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Gastrointestinal and Hepatic Manifestations
Chapter
33
Gastrointestinal and Hepatic Manifestations David S. Hallegua and Swamy Venuturupalli
GASTROINTESTINAL INVOLVEMENT
Gastrointestinal (GI) manifestations are common in patients with systemic lupus erythematosus (SLE), and the prevalence of their various manifestations is listed in Box 33-1. Abdominal symptoms and signs may be the result of SLE, medications that are used to treat SLE, or intercurrent processes. Historically, William Osler was impressed with the frequency of GI crises in those with lupus and labeled lupus as the new disease that could mimic any other disease.1
Prevalence
GI complaints were the initial presentation in 10% of Dubois’ patients2; 25% to 40% had protracted symptoms. Haserick and colleagues3 divided the GI symptoms of SLE among 87 patients into three groups: none (63%), minor (29%), and major (8%). Subclinical involvement of the GI tract is also common; chronic mucosal infiltration with inflammatory cells was found in 96% of 26 autopsied children with SLE.4 Younger patients with lupus are more susceptible. Among 272 patients with SLE, the prevalence of GI manifestations ranged from 10% in children to none in patients over the age of 50 years.5 A review on GI manifestations in SLE published by Sultan and colleagues6 found anorexia to be the most common reported manifestation with a prevalence of 36% to 71% in published studies. A recent publication found the cumulative prevalence of GI symptoms in patients with lupus who are of Asian descent to be 3.8% to 18%.7 In one recent study, patients with lupus who required hospitalization had a higher prevalence of GI symptoms with 39 out of 177 patients being admitted for a GI complaint.8
Pharyngitis, Dysphagia, and Esophagitis
Recurrent sore throat is not an infrequent finding, especially in children9 (see Chapter 23 for discussions on mucous membrane lesions and other features of oral pathologic conditions). Dysphagia and heartburn occur in 1% to 7.3%2,9,10 and 11% to 50%9 of patients, respectively. Chong and colleagues11 found that patients with lupus in comparison with patients with rheumatoid arthritis (RA) had significantly more heartburn. In a literature review, Zizic12 related that although only 5% of patients with SLE complained of dysphagia, 25% had impaired esophageal peristalsis, compared with 67% of patients with scleroderma. Several studies using esophageal manometry noted aperistalsis or hypoperistalsis of the esophagus in approximately 10% of patients with SLE.13-15 Aperistalsis is sometimes correlated with the presence of Raynaud phenomenon. Gutierrez and colleagues16 compared esophageal motility in 14 patients with SLE and 17 patients with mixed connective-tissue disease (MCTD). A definite correlation was found between Raynaud phenomenon and hypoperistalsis, with the latter being more common in MCTD. The patients in the SLE group had only a slightly decreased lower esophageal sphincter pressure. Esophageal motor dysfunction in SLE can also produce diffuse spasm and result in symptoms of chest pain. Ramirez-Mata and colleagues17 performed esophageal manometric studies in a group of unselected patients with SLE and noted abnormalities in 16 patients. An absence of or abnormally low
contractions were found at the upper one third in seven patients, at the lower two thirds in three patients, in the entire esophagus in two patients, at the lower esophageal sphincter in two patients, and at the lower two thirds plus the lower sphincter in the remaining two patients. No relationship was found among the presence of esophageal dysfunction and activity, duration, and therapy of SLE. Interestingly, five of the 34 patients who had normal studies complained of dysphagia and heartburn. Esophageal imaging with Gastrografin, computed tomographic (CT) scanning, or endoscopy is required to make the diagnosis of esophageal ulceration or perforation from systemic vasculitis.10 The treatment of esophageal symptoms include small and frequent meals, the avoidance of postprandial recumbency, and the admin istration of antacids, proton-pump inhibitors, histamine-2 (H2) antagonists, or parasympathomimetic agents. Fungal esophagitis from the use of antibiotics and steroids may need treatment with fluconazole.
