| Abstract | Autoimuni pankreatitis (AIP) rijetka je bolest koja je prvi put opisana krajem prošlog stoljeća u Japanu. Ova bolest oblik je kroničnog pankreatitisa s ponavljajućim fazama pogoršanja i poboljšanja. Postoje dva tipa AIP-a: tip 1 koji je češći i predstavlja sistemsku imunološku bolest povezanu s IgG4, te tip 2 koji je rjeđi, ograničen na gušteraču i često se pojavljuje u komorbiditetu s ulceroznim kolitisom. Kod tipa 1 najčešći je simptom bezbolna opstruktivna žutica, slijede bolovi u abdomenu i simptomi povezani s drugim organima u sklopu IgG4 povezane bolesti. Tip 2 češće se manifestira kao akutni pankreatitis, odnosno bolovima u abdomenu. Dijagnostika AIP-a složena je i uključuje histološke pretrage, slikovne nalaze, serološke testove, procjenu zahvaćenosti drugih organa i odgovor na terapiju. Serologija i zahvaćanje drugih organa specifični su za dijagnostiku tipa 1, pri čemu se koristi povišena razina IgG4 kao serumski biomarker. Zahvaćanje drugih organa kod IgG4 povezane bolesti može uključivati bilijarni sustav, žlijezde slinovnice i suzne žlijezde, bubrege, aortu i arterije, te dišni sustav i retroperitoneum. Važno je diferencijalno-dijagnostički razlikovati AIP od duktalnog adenokarcinoma gušterače zbog njihovih sličnih karakteristika. Endoskopski ultrazvuk s ciljanom biopsijom smatra se zlatnim standardom u dijagnostici. Liječenje AIP-a temelji se na primjeni glukokortikoida. Terapija započinje višom dozom koja se postupno smanjuje na dozu održavanja. U slučaju refraktornosti ili kontraindikacije na steroidnu terapiju, koriste se rituksimab i drugi imunomodulatori kao druga linija liječenja. |
| Abstract (english) | Autoimmune pancreatitis (AIP) is a rare disease first described in Japan at the end of the last century. This disease is a form of chronic pancreatitis, characterized by recurring phases of exacerbation and improvement. There are two types of AIP: type 1, which is more common and represents a systemic immune disease associated with IgG4, and type 2, which is rarer, limited to the pancreas, and often occurs in comorbidity with ulcerative colitis. In type 1, the most common symptom is painless obstructive jaundice, followed by abdominal pain and symptoms related to other organs as part of IgG4-related disease. Type 2 more often manifests as acute pancreatitis, that is, with abdominal pain. The diagnosis of AIP is complex and includes histological examinations, imaging findings, serological tests, assessment of other organ involvement, and response to therapy. Serology and involvement of other organs are specific to the diagnosis of type 1, using elevated IgG4 levels as a serum biomarker. Involvement of other organs in IgG4-related disease can include the biliary system, salivary and lacrimal glands, kidneys, aorta and arteries, as well as the respiratory system and retroperitoneum. It is important to differentiate AIP from ductal adenocarcinoma of the pancreas due to their similar characteristics. Endoscopic ultrasound with targeted biopsy is considered the gold standard in diagnosis. The treatment of AIP is based on the administration of glucocorticoids. The therapy starts with a higher dose that is gradually reduced to a maintenance dose. In case of refractoriness or contraindication to steroid therapy, rituximab and other immunomodulators are used as a second line of treatment. |
