Necessity of early intervention for IgG4-related disease--delayed treatment induces fibrosis progression

Shimizu, Yoshitaka; Yamamoto, Motohisa; Naishiro, Yasuyoshi; Sudoh, Gohta; Ishigami, Keiichi; Yajima, Hidetaka; Tabeya, Tetsuya; Matsui, Mikiko; Suzuki, Chisako; Takahashi, Hiroki; Seki, Nobuhiko; Himi, Tetsuo; Yamashita, Ken; Noguchi, Hiroko; Hasegawa, Tadashi; Suzuki, Yasuo; Honda, Saho; Abe, Takashi; Imai, Kohzoh; Shinomura, Yasuhisa

doi:10.1093/rheumatology/kes358

Cited by 99 publications

(53 citation statements)

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“…Previous reports have also described that the clinical presentation and pathology may be distinct according to the damaged tissues (3,22,23). As evidenced in other inflammatory disorders, inflammatory cell infiltration may be observed in the early stage of IgG4-RD and inflammation is replaced with severe fibrosis in the advance stage (2,24).…”

Section: Discussionmentioning

confidence: 86%

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

et al. 2015

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IgG4-related pericardial involvement has rarely been reported and its clinical features remain unknown. We herein report a case of a 50-year-old woman with pericarditis who presented with a fever, elevated C-reactive protein levels, elevated serum IgG4 concentrations, and thickened pericardium with a patchy 18 Ffluorodeoxyglucose (FDG) uptake. A biopsy specimen of 18 F-FDG accumulated in the mediastinal lymph nodes revealed an abundant infiltration of IgG4-bearing plasma cells without fibrosis. Moderate-dose glucocorticoids promptly resolved the physical, serological, and imaging abnormalities, thus indicating a relatively acute and reversible nature of IgG4-related pericardial involvement.

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Section: Discussionmentioning

confidence: 86%

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

et al. 2015

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“…15 Biopsies of the inflamed tissues are the only warrant of a prompt and accurate diagnosis in this condition. FDG-PET scan is the most useful radiological examination to investigate for other organs involvement and to find the most accessible sites to proceed to a biopsy.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related Disease Masquerading as Recurrent Scleritis and Chronic Conjunctivitis

Philippakis

Cassoux

Charlotte

et al. 2014

Ocular Immunology and Inflammation

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“…As our study indicates, type 1 AIP is often accompanied by other immunologic diseases, the most common being sialadenitis (75%). Shimizu et al [14] found that salivary secretion could be improved even in cases with lower levels of salivation before treatment. When the B lymphocytes of the pancreas are inhibited by T lymphocytes and cytokine secreted by macrophages, they will accompany DM with a rate of 33%.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

Wen²,

Huang³

et al. 2013

Digestion

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Aim: To search for the key factors of autoimmune pancreatitis (AIP) in China and to improve the early identification of AIP in order to avoid misdiagnosing it as pancreatic carcinoma. Methods: Clinical, imaging, laboratory, and pathological data about AIP were collected and analyzed from 1996 to 2011 in The Chinese People's Liberation Army General Hospital. Results: A total of 33 patients (male 29, female 4) aged 35-76 years (52.3 ± 9.3 years) meeting the International Consensus Diagnostic Criteria for type 1 (n = 32) or type 2 (n = 1) AIP were included. With improved understanding of AIP, the misdiagnosis rate and unnecessary laparotomy rate was decreased from 95.7% (22/23) and 91.3% (21/23) before 2006, to 20.0% (2/10) (p = 0.001) and 0.0% (p = 0.001) respectively after 2006. The major symptoms were jaundice in (24/33, 72.7%) and abdominal pain (12/33, 36.4%). Half of the patients had other accompanied autoimmune disorders. Laboratory tests showed an elevated level of serum IgG in 9 out of 11 patients (81.8%) and an increased eosinophil count in 16 out of 33 patients (48.5%). The serum IgG4 levels were twice the upper limit of the normal value. CT scan showed a low-contrast margin characterized by a capsule-like rim in 24 of 32 patients (75.0%). Pathologic examinations showed fibrosis and infiltration of massive lymphocytes and plasma cells in the pancreas. Prednisone was given to the patients and proved to be quite effective for all of them (12/12, 100%). Conclusion: Type 1 AIP shows a sex predilection compared with type 2 AIP in China. In order to diagnose AIP exactly, a comprehensive strategy, especially including imaging and pathological examination, should be used. With growing awareness, fewer AIP patients with this diagnosis would be misdiagnosed and incorrectly treated.

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Necessity of early intervention for IgG4-related disease--delayed treatment induces fibrosis progression

Cited by 99 publications

References 13 publications

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

Elevated Serum IgG4 Complicated by Pericardial Involvement with a Patchy <sup>18</sup>F-FDG Uptake in PET/CT: Atypical Presentation of IgG4-related Disease

IgG4-related Disease Masquerading as Recurrent Scleritis and Chronic Conjunctivitis

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

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