Anorexia, Nausea, Vomiting, and Diarrhea
The most common cause of anorexia, nausea, vomiting, and diarrhea in patients with SLE is related to the use of salicylates, nonsteroidal antiinflammatory drugs (NSAIDs), antimalarial drugs, corticosteroids, and cytotoxic agents. Anorexia, nausea, vomiting, and diarrhea symptoms can continue to occur for weeks after therapy is stopped. When caused by the disease, manifestations are persistent and are not explained by other factors. Anorexia occurs in 49% to 82% of patients,2,9 especially if untreated. Nausea has been reported in 11% to 38% of patients.2,9 When medications are excluded as a cause, however, the incidence is approximately 8%.18 Vomiting and diarrhea can be prominent in patients who are hospitalized with lupus and GI symptoms2,9,18 and are observed in up to 56.4% and 30.8% of patients, respectively, who are admitted. Children appear to have an increased incidence of all these symptoms.5
Motility Disorders
Chronic intestinal pseudoobstruction (CIPO) reflects a dysfunction of the visceral smooth muscle or the enteric nervous system.19,20 Symptoms and signs of CIPO in patients with SLE include a subacute onset of abdominal pain and distention associated with vomiting and constipation and a distended tender abdomen with hypoactive or absent bowel sounds or a complete lack of bowel sounds. Radiologic examination reveals dilated, fluid-filled bowel loops and occasional bilateral ureteral dilation with a reduced bladder capacity. Antroduodenal manometry demonstrates intestinal and esophageal hypomotility. Nojima and colleagues20 described two patients with CIPO who had antibodies to proliferating cell nuclear antigen (PCNA) but no other specific antibodies or clinical manifestations of SLE. Treatment of CIPO usually involves high doses of steroids, broad-spectrum antibiotics, and promotility drugs. Perlemuter and colleagues21 reported the use of octreotide at a dose of 50 μg twice a day sub cutaneously in CIPO in SLE and scleroderma. The symptoms of CIPO resolved in the three patients receiving treatment within 415
33
Gastrointestinal and Hepatic Manifestations David S. Hallegua and Swamy Venuturupalli
GASTROINTESTINAL INVOLVEMENT
Gastrointestinal (GI) manifestations are common in patients with systemic lupus erythematosus (SLE), and the prevalence of their various manifestations is listed in Box 33-1. Abdominal symptoms and signs may be the result of SLE, medications that are used to treat SLE, or intercurrent processes. Historically, William Osler was impressed with the frequency of GI crises in those with lupus and labeled lupus as the new disease that could mimic any other disease.1
Prevalence
GI complaints were the initial presentation in 10% of Dubois’ patients2; 25% to 40% had protracted symptoms. Haserick and colleagues3 divided the GI symptoms of SLE among 87 patients into three groups: none (63%), minor (29%), and major (8%). Subclinical involvement of the GI tract is also common; chronic mucosal infiltration with inflammatory cells was found in 96% of 26 autopsied children with SLE.4 Younger patients with lupus are more susceptible. Among 272 patients with SLE, the prevalence of GI manifestations ranged from 10% in children to none in patients over the age of 50 years.5 A review on GI manifestations in SLE published by Sultan and colleagues6 found anorexia to be the most common reported manifestation with a prevalence of 36% to 71% in published studies. A recent publication found the cumulative prevalence of GI symptoms in patients with lupus who are of Asian descent to be 3.8% to 18%.7 In one recent study, patients with lupus who required hospitalization had a higher prevalence of GI symptoms with 39 out of 177 patients being admitted for a GI complaint.8
Pharyngitis, Dysphagia, and Esophagitis
