It is due to oropharyngeal dysmotility, hindering the passage of the bolus from the mouth to the oropharynx.5 Patients may have skin involvement of the face and tongue, but these changes rarely affect oral4 intake. However, 20% of patients with SS have a associated Sjögren's syndrome.4 In this case, the reduction in saliva production is associated with oropharyngeal dysphagia due to the inability to properly lubricate the food bolus before being transferred into the esophagus. In addition, impairment of salivary secretion increases the risk of developing periodontal disease and accelerates tooth decay, making mastication difficult,6 so the evaluation of the oral cavity should be included routinely in the physical examination of such patients.3,4,7

Oropharyngeal dysphagia is characterized by occurring a few seconds after initiating the act of swallowing, and the feeling is manifested by retention of food in the throat and the need to swallow repeatedly.5 It is often accompanied by regurgitation of the bolus into the nasopharynx, which may expel food through the nose or larynx. Dysfunction of the constrictor muscles of the pharynx can cause dysarthria, nasal voice and pharyngonasal regurgitation.5,8 In more severe cases, the patient cannot swallow saliva and drools.5,8

The difficulty in swallowing arises once the bolus has passed through the pharynx and upper esophageal sphincter. This type of dysphagia may be due to a motor or sensitive mechanical disturbance.9

In SS, the overproduction of collagen leads to thickening and fibrosis. Muscles weaken and cause dysmotility, a mechanism that involves esophageal dysphagia.3,4 This alteration can range from a lack of coordination of esophageal motility to the existence of complete paralysis of the esophagus.1,3,4

Esophageal dysfunction in SS mainly occurs in the lower two-thirds of the esophagus, causing esophageal sphincter weakness and profound loss of peristaltic3 action. This dysfunction leads to reflux of stomach contents into the esophagus. Esophageal damage may manifest as a peptic ulcer or erosive esophagitis progressing to hemorrhage and ulceration.3 The ulcer may scar or progress to an esophageal stricture, fistula, achalasia and Barrett's esophagus with the risk of neoplasia.1,3,4

Dietary interventions not only depend on the degree of impairment but also must ensure adequate nutritional support, and are intended to modify the texture of foods, attaining adequate lubrication of food and a smooth consistency for each type of dysphagia, as well as reducing and/or preventing problems associated with itself.5,8,10–12 Dietary recommendations in cases of oropharyngeal dysphagia are shown in Table 1.5,8,11,12

Gastroesophageal reflux is defined as the regurgitation of gastric contents into the esophagus and duodenum mainly due to loss of lower esophageal sphincter pressure 13. Recommendations are based on dietary changes and avoiding foods that promote reflux as well as reflux-inducing positions (Table 2).13

Esophageal stricture is a narrowing of the esophagus causing difficulty for swallowing. It is characterized by the appearance of a feeling of difficulty passing food or retrosternal pain right after swallowing, chest pain during eating and regurgitation of undigested food.4,8 Most patients are able to pinpoint the zone where this occurs.8

In this case, liquids are easier to swallow than solids. Virtually all foods can be liquefied, although for some of them it may be necessary to add a liquid (e.g. milk for grain, broth for meat, vegetables or fish). Dietary recommendations are presented in Table 3.8,11,12

Sometimes, despite performing a dietary intervention with conventional diet components, one may need to resort to commercial preparations or enteral14 nutrition.

Gastroparesis is a gastric motility disorder characterized by delayed emptying of solids and/or fluids from the stomach without any mechanical obstruction.15 Gastroparesis can worsen gastroesophageal reflux.3

Vascular ectasia is a cause of chronic gastrointestinal bleeding in patients with SS, who may experience a frank hemorrhage or bleeding causing microcytic or ferropenic anemia micro.3

Although no controlled trials evaluating the efficacy of dietary modification in the treatment of gastroparesis exist, and the clinical benefits are modest, some dietary recommendations may be useful in patients with milder forms of the disease.15

Nutritional intervention is based on dietary measures to encourage gastric emptying such as correct mastication, eliminating alcohol because it slows gastric emptying, fractionating a diet characterized by low but frequent volumes of food (5–6 meals a day), which may be low-fat and low in fiber to prevent bezoar formation. The soft consistency food or liquid is better tolerated. Fluid intake during a meal may promote gastric emptying. Orthostatic position or simply walking after eating in addition to other measures may facilitate gastric emptying. Nutritional therapy should be tailored to the severity of the disease. When weight loss occurs, the patient should be evaluated for the use of oral supplementation with enteral diets (complete diets in liquid form which need not be masticated or require stomach emptying). In later stages, when the stomach cannot empty liquids, enteral nutrition via a jejunostomy tube may be required, but parenteral nutrition while should be restricted to patients with severe gastric dysmotility of the small intenstine.7,15

SS may affect both the small and large intestine, including the rectum and anus.

In the small intestine, dysmotility involves the immobilization of intraluminal contents, which promotes bacterial growth and consequent diarrhea.1,3,4 Pharmacological treatment with antacids also can support and/or aggravate bacterial2 overgrowth. All this contributes to impaired absorption of nutrients, causing malabsorption, which together with pseudoobstructions may lead to severe malnutrition.1–4,7

Complications including the colon are: luminal dilatation or pneumatosis, pseudo-obstruction, constipation, diarrhea, fecal incontinence and bleeding, often hidden by telangiectasia1 bleeding.3,4

In patients with severe manifestations of the small intestine and loss of response to antibiotics, one must consider artificial nutrition. An alternative may be nocturnal enteral nutrition, although in most cases it is more convenient and acceptable for the patient to undergo a percutaneous3 gastrostomy. The natural history of the disease may require parenteral3 nutrition.

• (1)

  Constipation: due to intestinal4 dysmotility. In the early stages there appears to be effective constipation3 that can be initially treated with laxatives and soluble dietary fiber, which can also help improve fecal incontinence. Avoidance of non-absorbable carbohydrates such as sorbitol or lactulose is recommended as they may worsen the symptoms of bloating and discomfort, also favoring bacterial overgrowth.

• (2)

  Diarrhea: dietary advice in this case has a limited effect, with the main objective being the reduction of clinical symptoms such as postprandial discomfort, bloating and loss of weight.4 The first step is to prescribe a diet low in fiber which may be easily digested and absorbed.

Restricting carbohydrates from the diet may reduce intestinal fermentation, as they are the main energy source of bacteria, and helps reduce symptoms.4 For this reason, the dietitian-nutritionist's diet should be tailored individually, based on progression, patient tolerance and habits.

Sometimes lactose intolerance appears, forcing its removal from the diet plan. Some studies have detected the anomalous presence of Lactobacillus or other probiotics in the small intestine, so their usefulness is questionable in these patients.4 In any case, patients with advanced stages of the disease may be candidates for artificial nutrition.