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634    PART IV   Hepatobiliary and Exocrine Pancreatic Disorders


            architectural changes in the organ, whereas the prevalence   Autoimmune chronic pancreatitis
            of acute, totally reversible diseases will be underestimated   The particular form of chronic pancreatitis recognized
  VetBooks.ir  unless the animal dies during the episode. Nevertheless, it is   in English Cocker Spaniels in Britain is thought to be an
                                                                 immune-mediated disorder (Watson et al., 2011; see  Fig.
            clear that there are many more cases of chronic pancreatitis
            in veterinary practice than currently recognized, and a
                                                                 affects middle-aged to older dogs, with a higher prevalence
            number of these are clinically relevant.             37.7). As in human autoimmune pancreatitis, it typically
                                                                 in males, and at least 50% of affected dogs subsequently
            Idiopathic chronic pancreatitis                      develop DM, EPI, or both. Dogs also often have another
            As in acute pancreatitis, the cause of chronic pancreatitis in   concurrent autoimmune disease, particularly keratocon-
            dogs is usually unknown. Any age or breed of dog can be   junctivitis sicca and glomerulonephritis. There may also be
            affected, but in Britain the most typical is a middle-aged to   an association with anal sacculitis and increased risk of anal
            old dog, particularly a Cavalier King Charles Spaniel, Cocker   sac carcinoma. There is often a mass-like lesion on ultraso-
            Spaniel, Collie, or Boxer (Watson et al., 2007, 2010;  Fig.   nography (see Fig. 37.5, B). Biopsies show a typical peril-
            37.7). One recent study in the United States suggested that   obular, diffuse, fibrotic, and lymphocytic disease centered
            breeds defined by the American Kennel Club as toy and non-  on perilobular ducts and vessels, with loss of large ducts.
            sporting breeds have a higher prevalence of chronic pancre-  Immunohistochemistry shows a preponderance of duct- and
            atitis (Bostrom et al., 2013). An independent large study of   vein-centered CD3+ lymphocytes (i.e., T cells). The disease in
            EPI in Britain found an increased prevalence in older Cava-  humans is believed to be a duct-centered immune reaction.
            lier King Charles Spaniels, supporting a breed association.   Recent work has identified a strong association with plasma
            Other parts of the world have also reported a high prevalence   cells that secrete one subgroup of immunoglobulin G, IgG4.
            in Arctic-type breeds such as Siberian Huskies. There is   The disease in humans has been redefined as multisystemic
            likely to be some overlap with acute disease, although some   because  of  the  frequent  involvement  of  other  organs.  It  is
            cases will have a separate etiology. In humans, both genetic   now defined as IgG4-related disease (Bledsoe et al., 2018),
            and environmental factors determine whether recurrent   and concurrent keratoconjunctivitis sicca, sialoadenitis,
            acute pancreatitis becomes more chronic with development   biliary tract disease, and glomerulonephritis are common.
            of fibrosis. One environmental factor predisposing to fibrosis   Early work in English Cocker Spaniels also shows IgG4-
            is smoking, which may also be relevant in dogs that can be   positive plasma cells in  the  pancreas  and  kidney  (Watson
            passive smokers, but no studies have yet investigated this.   et al., 2012). The disease in humans responds well to steroid
            Some cases are chronic  from the  outset, with  an  initial   therapy, including a reduction in insulin requirements in
            mononuclear infiltrate. Genetic causes are likely to be impor-  some diabetics. This is clearly differentiated from the pro-
            tant in dogs, which explains the high risk in certain breeds.   posed autoimmunity in young German Shepherd Dogs with
            No particular breed prevalence has been reported for cats   pancreatic acinar atrophy (PAA), which is acinar-centered
            with chronic pancreatitis; domestic shorthairs are most com-  and does not result in DM (see later). There are not yet any
            monly affected.                                      controlled trials evaluating the use of immunosuppressive
                                                                 drugs in English Cocker Spaniels with chronic pancreatitis,
                                                                 but there is now enough circumstantial evidence to justify
                                                                 their use in this particular breed, and the author has had
                                                                 anecdotal success with both prednisolone and cyclosporine
                                                                 therapy in affected dogs. The decision to start immunosup-
                                                                 pressive therapy is based on clinical severity including the
                                                                 presence of recurrent bouts of pancreatitis and/or renal
                                                                 involvement  with  marked  proteinuria  nonresponsive  to
                                                                 conventional therapies. As in humans, less severely affected
                                                                 cases can be treated symptomatically and with “watchful
                                                                 waiting” (Bledsoe et al., 2018). However, the clinician should
                                                                 note  that  many  cases  of  chronic  pancreatitis  in  dogs  are
                                                                 not autoimmune; most terriers in Britain, for example, have
                                                                 a different histopathologic and clinical picture  of disease
                                                                 that  does  not  appear  to  be  autoimmune.  Confirmation of
                                                                 autoimmune disease requires histologic confirmation, which
                                                                 is rarely available. In the absence of pancreatic histopathol-
                                                                 ogy, presence of autoimmune disease in other organs (for
                                                                 example, keratoconjunctivitis sicca and glomerulonephritis)
                                                                 is  highly  suggestive  and justifies  the  use of  immunosup-
            FIG 37.7                                             pressives. The use of immunosuppressives in dogs that are
            Eight-year-old neutered male English Cocker Spaniel with   not Cocker Spaniels with isolated chronic pancreatitis is not
            chronic pancreatitis.                                recommended.
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