Pancreatitis autoinmune. Serie de casos y actualización bibliográfica

The concept and diagnostic criteria for autoimmune pancreatitis have evolved since the first case reports published in the 1960s. The advances in knowledge during the past two decades have resulted in the recognition of several pancreatic conditions that show, on histological evaluation, variable combinations of inflammation and fibrosis. In fact, autoimmune pancreatitis comprises two distinct diseases: type 1 and type 2. Type 2 autoimmune pancreatitis is a particular pancreatic disease about which there is limited data; it differs from the ‘classic’ IgG4-related type 1 autoimmune pancreatitis in terms of epidemiology, pathological characteristics and natural evolution. There are patients with complex pancreatic conditions where an autoimmune component and good response to steroids are demonstrated, but they do not meet the suggested criteria for the diagnosis of type 1 or type 2 autoimmune pancreatitis. Although the term steroid-responsive pancreatitis may be interpreted as a synonym to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that improve with this type of treatment. Here, we present three clinical cases and a literature review related to the diagnosis and treatment of autoimmune pancreatitis and other less frequent pancreatic disorders that present a good response to steroid therapy.

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A Novel Pathogenic CALR Exon 9 Mutation in a Patient with Essential Thrombocythemia

Laboratory Medicine ◽

10.1093/labmed/lmz064 ◽

2019 ◽

Vol 51 (3) ◽

pp. 306-309

Author(s):

Jee-Soo Lee ◽

Ho Young Kim ◽

Miyoung Kim ◽

Young Kyung Lee

Keyword(s):

Essential Thrombocythemia ◽

Frameshift Mutation ◽

Myeloproliferative Neoplasms ◽

Case Reports ◽

Individual Case ◽

First Case ◽

Exon 9 ◽

Calr Mutations

Abstract The clinical phenotypes and prognoses of CALR-mutant myeloproliferative neoplasms depend on the mutation type. The 2 most common mutations, type 1 (52-bp deletion) and type 2 (5-bp insertion), account for 85% of CALR-mutated neoplasms. The former confers a myelofibrotic phenotype, and the latter is associated with a low risk of thrombosis and an indolent clinical course. Individual case reports for patients with novel pathogenic CALR mutations are rare. Herein, we present the first case in the literature, to our knowledge, of a 63-year old ethnic Korean man with essential thrombocythemia who was diagnosed with a novel +1-bp frameshift mutation in CALR, which was predicted to exhibit a type 2–like phenotype.

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A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma

Case Reports in Gastroenterology ◽

10.1159/000486443 ◽

2018 ◽

Vol 12 (1) ◽

pp. 46-55 ◽

Cited By ~ 2

Author(s):

Partha Hota ◽

Tejas Patel ◽

Xiaofeng Zhao ◽

Nirag Jhala ◽

Omar Agosto

Keyword(s):

Acute Pancreatitis ◽

Mr Imaging ◽

Pancreatic Adenocarcinoma ◽

Autoimmune Pancreatitis ◽

Diagnostic Pitfall ◽

First Case ◽

Imaging Characteristics ◽

Sclerosing Pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized form of acute pancreatitis characterized by obstructive jaundice with a rapid and dramatic treatment response to steroid therapy. Recently, AIP has been divided into two distinct phenotypes: lymphoplasmocytic sclerosing pancreatitis AIP (type 1) and idiopathic duct-centric pancreatitis AIP (type 2); each of which have their own distinct demographics, diagnostic criteria, and histopathological features. We report, to the best of our knowledge, the first case of a multifocal pattern of type 2 AIP characterized with both CT and MR imaging. This rare imaging pattern of AIP may mimic the appearance of more worrisome malignant etiologies such as multifocal pancreatic adenocarcinoma or lymphoma, with overlapping imaging characteristics potentially complicating or delaying diagnosis. Therefore, recognition of this atypical pattern of AIP and avoidance of this potential diagnostic pitfall is crucial.

