“…Radiological findings are also largely non-specific in most affected organs 8687. IgG4 related AIP with diffuse involvement is the sole exception, because computed tomography and magnetic resonance imaging classically show a diffusely enlarged “sausage shaped” pancreas with a surrounding halo of edematous tissue (fig 2).…”
Section: Diagnosis Of Igg4 Related Diseasementioning
IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.
“…Radiological findings are also largely non-specific in most affected organs 8687. IgG4 related AIP with diffuse involvement is the sole exception, because computed tomography and magnetic resonance imaging classically show a diffusely enlarged “sausage shaped” pancreas with a surrounding halo of edematous tissue (fig 2).…”
Section: Diagnosis Of Igg4 Related Diseasementioning
IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.
“…Da bildgebende sowie klinische Parameter oft überlappend und in manchen Fällen sogar wiederholte Biopsie-Ergebnisse nicht aussagekräftig bzw. unklar sind [17], sollen hier weitere sekundäre bildgebende Merkmale diskutiert werden, die zwischen entzündlichen und malignen Entitäten differenzieren können [26,28]. Die genaue Kenntnis dieser sekundären Zeichen kann dem Radiologen bei der Unterscheidung CP vs. Pankreaskarzinom helfen und auch die Malignitätswahrscheinlichkeit abschätzen.…”
Section: Chronic Pancreatitis Characterization and Differentiation From Pancreatic Cancerunclassified
Zusammenfassung
Klinisches/methodisches Problem
Bei der chronischen Pankreatitis (CP) handelt es sich um eine langanhaltende Entzündung der Bauchspeicheldrüse, welche die normale Struktur und Funktion des Organs schädigt. Das breite Spektrum an entzündlichen Pankreaserkrankungen umfasst einzelne Entitäten, wie die fokale Pankreatitis (FP) oder den Pseudotumor („mass-forming pancreatitis“), welche radiomorphologisch ein Adenokarzinom der Bauchspeicheldrüse (PDAC) nachahmen können. In weiterer Folge kann eine Fehldiagnose zu einem vermeidbaren und unnötigen operativen Eingriff oder zu einer Therapieverzögerung führen.
Radiologische Standardverfahren
Der Ultraschall (US) ist das primäre bildgebende Verfahren zur Abklärung von Pankreaserkrankungen, gefolgt von kontrastmittelverstärkter Computertomographie (KM-CT), die als meistverwendete Methode bei der diagnostischen Abklärung von Bauchspeicheldrüsenerkrankungen gilt. Die Magnetresonanztomographie (MRT) und/oder die MR-Cholangiopankreatographie (MRCP) können als Problemlöser eingesetzt werden, um zwischen soliden und zystischen Läsionen zu unterscheiden sowie auch Anomalien der Pankreasgänge auszuschließen, welche bei rezidivierender akuter Pankreatitis (AP) vorhanden sein können, oder um frühe Anzeichen einer CP zu visualisieren. Die MRCP hat dabei die diagnostische endoskopische retrograde Cholangiopankreatographie (ERCP) in der Abklärung von therapeutischen Interventionen im Wesentlichen ersetzt.
Empfehlung für die Praxis
Folgender Übersichtsartikel fasst die relevanten Merkmale in der Computertomographie (CT) und MRT zusammen, um eine akkurate, frühzeitige Diagnose einer CP zu stellen und eine Differenzierung zwischen FP und Pankreaskarzinom zu ermöglichen, um somit – auch in schwierigen Fällen – ein adäquates Therapiemanagement zu gewährleisten.
“…42 Typical imaging features at CT-scan or MRI include a diffusely enlarged pancreas with featureless borders ("sausage-shaped" appearance) and delayed enhancement with or without a capsule-like rim. 52 MRCP may reveal a diffuse irregular narrowing or a focal stricture of the main pancreatic duct (Figure 8). 2.…”
It is frequently challenging to make the correct diagnosis in patients with biliary strictures. This is particularly important as errors may have disastrous consequences. Benign-appearing strictures treated with stents may later be revealed to be malignant and unnecessary surgery for benign strictures carries a high morbidity rate. In the first part of the review, the essential information that clinicians need to know about diseases responsible for biliary strictures is presented, with a focus on the most recent data. Then, the characteristics and pitfalls of the methods used to make the diagnosis are summarized. These include serum biomarkers, imaging studies, and endoscopic modalities. As tissue diagnosis is the only 100% specific tool, it is described in detail, including techniques for tissue acquisition and their yields, how to prepare samples, and what to expect from the pathologist. Tricks to increase diagnostic yields are described. Clues are then presented for the differential diagnosis between primary and secondary sclerosing cholangitis, IgG4-related sclerosing cholangitis, cholangiocarcinoma, pancreatic cancer, autoimmune pancreatitis, and less frequent diseases. Finally, algorithms that will help to achieve the correct diagnosis are proposed.
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