Recurrent sore throat is not an infrequent finding, especially in children9 (see Chapter 23 for discussions on mucous membrane lesions and other features of oral pathologic conditions). Dysphagia and heartburn occur in 1% to 7.3%2,9,10 and 11% to 50%9 of patients, respectively. Chong and colleagues11 found that patients with lupus in comparison with patients with rheumatoid arthritis (RA) had significantly more heartburn. In a literature review, Zizic12 related that although only 5% of patients with SLE complained of dysphagia, 25% had impaired esophageal peristalsis, compared with 67% of patients with scleroderma. Several studies using esophageal manometry noted aperistalsis or hypoperistalsis of the esophagus in approximately 10% of patients with SLE.13-15 Aperistalsis is sometimes correlated with the presence of Raynaud phenomenon. Gutierrez and colleagues16 compared esophageal motility in 14 patients with SLE and 17 patients with mixed connective-tissue disease (MCTD). A definite correlation was found between Raynaud phenomenon and hypoperistalsis, with the latter being more common in MCTD. The patients in the SLE group had only a slightly decreased lower esophageal sphincter pressure. Esophageal motor dysfunction in SLE can also produce diffuse spasm and result in symptoms of chest pain. Ramirez-Mata and colleagues17 performed esophageal manometric studies in a group of unselected patients with SLE and noted abnormalities in 16 patients. An absence of or abnormally low
contractions were found at the upper one third in seven patients, at the lower two thirds in three patients, in the entire esophagus in two patients, at the lower esophageal sphincter in two patients, and at the lower two thirds plus the lower sphincter in the remaining two patients. No relationship was found among the presence of esophageal dysfunction and activity, duration, and therapy of SLE. Interestingly, five of the 34 patients who had normal studies complained of dysphagia and heartburn. Esophageal imaging with Gastrografin, computed tomographic (CT) scanning, or endoscopy is required to make the diagnosis of esophageal ulceration or perforation from systemic vasculitis.10 The treatment of esophageal symptoms include small and frequent meals, the avoidance of postprandial recumbency, and the admin istration of antacids, proton-pump inhibitors, histamine-2 (H2) antagonists, or parasympathomimetic agents. Fungal esophagitis from the use of antibiotics and steroids may need treatment with fluconazole.
Anorexia, Nausea, Vomiting, and Diarrhea
The most common cause of anorexia, nausea, vomiting, and diarrhea in patients with SLE is related to the use of salicylates, nonsteroidal antiinflammatory drugs (NSAIDs), antimalarial drugs, corticosteroids, and cytotoxic agents. Anorexia, nausea, vomiting, and diarrhea symptoms can continue to occur for weeks after therapy is stopped. When caused by the disease, manifestations are persistent and are not explained by other factors. Anorexia occurs in 49% to 82% of patients,2,9 especially if untreated. Nausea has been reported in 11% to 38% of patients.2,9 When medications are excluded as a cause, however, the incidence is approximately 8%.18 Vomiting and diarrhea can be prominent in patients who are hospitalized with lupus and GI symptoms2,9,18 and are observed in up to 56.4% and 30.8% of patients, respectively, who are admitted. Children appear to have an increased incidence of all these symptoms.5
Motility Disorders
Chronic intestinal pseudoobstruction (CIPO) reflects a dysfunction of the visceral smooth muscle or the enteric nervous system.19,20 Symptoms and signs of CIPO in patients with SLE include a subacute onset of abdominal pain and distention associated with vomiting and constipation and a distended tender abdomen with hypoactive or absent bowel sounds or a complete lack of bowel sounds. Radiologic examination reveals dilated, fluid-filled bowel loops and occasional bilateral ureteral dilation with a reduced bladder capacity. Antroduodenal manometry demonstrates intestinal and esophageal hypomotility. Nojima and colleagues20 described two patients with CIPO who had antibodies to proliferating cell nuclear antigen (PCNA) but no other specific antibodies or clinical manifestations of SLE. Treatment of CIPO usually involves high doses of steroids, broad-spectrum antibiotics, and promotility drugs. Perlemuter and colleagues21 reported the use of octreotide at a dose of 50 μg twice a day sub cutaneously in CIPO in SLE and scleroderma. The symptoms of CIPO resolved in the three patients receiving treatment within 415