Celiac disease natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Natural History, Complications, and Prognosis

Natural History

The symptoms of celiac disease usually develop in the firs decade of life, and start with symptoms suggestive of malabsorption such as pain and distension, diarrhea, malnutrition, and failure to thrive within the first few years of life. These are classical celiac disease findings also known as “celiac disease iceberg” manifestations. Clinical presentations in celiac disease have been described as an iceberg model. Based on the iceberg model of celiac disease, the classic malabsorption manifestation of disease is just the tip of the iceberg, and other more specific manifestations are invisible below the waterline. According to iceberg model of celiac disease, the traditional method of diagnosing celiac disease based on malnutrition manifestations is unreliable due to different disease variation with more subtle manifestations that may be unrecognized.

Most people with either diagnosed or undiagnosed celiac disease have only minor symptoms that are more inconvenient than life threatening. The few data available regarding quality of life of patients with celiac disease suggest that health‐related quality of life and psychological general well‐being are poor in undiagnosed patients, and improve with treatment in men but not significantly in women. The frequency of psychiatric disorders are also high among celiac disease patients, with a questionnaire study reported a mean duration of depression and anxiety symptoms for 11 years before the diagnosis of celiac disease. The mentioned depression was reported to get improved in 77% of cases after treatment. A high prevalence of depression was well documented in patients with coeliac disease.

The adult patients with celiac disease are at higher risk of:

• Mortality from malignancy especially lymphomas in patients aged 45-65
• Suicide
• Diabetes
• Inflammatory bowel disease

There are different unknown variations of celiac disease which include:

• Asymptomatic
• Silent: Asymptomatic and no disease manifestation including evident malabsorption
• Potential: Positive celiac-specific serology with normal histology

Natural history of these celiac disease variations is not completely understood. Particularly, the long-term risk of complications in patients who are asymptomatic or silent is not available. Undiagnosed celiac disease has been shown to be associated with a nearly 4-fold increased risk of death compared with asymptomatic patients whom are negative for serologic evidence of celiac disease.

As the risk of malignancy in celiac disease exist, it is recommended to asymptomatic and silent patients should patients to comply with a gluten-free diet. The majority of patients with celiac disease respond to a gluten-free diet. The most common reasons for a lack of response are poor compliance or inadvertent gluten ingestion.

Complications

Disease		Manifestations
Vitamine deficiencies	Vitamin K deficiency	Easy bruising Mucosal bleeding Splinter hemorrhages Melena Hematuria
	Vitamin B12 deficiency	Unexplained anemia with macrocytosis Pancytopenia Hypersegmented neutrophils Unexplained neurologic or psychiatric symptoms Symmetric paresthesias or numbness and gait problems Subacute combined degeneration of the dorsal (posterior) and lateral columns (white matter) of the spinal cord due to demyelination
	Vitamin D deficiency	secondary hyperparathyroidism: phosphaturia Demineralization of bones Osteomalacia in adults Rickets and osteomalacia in children Bone pain and tenderness Muscle weakness Fracture Difficulty walking
Micronutrient deficiencies	Calcium deficiency
	Iron deficiency	anemia with non-specific symptoms include: Fatigue Weakness and irritability Headache Veretigo Microcytic hypochromic anemia
	Folate deficiency	Unexplained anemia with macrocytosis Pancytopenia
Enamel hypoplasias		In greater than twenty percent of celiac disease cases in children Linear hypoplasia on the teeth
Dermatitis herpetiformis		cutaneous manifestations of gluten-sensitivity: Multiple intensely pruritic papules and vesicles that occur in grouped ("herpetiform") arrangements Mainly in The elbows, dorsal forearms, knees, scalp, back, and buttocks Oral manifestations of gluten-sensitivity: vesicles, erosions, or erythematous macules on the oral mucosa or tongue
Neurological abnormalities		All more common in children Include: Hypotonia Developmental delay Learning disorders ADHD Headache Cerebellar ataxia
gastrointestinal malignancies	Small bowel lymphoma	Fever Hepatosplenomegaly Duodenal mass Ascites
hyposplenism		Increase risk of sepsis and bacteremia due to encapsulated pathogens
Ulcerative jejunitis		Multiple chronic, benign-appearing ulcers, most frequently in the jejunum Recurrent symptoms of despite being on a gluten-free diet: Malabsorption Lassitude Anorexia Weight loss Fever
Infertility		The failure of a couple to conceive: In women under age 35 after 12 months of frequent intercourse without use of contraception In women over age 35, and after six months of frequent intercourse without use of contraception

Prognosis

• Depending on the extent of the celiac disease at the time of diagnosis and also extent of complications and diet complication, the prognosis may vary. However, the prognosis is generally regarded as good.
• People with celiac disease are at higher risk of mortality than the general population. Although people with celiac disease had an increased risk of gastrointestinal and lymphoproliferative malignancies compared with the general population, it has been shown that they are in a lower risk of breast or lung cancer.
• The presence of gastrointestinal malignancies is associated with a particularly poor prognosis among patients with celiac disease.