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Management of autoimmune pancreatitis

Herald of Pancreatic Club ◽

10.33149/vkp.2020.03.02 ◽

2020 ◽

Vol 48 (3) ◽

pp. 13-17

Author(s):

V. S. Akshintala ◽

V. K. Singh

Keyword(s):

Autoimmune Pancreatitis ◽

Pancreatic Disease ◽

Lymphoplasmacytic Sclerosing Pancreatitis ◽

Sclerosing Pancreatitis ◽

Histological Characteristics ◽

Igg4 Level

The article contains modern data on such a chronic pancreatic disease as autoimmune pancreatitis (AIP). Statistical data on the prevalence and incidence of AIP are presented, the current international classification of this pathology is considered, the main features of type 1 AIP (lymphoplasmacytic sclerosing pancreatitis, LPSP) and type 2 AIP (idiopathic duct centric pancreatitis, IDCP) are put forward. The clinical manifestation of these types is different: obstructive jaundice develops more often in patients with LPSP, while patients with IDCP mostly have acute pancreatitis. The presence and variety of extrapancreatic manifestations of the disease with extremely frequent concomitant development of ulcerative colitis is emphasized. The features of serological diagnosis of AIP and the role of IgG4 level in determining the type of disease are considered. The proper techniques of AIP instrumental diagnostics are listed, the typical changes detected during computed tomography and the distinctive histological characteristics of LPSP and IDCP are considered. The feasibility of a differential diagnosis between AIP and pancreatic cancer is indicated by analyzing the results of serological, imaging, and histological studies. The algorithm of management of AIP patients depending on the type of disease is described, as well as the tactics of prescribing corticosteroids, immunomodulators upon LPSP and IDCP. Immunomodulators of choice (mycophenolate mofetil, azathioprine) are indicated, and rituximab administration features are considered. Possible signs of AI recurrence are listed (IgG4-sclerosing cholangitis, high IgE level).

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Diagnostic and therapeutic considerations in biopsy‐proven type 2 autoimmune pancreatitis: comparative analysis with biopsy‐proven type 1 autoimmune pancreatitis

ANZ Journal of Surgery ◽

10.1111/ans.16445 ◽

2020 ◽

Author(s):

Krishna Pattabathula ◽

Peadar S. Waters ◽

Jason Hwang ◽

Mark Bettington ◽

Mahendra Singh ◽

...

Keyword(s):

Comparative Analysis ◽

Autoimmune Pancreatitis ◽

Type 1 Autoimmune Pancreatitis ◽

Therapeutic Considerations

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Clinical features and relapse rates after surgery in type 1 autoimmune pancreatitis differ from type 2: A study of 114 surgically treated European patients

Pancreatology ◽

10.1016/j.pan.2012.03.055 ◽

2012 ◽

Vol 12 (3) ◽

pp. 276-283 ◽

Cited By ~ 55

Author(s):

Sönke Detlefsen ◽

Giuseppe Zamboni ◽

Luca Frulloni ◽

Bernd Feyerabend ◽

Felix Braun ◽

...

Keyword(s):

Clinical Features ◽

Autoimmune Pancreatitis ◽

Type 1 Autoimmune Pancreatitis ◽

Relapse Rates

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Faculty Opinions recommendation of Autoimmune pancreatitis (AIP) type 1 and type 2: an international consensus study on histopathologic diagnostic criteria.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.13383010.14750253 ◽

2011 ◽

Author(s):

Chris Forsmark ◽

Dennis Collins

Keyword(s):

Diagnostic Criteria ◽

Autoimmune Pancreatitis ◽

International Consensus

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Sex-Related Differences of Acute and Chronic Pancreatitis in Adults

Journal of Clinical Medicine ◽

10.3390/jcm10020300 ◽

2021 ◽

Vol 10 (2) ◽

pp. 300

Author(s):

Madeline Drake ◽

Shah-Jahan M. Dodwad ◽

Joy Davis ◽

Lillian S. Kao ◽

Yanna Cao ◽

...

Keyword(s):

Chronic Pancreatitis ◽

The United States ◽

Prevention Strategies ◽

Endoscopic Retrograde ◽

Effective Prevention ◽

Type 1 Autoimmune Pancreatitis ◽

Acute And Chronic Pancreatitis ◽

Effective Prevention Strategies

The incidence of acute and chronic pancreatitis is increasing in the United States. Rates of acute pancreatitis (AP) are similar in both sexes, but chronic pancreatitis (CP) is more common in males. When stratified by etiology, women have higher rates of gallstone AP, while men have higher rates of alcohol- and tobacco-related AP and CP, hypercalcemic AP, hypertriglyceridemic AP, malignancy-related AP, and type 1 autoimmune pancreatitis (AIP). No significant sex-related differences have been reported in medication-induced AP or type 2 AIP. Whether post-endoscopic retrograde cholangiopancreatography pancreatitis is sex-associated remains controversial. Animal models have demonstrated sex-related differences in the rates of induction and severity of AP, CP, and AIP. Animal and human studies have suggested that a combination of risk factor profiles, as well as genes, may be responsible for the observed differences. More investigation into the sex-related differences of AP and CP is desired in order to improve clinical management by developing effective prevention strategies, diagnostics, and therapeutics.

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