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Immunodeficiency Syndromes, Dog   531




            Immunodeficiency Syndromes, Dog
  VetBooks.ir                                  Clinical Presentation                                                  Diseases and   Disorders


            BASIC INFORMATION
                                               HISTORY, CHIEF COMPLAINT               able to synthesize IgM, but IgG concentra-
                                                                                      tions are significantly reduced, and IgA is
           Definition                          •  Recurrent  infections  seen  at  a  young  age   not detectable.
           Primary  immunodeficiency  syndrome:  an   that may or may not respond to appropriate   •  Complement deficiency: autosomal recessive
           inherited defect involving the humoral (B-cell)   therapy.               mode of inheritance; dogs that are homo-
           or cell-mediated (T-cell) immune system, a   •  Type of infection may vary with defect in   zygous for the trait have no detectable C3,
           combination of the two, or the phagocytic   the immune system.           which is required for opsonization of bacteria.
           system                                                                   Affected individuals suffer recurrent sepsis,
                                               PHYSICAL EXAM FINDINGS               bronchopneumonia, pyometra, or wound
           Epidemiology                        •  Humoral (B-cell) immunodeficiency disorder:   infections.
           SPECIES, AGE, SEX                    physical exam findings are remarkable only   •  Functional  phagocytic  immunodeficiency:
           •  Signs often first manifest about 8-12 weeks of   for signs associated with chronic/recurring   affected animals have an increased risk
             age, when the protective effects of maternal   infections; increased incidence of chronic   of systemic or superficial infections with
             antibody are lost.                 respiratory, skin, and intestinal infections  pyogenic microorganisms.
           •  No sex predisposition except for sex-linked   •  Cell-mediated  (T-cell)  immunodeficiency   ○   Weimaraner immunodeficiency syndrome:
             severe combined immunodeficiency  syn-  disorder: absence of palpable lymph nodes;   inherited, exact mechanism unknown but
             drome (X-SCID) in males            tonsils are not visible. Affected dogs typi-  there is neutrophil dysfunction at the site
           •  More syndromes are described for dogs versus   cally have signs of growth retardation and   of lesions, and there appears to be a failure
             cats.                              unthriftiness  compared  with  the  other   to produce IgA and IgG.
                                                puppies in the litter.              ○   CLAD:  deficiency  of  leukocyte  surface
           GENETICS, BREED PREDISPOSITION      •  Combined  (B-cell  and  T-cell)  immuno-  glycoprotein (beta 2  integrin)  associated
           •  Humoral (B-cell) immunodeficiency disorders  deficiency disorder: similar findings as for   with leukocyte adherence and egress into
             ○   Selective immunoglobulin A (IgA) defi-  humoral and cell-mediated  immunodefi-  affected tissues; failure to express the beta 2
               ciency: German shepherd dog, Shar-pei,   ciency disorder               integrin CD18
               and beagle                      •  Functional  phagocytic  immunodeficiency   ○   TNS:  retention  of  neutrophils  in  the
             ○   IgG  deficiency:  Cavalier  King  Charles   disorder: fever, generalized lymphadenopathy,   bone marrow leads to neutropenia and
               spaniel                          dermatitis,  pododermatitis,  gingivitis,  secondary infections
             ○   IgM-IgA-IgG  deficiency:  some  lines  of   osteomyelitis; striking absence of exudate
               miniature dachshunds             formation (CLAD), and poor wound healing   DIAGNOSIS
           •  Complement deficiency: Brittany spaniel
           •  Cell-mediated  (T-cell)  immunodeficiency   Etiology and Pathophysiology  Diagnostic Overview
             disorder                          •  Humoral (B-cell) immunodeficiency: decreased     Affected puppies present with various, often
             ○   Thymic hypoplasia: dwarf Weimaraner  concentrations or absence of certain immu-  nonspecific clinical signs and commonly with
           •  Combined (B-cell and T-cell) immunodefi-  noglobulins, with increased susceptibility to   recurrent infections. It is the recurrence of the
             ciency disorder                    bacterial infections              infections and the poor response to therapy
             ○   SCID: Jack Russell terrier, Frisian water   ○   IgA  deficiency:  mode  of  inheritance  is   that warrant further investigation of a primary
               dog                                unknown. Epidemiologic studies show   immunodeficiency syndrome. Multiple animals
             ○   X-SCID: basset hound, Cardigan Welsh   that puppies born to dams with IgA   in a litter may be affected.
               corgi                              deficiency are at increased risk for infec-
           •  Functional  phagocytic  immunodeficiency   tions related to mucosal surfaces such as   Differential Diagnosis
             disorder                             upper respiratory infections,  recurrent   •  Humoral immunodeficiency: varies, based
             ○   Canine leukocyte adhesion disorder   bronchopneumonia, pyoderma, otitis,   on presenting complaint
               (CLAD): Irish setter               demodicosis, and enteritis.       ○   Upper  respiratory  infection:  Bordetella
             ○   Weimaraner immunodeficiency syndrome:   •  Cell-mediated  (T-cell)  immunodeficiency:   bronchiseptica
               Weimaraner                       affected animals have low numbers of or   ○   Primary ciliary dyskinesia
           •  Cyclical neutropenia: border collies, collie   nonfunctional T cells. Findings include a   ○   Otitis
             (gray)                             small thymus and lack of lymph nodes,   ○   Stomatitis
           •  Trapped neutrophil syndrome (TNS): Border   tonsils, and Peyer’s patches on postmortem   ○   Staphylococcal dermatitis
             collies                            exam. Affected animals are at increased risk   ○   Atopic dermatitis
                                                for infections with intracellular bacterial,   •  Cell-mediated immunodeficiency
           CONTAGION AND ZOONOSIS               fungal, protozoal, and viral organisms.  ○   Fungal (cryptococcosis, aspergillosis,
           Most opportunistic infections that affect   •  Combined (B-cell and T-cell) immunodefi-  blastomycosis, dermatophytosis)
           immunocompromised individuals are over-  ciency                          ○   Protozoal (toxoplasmosis, giardiasis)
           growths or infections with organisms that   ○   SCID:  autosomal  recessive:  affected   ○   Viral infections can be seen after vaccina-
           otherwise are not pathogenic to immunocom-  individuals are unable to mount an appro-  tion with modified live virus vaccine.
           petent hosts. However, immunocompromised   priate antigen-specific immune response   ○   Intracellular bacteria (mycobacterial
           animals are more susceptible than normal   because of a lack of DNA protein kinase   infections)
           individuals to infection with organisms that are   (DNA-PK) activity; DNA-PK is required   •  Combined  immunodeficiency:  affected
           potentially zoonotic (e.g., dermatophytosis) or   for lymphocyte precursors to mature.  animals  are susceptible  to bacterial,  viral,
           contagious to other dogs (e.g., respiratory tract     ○   X-SCID:  sex-linked  mutation  in  the   fungal, and protozoal agents.
           pathogens).                            gamma chain of the interleukin 2 (IL-2)   •  Functional phagocytic immunodeficiency
                                                  receptor required for normal B-cell and   ○   Sepsis
                                                  T-cell function. Affected male puppies are   ○   Bacteremia

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