IgG4-associated pancreatitis – difficulties of diagnostics and treatment

Abstract

To date, IgG4-associated pancreatitis – type 1 autoimmune pancreatitis is a fairly rare disease of the pancreas, which causes great difficulties in the choice of therapeutic and diagnostic tactics for both surgeons and gastroenterologists. The disease is poorly understood and rarely discussed in routine clinical practice. Although, perhaps, in fact, the number of such patients is significantly greater. The prevailing etiological variant of the development of autoimmune pancreatitis remains the theory of lymphocyte activation under the influence of IgG4, which is normally one of the smallest immunoglobulins of class G, exhibiting both pro-inflammatory and anti-inflammatory activity. The inflammatory process caused by activated IgG4-positive leukocytes is characterized by the development of a diffuse or local process due to pronounced infiltration by lymphocytes and plasma cells, followed by their activation into fibroblasts and the formation of focal fibrosis. In the presented clinical case, we tried to describe all the difficulties faced by the patient, his family and friends, his attending physicians. A disease that developed over the years and led to a traumatic operation, the diagnosis – the setting of which required many years of diagnostic search, a treatment that allowed to save life and improve its quality, but due to delay, it turned out to be insufficient to fully rehabilitate the patient.