Learning objectives
To understand the pathophysiology,
disease spectrum and subsequent clinical manifestations
To understand current multidisciplinary diagnostic criteria and classification including pertinent radiological features
To become familiar with therapeutic advances including the role of steroids and immunomodulators
Background
History
1961 – Sarles (Fig.
2) et al observed “chronic inflammatory sclerosis of the pancreas” with hypergammaglobulinaemia1
1989 – Dreiling et al described pancreatic involvement in patients with known autoimmune collagen disease 2
1995 – Yoshida et al described a patient with an autoimmune aetiology of chronic pancreatitis in the absence of other another established autoimmune disease and hence defined the concept of autoimmune pancreatitis (AIP) as chronic pancreatitis with respect to specific clinical,
histological and serological findings 3
2001 – Hammano et al discovered...
Findings and procedure details
Imaging Findings - Pancreatic Parenchymal
Should be considered in the context of clinical and pathological findings
Considerable similarity in appearances compared with other differential diagnoses (carcinoma,
lymphoma)
Findings depend upon degree of inflammation and resulting fibrosis
Multimodality approach may be required including CT,
MRI,
ultrasound (transabdominal and endoscopic),
ERCP and FDG PET/CT more recently
Transabdominal Ultrasound
Often the initial imaging modality
Non-specific appearances
Similar to acute pancreatitis features - namely diffusely enlarged hypoechoic pancreas (Fig.
16)
Endoscopic Ultrasound
Significant role in diagnosis of AIP
EUS-guided...
Conclusion
Auto Immune Pancreatitis Key Points
Two subtypes:
Type 1 - IgG4-related disease spectrum - Lymphoplasmacytic sclerosing pancreatitis (LPSP)
Type 2 - Pancreas specific - Idiopathic duct centric pancreatitis (IDCP)
Range of characteristic imaging appearances - can overlap with other pathologies (carcinoma)
Diagnosis relies upon correlation between clinical,
radiological and histological findings
Multidisciplinary approach (ICDC/HISORt)
Careful consideration of both radiological and clinical/biochemical features in focal AIP (rule out pancreatic cancer)
Dramatic reponse to steroids with possible use of immunomodulators
Personal information
Dr.
Gemma Miles MB BS BSc(Hons) FRCR Specialist Registrar
Peninisula Radiology Academy,
William Prance Road,
Plymouth,
UK.
Dr.
Tarig Adlan MB BS MRCP FRCR Specialist Registrar
Dr.
Francesca Wotton MB BS MRCP FRCR Specialist Registrar
Dr.
Simon Jackson FRCS FRCR Consultant Gastrointestinal Radiologist,
Derriford Hospital,
Plymouth,
UK
References
Sarles H,
Sarles JC,
Muratore R,
et al.
Chronic inflammatory sclerosis of the pancreas – an autonomous pancreatic disease? Am J Dig Dis 1961;6:688-98.
Dreiling DA,
Soto JM.
The pancreatic involvement in disseminated “collagen” disorders.
Yoshida K,
Toki F,
Takeuchi T,
et al.
Chronic pancreatitis caused by an autoimmune abnormality.
Proposal of the concept of autoimmune pancreatitis.
Dig Dis Sci 1995;40:1561-8.
Hamano H,
Kawa S,
Horiuchi A ,
Unno H,
Furuya N,
Akamatsu T,
et al.
High serum IgG4 concentrations in patients with sclerosing pancreatitis....