusual manner with vision loss due to mucocele formation in the sphenoid sinus.Case Description: A 19-year-old man, with an unremarkable medical history, was referred with decreased vision in the left eye, headaches, and a sharp pain in the left orbit and ear. Compression of the left optic nerve due to a large mucocele caused papillary edema and emergency endoscopic marsupialization of the mucocele was performed. When the vision decreased again, a more extensive decompressing sphenoidotomy was performed. Histopathology showed IgG4-RD. Despite dexamethasone, the lesion expanded to the anterior skull base and the patient required repeat endoscopic surgery. After 3 months, a decrease in smell and vision warranted for a fourth extensive endoscopic decompressing surgery, complicated by a cerebrospinal fluid leak. Prednisone and later rituximab were commenced. Unfortunately, the patient reported a complete loss of vision after 4 months of rituximab due to increased mass effect on the optic nerve. An extensive combined craniofacial-endoscopic surgery was performed to remove the entire mucocele and to prevent further contralateral and intracranial progression. Methylprednisolone monthly was commenced to prevent further complications.Discussion: This case illustrates that in therapy-resistant sino-orbital IgG4-RD, extensive surgery might be necessary at an earlier stage. It may even be the only option to prevent irreversible damage to the surrounding tissues. A

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Vision loss caused by immunoglobulin G4–related disease of the skull base complicated by a mucocele of the sphenoid sinus

Paridaens

Verdijk

Laar

et al. 2022

Int Forum Allergy Rhinol

View full text Add to dashboard Cite

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“…8 However, the authors stated the absence of more specific and diagnostic histopathologic, such as obliterative phlebitis and storiform fibrosis. 10 Similarly in 2018, Veerankutty et al briefly described an appendiceal lesion that mimicked a mucocel on computed tomography scan, but the histology revealed patchy lymphoplasmacytic infiltration and storiform pattern of fibrosis 9 but lacking obliterative phlebitis. The International Committee stated that in the absence of other histologic and clinical findings, IgG4 to IgG ratio alone is insufficient to make diagnosis of IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

“…1 However, reports on appendiceal involvement are very limited. Currently, there are only 3 cases that describe a mass-forming lesion with increased IgG4 plasma cells in the appendix, [7][8][9] but none of the cases fulfill all 3 histologic criteria. 10 In this report, we present the first case of IgG4-RD in the appendix that satisfies the full spectrum of histologic…”

Section: Introductionmentioning

confidence: 99%

IgG4-Related Appendiceal Disease: A First Case Report Fulfilling All Pathological Diagnostic Criteria and With Concomitant S100-Positive Dendritic/Schwann Cell Hyperplasia

et al. 2019

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IgG4-related disease is a recent entity that has been described in a wide variety of organ systems. A 46-year-old female presented with acute appendicitis accompanied by a mass-forming lesion, raising a concern for neoplasm, and therefore, hemicolectomy was performed. The lesion revealed a dense lymphoplasmacytic infiltrate accompanied by storiform fibrosis and obliterative phlebitis. The IgG4/IgG plasma cell ratio was >50%, and the number of IgG4-positive plasma cells was >100/high-power field. In order to assess the IgG4/IgG plasma cell ratio, MUM1 was employed instead of IgG to successfully estimate the plasma cell concentration. There was also a concomitant hyperplasia of S100-positive cell, which could represent dendritic or Schwannian origin and possibly play a pathophysiologic role. The hyperplasia was significant by itself that it may mimic a mass-forming lesion. This newly described entity of the past decade deserves increased recognition due to clinical implication and surgical morbidity. This is the first case of IgG4-related disease in the appendix to our knowledge that fully satisfied all the pathological diagnostic criteria. We would like to also highlight our innovative approach of evaluating the IgG4/IgG plasma cell.

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Non-neoplastic Diseases of Appendix

Said

2019

Surgical Pathology of Non-Neoplastic Gastrointestinal Diseases

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IgG4-Related Disease of the Appendix

Cited by 8 publications

References 3 publications

Vision loss caused by immunoglobulin G4–related disease of the skull base complicated by a mucocele of the sphenoid sinus

Vision loss caused by immunoglobulin G4–related disease of the skull base complicated by a mucocele of the sphenoid sinus

IgG4-Related Appendiceal Disease: A First Case Report Fulfilling All Pathological Diagnostic Criteria and With Concomitant S100-Positive Dendritic/Schwann Cell Hyperplasia

Non-neoplastic Diseases of Appendix

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