IgG4-related disease manifesting as symptoms of appendicitis: Case report and literature review.

Published on Jan 18, 2021in Clinical Journal of Gastroenterology
· DOI :10.1007/S12328-020-01337-8
Iván Eduardo Cabrales-Escobar (Mexican Social Security Institute), Enrique Murcio-Pérez3
Estimated H-index: 3
(Mexican Social Security Institute),
Alejandra Albarrán-Sánchez2
Estimated H-index: 2
(Mexican Social Security Institute)
Immunoglobulin G subclass 4 (IgG4)-related disease is a recently described fibroinflammatory condition. Reports of appendix involvement are extremely limited. A young man with abdominal pain and symptoms of acute appendicitis accompanied by the finding of an appendix-dependent tumor during surgery is presented. Histopathological study revealed lymphoplasmocytic infiltrate, storiform fibrosis and obliterative phlebitis. The number of IgG4-positive plasma cells was greater than 50 per high power field. Postsurgical steroid treatment and radiological findings are also described.
#1Dong Hyang Kwon (Georgetown University)H-Index: 2
#2Bhaskar Kallakury (Georgetown University)H-Index: 33
Last. Norio Azumi (Georgetown University)H-Index: 27
view all 4 authors...
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....
3 CitationsSource
#1Fadl H Veerankutty (Aster)H-Index: 3
#2Suhail Saleem (Aster)H-Index: 1
Last. Prakash Kurumboor (Aster)H-Index: 1
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5 CitationsSource
#1Orwah M Al-Khalili (UNMC: University of Nebraska Medical Center)H-Index: 1
#2Alan R Erickson (UNMC: University of Nebraska Medical Center)H-Index: 7
: IgG4-related disease (IgG-RD) describes a group of fibroinflammatory diseases that affect a variety of tissues resulting in tumor-like effect and/or organ dysfunction. Clinical presentation varies according to the tissue(s) involved, and diagnosis relies on tissue findings of dense infiltration of IgG4-positive plasma cells and a characteristic storiform fibrosis. Treatment is mainly with glucocorticoids, while multiple immunosuppressive medications can be used as adjuvant agents. Rituximab ha...
5 Citations
#1Hisanori UmeharaH-Index: 43
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 68
Last. Tsutomu Chiba (Kyoto University)H-Index: 81
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AbstractIgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of He...
93 CitationsSource
#1Tsukasa Ikeura (Kansai Medical University)H-Index: 17
#2Shunsuke Horitani (Kansai Medical University)H-Index: 1
Last. Kazuichi Okazaki (Kansai Medical University)H-Index: 68
view all 11 authors...
: A 63-year-old woman presented to our hospital with elevated levels of serum IgG4, marked wall thickening of the gallbladder, hepatomegaly, and abdominal lymphadenopathy. She experienced a recurrent fever and leg edema. Her laboratory data demonstrated anemia, hypoalbuminemia, and elevated serum levels of interleukin-6 and C-reactive protein. The patient was eventually diagnosed with IgG4-related disease according to the comprehensive diagnostic criteria, although the patient exhibited common c...
7 CitationsSource
#1Hiroshi Sekiguchi (Mayo Clinic)H-Index: 17
#2Ryohei Horie (Mayo Clinic)H-Index: 6
Last. Jay H. Ryu (Mayo Clinic)H-Index: 80
view all 6 authors...
Objective To investigate clinical and pathologic aspects of IgG4-related disease (IgG4-RD) in non-Asian populations. Methods We conducted a retrospective review of the medical records of patients with IgG4-RD who presented to an academic medical center from January 1994 to September 2012. Results Among 166 patients identified, the median age at diagnosis was 61 years (interquartile range [IQR] 49–70 years), 75% were male, and 80% were white. The median number of organs involved was 2 (IQR 2–3). ...
41 CitationsSource
#1Pilar Brito-ZerónH-Index: 45
#2Belchin KostovH-Index: 16
Last. John H. StoneH-Index: 76
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To review the reported evidence on the therapeutic management of IgG4-related disease (IgG4-RD) in clinical practice. A systematic search of the literature was conducted. The primary outcome measured was the rate of efficacy of first-line therapeutic approaches. Secondary outcomes measured included the rate of disease relapse, the outcome of untreated patients, the rate of patients without drug therapy at the end of follow-up, the rate of side effects, and mortality. The MOOSE, AHRQ, STROBE, and...
76 CitationsSource
#1Faiz Karim (EUR: Erasmus University Rotterdam)H-Index: 4
#2J. Loeffen (Boston Children's Hospital)H-Index: 1
Last. Jan A. M. van Laar (EUR: Erasmus University Rotterdam)H-Index: 12
view all 7 authors...
Background Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature provides an overview of all reports published on IgG4-RD in children in order to create awareness of Ig...
49 CitationsSource
#1Hyun Soo Kim (Catholic University of Korea)H-Index: 5
#2Won Kyung Kang (Catholic University of Korea)H-Index: 16
Last. Dong Jin Chung (Catholic University of Korea)H-Index: 5
view all 3 authors...
Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a ma...
10 CitationsSource
#1Zachary S. Wallace (Harvard University)H-Index: 25
#2Vikram Deshpande (Harvard University)H-Index: 100
Last. John H. Stone (Harvard University)H-Index: 76
view all 7 authors...
Objective IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect nearly any organ. Prior studies have focused on individual cases of IgG4-RD or small case series. This study was undertaken to report detailed clinical and laboratory findings in a larger group of patients with IgG4-RD whose diagnosis was established by strict clinicopathologic correlation. Methods The baseline features of 125 patients with biopsy-proven IgG4-RD were reviewed. The diagnosis...
265 CitationsSource
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