Who is most affected
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women more common than men (2.3:1)
Incidence/prevalence
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annual incidence
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0.9 to 12.9 per 100,000 worldwide
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6.5 per 100,000 in 2008 in USA
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prevalence 8.1 to 204 per 100,000
Likely risk factors
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family history of CD
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Down syndrome
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HLA haplotype DR3-DQ2
Possible risk factors
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cesarean section
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Turner syndrome
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Williams syndrome
Factors not associated with increased risk: in children
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breastfeeding duration, age, concurrent infection or gastroenteritis at time of gluten introduction
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pertussis, Haemophilus influenzae type b, and measles/mumps/rubella vaccines
Associated conditions
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Malignancy
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lymphoma
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small bowel and stomach cancer
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non-Hodgkin lymphoma
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Dermatologic
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dermatitis herpetiformis
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xerosis
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keratosis pilaris
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Immunologic
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IgA deficiency
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common variable immune deficiency
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Rheumatologic
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Sjogren syndrome
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rheumatoid arthritis
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Gastrointestinal
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esophagitis
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microscopic colitis
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irritable bowel syndrome
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Endocrine
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diabetes mellitus type 1
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thyroid disease in children
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Neurologic
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epilepsy
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Kidney disease
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IgA nephropathy
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Liver disease
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non-alcoholic fatty liver disease, PBCs, autoimmune cholangitis, elevated liver transaminases
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Causes
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autoimmune disorder with 2 primary components
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genetic predisposition (presence of HLA-DQ2 or HLA-DQ8 haplotypes)
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environmental trigger (ingestion of gluten-a storage protein found in wheat, barley, and rye)
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History
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Chief concern
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gastrointestinal symptoms may include chronic diarrhea, chronic constipation, abdominal pain, nausea vomiting, distended abdomen, and flatulence
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abdominal complaints most common clinical presentation among children
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Review of systems
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extraintestinal signs and symptoms may include
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failure to thrive, stunted growth, delayed puberty
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chronic anemia, osteopenia/osteoporosis, dental enamel defects, irritability, chronic fatigue, neuropathy, arthritis/arthralgia, amenorrhea, increased alanine aminotransferase (ALT) and aspartate aminotransferase (AST)
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Physical
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Skin
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dermatitis herpetiformis
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HEENT
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aphthous stomatitis
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teeth discolored or enamel defects
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Abdomen
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abdominal distention, bloating with pain or cramping
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Neuro
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peripheral neuropathy
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Making the diagnosis
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confirm diagnosis if both
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positive CD-specific serology
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histologic changes in small intestinal mucosa include
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partial to complete villous atrophy
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crypt lengthening with increase in lamina propria
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increase in intraepithelial lymphocytes
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may need gluten challenge testing (if patients already on gluten-free diet, which can lead to normal serology and histology)
Differential diagnosis
- other causes of villous atrophy include
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tropical sprue
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collagenous sprue
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autoimmune enteropathy
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AIDS enteropathy
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allergic enterocolitis (milk protein allergy, soy allergy, rice allergy)
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infectious gastroenteritis
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microscopic colitis
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lymphoma
This article originally appeared on Cancer Therapy Advisor