Factors predictive of relapse and spontaneous remission of autoimmune pancreatitis patients treated/not treated with corticosteroids

Kubota, Kensuke; Watanabe, Seitaro; Uchiyama, Takashi; Kato, Shingo; Sekino, Yusuke; Suzuki, Kaori; Mawatari, Hironori; Iida, Hiroshi; Endo, Hideki; Fujita, Koji; Yoneda, Masato; Takahashi, Hirokazu; Kohno, Hiroyuki; Kobayashi, Noritoshi; Saito, Satoru; Sugimori, Kazuya; Hisatomi, Kantaro; Matsuhashi, Nobuyuki; Sato, Hirotaka; Tanida, Emiko; Sakaguchi, Takashi; Fujisawa, Nobutaka

doi:10.1007/s00535-011-0393-y

Cited by 84 publications

(86 citation statements)

References 27 publications

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“…The spontaneous remission rate for AIP is still not well understood because most patients receive corticosteroids as part of their standard therapy. Similar to the findings of the younger brother, however, AIP patients with IgG4 seronegativity have a high likelihood of achieving spontaneous remission (21,22).…”

Section: Discussionsupporting

confidence: 67%

Two Siblings with Type 1 Autoimmune Pancreatitis

et al. 2013

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Type 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the HLA-DRB1 * 04:05-DQB1 * 04:01 haplotype, for which family studies may disclose its immunogenetic significance. In the present study, we encountered two male siblings with type 1 AIP who exhibited diffuse pancreatic swelling with a capsule-like rim and diffuse pancreatic duct stricture. The younger brother also displayed characteristic IgG4-related sialadenitis and retroperitoneal fibrosis. Contrary to our expectations, the siblings showed only normal or slightly elevated values of serum IgG4 and no HLA DRB1 * 04: 05-DQB1 * 04:01 haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.

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Section: Discussionsupporting

confidence: 67%

Two Siblings with Type 1 Autoimmune Pancreatitis

et al. 2013

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“…Disease relapse is now recognised to occur in at least 40% of AIP/IgG4-RD patients, 28,30,31 but appropriate management of this is yet to be defi ned. It is of note that our patient with panhypopituitarism was on maintenance azathioprine at presentation (albeit at a low dose).…”

Section: Discussionmentioning

confidence: 99%

Cerebral involvement in IgG4-related disease

et al. 2015

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IgG4-related disease is a recently recognised multi-system disease. Common organ involvement includes the pancreas, biliary tree and salivary glands. Central nervous system involvement has been infrequently reported. In a single-centre cohort of 84 patients, we report cerebral involvement in three (4%) patients. Details of cerebral involvement in these patients are outlined, including pituitary involvement in two patients and a diffuse autoimmune-like encephalopathy in the other.

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“…Proposed hypotheses include that IgG4 is upregulated in response to microbial and non-microbial antigens, or due to increased production of anti-inflammatory cytokines at the site of chronic inflammation [27,28]. This seems credible, as IgG4 levels fall spontaneously and in response to steroid therapy in patients with type 1 AIP [11,29]. …”

Section: Pathogenesismentioning

confidence: 99%

“…IgG4-sclerosing cholangitis, IgG4-SC), in type 1 AIP [11,32]. Clinical factors associated with an increased risk of relapse in type 1 AIP include IgG4-SC, other-organ involvement, persistently raised serum IgG4 levels, and diffuse pancreatic swelling [29,32,33,34]. In a study of 70 patients with (type 1) AIP by Kubota et al [29], relapse occurred in 45% of patients, and did so within 3 years in 95% of these cases.…”

Section: Treatmentmentioning

confidence: 99%

“…Clinical factors associated with an increased risk of relapse in type 1 AIP include IgG4-SC, other-organ involvement, persistently raised serum IgG4 levels, and diffuse pancreatic swelling [29,32,33,34]. In a study of 70 patients with (type 1) AIP by Kubota et al [29], relapse occurred in 45% of patients, and did so within 3 years in 95% of these cases. Of interest, relapse occurred in 80% of patients receiving steroids for <12 months, but only in 26% of those receiving >12 months treatment.…”

Section: Treatmentmentioning

confidence: 99%

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Autoimmune Pancreatitis - A Riddle Wrapped in an Enigma

Webster¹

2016

Dig Dis

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Autoimmune pancreatitis (AIP) was recognized as a clinical entity, at least in the West little more than 10 years ago. Since then, studies globally, and international collaboration, have led to important advances in our understanding of its clinical features, disease course, and management, although the aetiopathogenesis of this curious disease remains to be fully elucidated. Types 1 and 2 AIP have been described, of which type 1 is the commonest form, and best defined. International consensus now recognizes it as one of the many clinical manifestations of IgG4-related disease, and is now termed IgG4-related pancreatitis (IgG4-RP). The disease is not confined to a particular race, gender, or age, but often presents after the fifth decade in men. A common presentation is with jaundice due to low bile duct obstruction related to diffuse pancreatic enlargement (historically often leading to a misdiagnosis of cancer). Acute pancreatitis is unusual. Other organ involvement is a particular feature, including biliary disease, retroperitoneal fibrosis, generalized lymphadenopathy, renal, and lung involvement. No single test makes the diagnosis, and diagnostic criteria for type 1 AIP/IgG4-RP, which incorporate clinical, laboratory, radiological, pathological, and therapeutic parameters should be applied. A particular attempt should be made to make a histological diagnosis, which is characterized by an IgG4-positive lymphoplasmacytic infiltrate. Management is not based on randomized studies, but corticosteroids are the mainstay of treatment, providing rapid clinical and radiological benefit. However, clinical relapse is common (particularly in type 1 AIP, and in those with associated other organ involvement). Additional immunosuppression may be required, including azathioprine, and rituximab may play an emerging role. The disease course is variable, but loss of organ function (especially pancreatic exocrine failure and pancreatic atrophy) may occur.

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Factors predictive of relapse and spontaneous remission of autoimmune pancreatitis patients treated/not treated with corticosteroids

Abstract: AIP patients with IgG4 seropositivity and jaundice are at a higher risk of relapse and they could therefore be candidates for over 3 years of maintenance CST. AIP patients with IgG4 seronegativity have a high likelihood of SR.

Cited by 84 publications

References 27 publications

Two Siblings with Type 1 Autoimmune Pancreatitis

Two Siblings with Type 1 Autoimmune Pancreatitis

Cerebral involvement in IgG4-related disease

Autoimmune Pancreatitis - A Riddle Wrapped in an Enigma

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