Page 1814 - Cote clinical veterinary advisor dogs and cats 4th

908.e2 Shar-pei Fever

Shar-pei Fever Client Education
Sheet
VetBooks.ir Etiology and Pathophysiology

BASIC INFORMATION
○ With renal insufficiency, isosthenuria to
Shar-pei dogs have hereditary cutaneous • Urinalysis
Definition mucinosis. minimally concentrated urine
A familial, systemic, autoinflammatory disorder • Excessive hyaluronan (HA) accumulation ○ Proteinuria in 25%-43% of affected
characterized by recurrent fever, swelling of the is caused by overexpression of hyaluronan dogs. If dipstick + protein, quantify by
tibiotarsal joints, and renal amyloidosis synthase 2 (HAS2) in dermal fibroblasts, urine protein/creatinine ratio. Pathologic
which gives the skin its wrinkled appearance. proteinuria is the hallmark of glomerular
Synonyms • Degradation of hyaluronan may activate involvement, when present.
Familial amyloidosis of Chinese Shar-pei dogs, the innate immune system, leading to ○ If proteinuria confirmed, culture and
familial Shar-pei fever, swollen hock syndrome elevated levels of interleukin-1beta and sensitivity (C&S) tests are indicated to
(lay term); resembles familial Mediterranean interleukin-6. rule out urinary tract infection.
fever of humans • Elevated levels of cytokines are thought to • Blood pressure (BP) measurement to rule
lead to chronic overproduction of acute-phase out systemic hypertension in cases with renal
Epidemiology proteins (APPs), including serum amyloid involvement
SPECIES, AGE, SEX A, the precursor of amyloid A. • Coagulation assessment: if proteinuria con-
Young adults, 1-5 years old • Amyloid is then deposited in many organs, firmed, consider antithrombin measurement
and deposition in the kidneys usually leads or thromboelastography for hypercoagulable
GENETICS, BREED PREDISPOSITION to renal failure and death. state
Shar-pei dogs: autosomal recessive disorder ○ Unlike in other dogs with primarily • Other tests to consider, depending on signs
glomerular involvement, amyloid deposits and geographic location, for identifying
ASSOCIATED DISORDERS in the Shar-pei are usually in the renal inciting (and treatable) causes of proteinuria
Vasculitis may occur concurrently. medulla, potentially progressing to involve and/or polyarthritis: Borrelia, Ehrlichia,
the cortex, and azotemic renal failure Anaplasma, Leptospira, and fungal titers;
Clinical Presentation occurs at a young age (median, 4.8 years). heartworm antigen test; and survey radio-
DISEASE FORMS/SUBTYPES ○ With relative cortical sparing, the graphs to screen for evidence of metastatic
• Early stage: episodes of fever, tibiotarsal nephrotic syndrome is a rare complication. or fungal disease.
(hock) swelling, muzzle swelling, abdominal
pain, anorexia, and mild vomiting or diarrhea DIAGNOSIS Advanced or Confirmatory Testing
lasting 24-36 hours • Abdominal ultrasound exam to evaluate
• Late stage: signs of renal amyloidosis, hepatic Diagnostic Overview organs and screen for thrombi
amyloidosis, or amyloid deposition in other Shar-pei fever is a diagnosis of exclusion. • Renal biopsy: affected animals have moderate
organs The diagnosis is suspected in Shar-pei dogs to severe amyloid deposition in the renal
• Not all dogs experiencing fever develop presenting with a history of episodes of fever medulla; approximately 80% also have
renal or liver disease, and not all dogs with and lameness. The workup is directed toward glomerular deposition of amyloid. This
renal disease have a history of fever (e.g., detecting organ damage and ruling out other distribution helps increase diagnostic yield
only 44% of Shar-pei dogs with late-stage causes of fever, polyarthritis, azotemia, or liver because glomerular density is greatest in the
disease/renal failure due to amyloidosis have disease. cortex, and a renal biopsy is limited to the
a history of recognized signs of early-stage renal cortex.
disease). Differential Diagnosis • Consider a liver biopsy (46% of affected
• Polyarthritis (p. 803) Shar-pei dogs have hepatic amyloid infil-
HISTORY, CHIEF COMPLAINT • Lyme disease, ehrlichiosis, anaplasmosis tration), especially if there are clinical and
• Early stage: lethargy, inappetence, joint (pp. 285, 393, 596) biochemical signs of hepatic dysfunction and/
swelling (usually hock), lameness, swollen • Other causes of fever (pp. 334 and 1223) or contraindications to renal biopsy.
muzzle, abdominal pain, reluctance to move, • Other causes of renal and/or liver disease • Histopathology is the only way to document
mild vomiting or diarrhea the presence of amyloid (using Congo red
• Late stage: signs of renal (p. 169) and liver Initial Database stains).
(p. 442) failure; signs include vomiting, • CBC may show an inflammatory leukogram • A genetic assay is available through the
anorexia, lethargy, polyuria and polydipsia and (in the presence of chronic kidney Cornell AHDC and the Swedish University
(PU/PD), and weight loss. disease) nonregenerative anemia. of Agricultural Sciences.
• Serum biochemistry profile
PHYSICAL EXAM FINDINGS ○ With renal insufficiency: azotemia TREATMENT
• Early stage: fever (103°F-107°F [39.4°C- (blood urea nitrogen [BUN], creatinine
41.7°C]); palpable swelling of tibiotarsal elevation), hyperphosphatemia, acidosis; Treatment Overview
joints; swollen muzzle, abdominal pain symmetric dimethylarginine (SDMA) may The three phases of treatment are acute care,
may be present. detect renal involvement at an earlier stage prevention of deterioration, and supportive care
• Late stage than traditional measures of azotemia in advanced cases as follows:
○ Signs of dehydration, weight loss, and (p. 1381) • Supportive care using nonsteroidal antiin-
other manifestations of chronic kidney ○ With hepatic involvement: increased liver flammatory drugs (NSAIDs) during fever
disease (p. 169) enzymes ± hyperbilirubinemia and lameness episodes
○ Liver disease; icterus, hepatomegaly, signs ○ With protein-losing nephropathy ○ Antioxidants such as vitamin E, omega
of dehydration, weight loss (minority of dogs): hypoalbuminemia, fatty acids, and antiinflammatory drugs
○ Splenomegaly hypercholesterolemia, normal to increased such as methylsulfonylmethane, a
○ Pale mucous membranes globulin metabolite of dimethylsulfoxide (DMSO),

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