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Canine Autoimmune Polyglandular Syndromes
Deborah Greco, DVM, PhD, DACVIM (SAIM)
Desert Veterinary Specialists, Palm Desert, CA, USA
Many endocrine disorders, such as lymphocytic thyroid to a decrease in suppressor T cell activity. Approximately
itis, hypoadrenocorticism, and type 1 diabetes mellitus, 45% of all human patients with idiopathic (autoimmune)
have an autoimmune basis. When endocrine immune adrenal insufficiency will develop one or more additional
disorders occur in the same individual, they are known endocrinopathies (usually hypothyroidism).
as autoimmune polyglandular syndromes (APS). These The genetics of APS has been studied extensively in
syndromes (APS) can be further divided into APS type 1 human beings. APS 2 is inherited as an autosomal domi
(diabetes mellitus, ectodermal mucositis, etc.), APS type nant trait in humans associated with the presence of
2 (hypoadrenocorticism, hypothyroidism, etc.), and APS human leukocyte antigens (HLA). In families where this
type 3 (liver cirrhosis plus endocrinopathies). The com syndrome has been documented in several individuals,
bination of hypoadrenocorticism and hypothyroidism is the inheritance of major histocompatibility loci haplo
called Schmidt syndrome after the pathologist who type correlates most strongly with the disease. Many
described concurrence of these two diseases in human individuals with this haplotype have circulating organ‐
beings. Autoimmune polyglandular syndrome type 2 is specific autoantibodies directed against cytochrome
the only one that has been described in dogs. P450 without clinical signs of disease. Recently, one study
found that a higher proportion of dogs with hypoadreno
corticism were P450scc autoantibody positive when
Etiology and Pathophysiology compared to hospital controls (24% vs 1%). Lymphocytic
and plasmacytic destruction of the affected endocrine
Polyendocrine gland failure or autoimmune polyglan glands may be documented histologically.
dular syndrome, the most common form of which is APS Hypoadrenocorticism is the most common initial endo
type 2, has been well documented in humans. Autoimmune crinopathy observed in polyendocrine gland failure in
polyglandular syndrome type 2 is defined as the occur dogs and is usually followed by the development of hypo
rence of two or more of the following disorders in the thyroidism. However, type 1 diabetes combined with
same individual: adrenal insufficiency, primary hypothy immune‐mediated thyroid disease (Hashimoto thyroiditis)
roidism, type 1 diabetes mellitus (DM), primary hypog is also a common combination of immune endocrinopa
onadism (premature ovarian failure, immune‐mediated thies in humans and has been described in dogs. Studies
orchitis), myasthenia gravis, megaesophagus, immune‐ show that 45–50% of humans with adrenal insufficiency
mediated hemolytic anemia (IMHA), immune‐mediated associated with polyendocrine gland failure (onset 20–30
thrombocytopenia (ITP), hypoparathyroidism, hypopitu years) develop a second endocrinopathy. The most com
itarism, and celiac disease. mon second endocrinopathy, primary hypothyroidism,
Circulating organ‐specific autoantibodies are com develops 10–20 years after the first endocrinopathy.
monly present in APS type 2. Environmental factors In a retrospective of 225 cases of canine hypoad
combined with an HLA‐associated genetic predisposi renocorticism, 4% of the dogs (n = 9) also suffered from
tion are thought to trigger the process. Cell‐mediated hypothyroidism, two dogs had concurrent IDDM and
immune abnormalities in the type 2 syndrome include hypoadrenocorticism, and one had concurrent hypoad
defects and alterations of cell surface markers, but the renocorticism, hypothyroidism, IDDM, and hypopar
most consistent abnormality is a functional defect leading athyroidism. Another retrospective of 45 dogs with
Clinical Small Animal Internal Medicine Volume I, First Edition. Edited by David S. Bruyette.
© 2020 John Wiley & Sons, Inc. Published 2020 by John Wiley & Sons, Inc.
Companion website: www.wiley.com/go/bruyette/clinical