Version May 2024
INTRODUCTION — Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of non-necrotizing (“noncaseating on gross pathology”) granulomas. Clinically recognizable gastrointestinal (GI) system involvement occurs in less than 1 percent of patients with sarcoidosis, while symptomatic hepatic involvement occurs in 5 to 20 percent [1]. However, the incidence of subclinical involvement may be much higher. The stomach is regarded as the most commonly involved portion of the GI tract, but sarcoidosis has been described with equal frequency in the oral cavity and less frequently in the esophagus, small intestine, appendix, colon, rectum, pancreas, and peritoneum [1-6]. It has been suggested there is an oral-anal gradient of involvement with 80 percent of reported cases involving the esophagus, stomach and duodenum [7].
The clinical features, diagnosis, and treatment of GI, hepatic, pancreatic, and peritoneal sarcoidosis will be reviewed here. The pathogenesis, clinical manifestations, diagnosis, and treatment of sarcoidosis affecting other organ systems are discussed separately. (See "Pathology and pathogenesis of sarcoidosis" and "Clinical manifestations and diagnosis of sarcoidosis" and "Overview of extrapulmonary manifestations of sarcoidosis".)
APPROACH — Gastrointestinal (GI) involvement by sarcoidosis may occur in a patient with known sarcoidosis or may be the initial manifestation. Definitive diagnosis of sarcoidosis in either context can be difficult, as granulomas in the tubular structures of the GI tract and liver have a number of etiologies. Demonstration of tissue granulomas is necessary for a diagnosis of sarcoidosis, but histology by itself is not enough to secure a diagnosis of sarcoidosis. Establishing a definitive diagnosis of GI sarcoidosis depends on three components:
●Biopsy evidence of non-necrotizing granulomas in the "symptomatic or incident organ"
●Exclusion of other causes of granulomatous disease, particularly mycobacterial, fungal, and parasitic infections
●Clinical, radiographic, and optimally histopathologic evidence of sarcoidosis in at least one other organ system
When sarcoidosis is suspected but not previously diagnosed, an evaluation for evidence of extra-intestinal involvement should be under-taken. The absence of extra-intestinal involvement sarcoidosis does not exclude a diagnosis of sarcoidosis, but the presence of multi-organ involvement does increase the confidence of a definitive diagnosis of sarcoidosis [8]. (See "Clinical manifestations and diagnosis of sarcoidosis", section on 'Postdiagnostic assessment of disease severity and extent' and "Clinical manifestations and diagnosis of sarcoidosis", section on 'Diagnostic approach'.)
ORAL — Sarcoidosis involving the oral cavity presents as nodules, edema, ulcers, or gingival abnormalities (eg, gingivitis, gingival hyperplasia, gingival recession). The most commonly affected site is the buccal mucosa, followed by the gingiva, lips, floor of the mouth/sublingual glands, tongue, and palate. Cheilitis granulomatosa, a rare process in the differential diagnosis of granulomatous lip swelling, can be clinically and histologically indistinguishable from sarcoidosis except that it lacks multi-systemic involvement. The manifestations of oral sarcoid and cheilitis granulomatosa are discussed separately. (See "Cutaneous manifestations of sarcoidosis", section on 'Sarcoidosis of the oral cavity' and "Cheilitis", section on 'Cheilitis granulomatosa'.)
ESOPHAGEAL — More than 40 patients with esophageal involvement have been reported [7,9]. The lower esophagus (56 percent) was more commonly involved than the upper esophagus (26 percent) [10].
Symptoms arise from mucosal, muscular, or myenteric involvement of the esophagus or from extramural compression by enlarged mediastinal nodes [10]. The most common presenting symptom is dysphagia, reported in 21 of 23 cases (91 percent); but other manifestations, such as weight loss (22 percent), abdominal or chest pain (9 percent), hoarseness (9 percent), or odynophagia (4 percent), are also reported. Heartburn was not reported as a symptom associated with esophageal sarcoidosis in the 23 reported patients [10]. In a case report, an achalasia-like syndrome developed in a patient with known sarcoidosis and was attributed to involvement of the myenteric plexus [11].
The endoscopic appearance of esophageal sarcoidosis is nonspecific and includes discrete, gray, plaque-like lesions 3 to 10 mm in diameter; mucosal hyperemia; and nodularity [10]. A biopsy should be obtained at the time of endoscopy. As noted above, histopathology showing non-necrotizing granulomas and negative special stains for mycobacteria and fungi is required to establish the diagnosis, in addition to evidence of systemic sarcoidosis.
GASTRIC — Over 150 cases of symptomatic gastric sarcoidosis have been described [7]. While gastric involvement is the most common manifestation of sarcoidosis in the gastrointestinal (GI) tract, sarcoidosis is not the most common cause of granulomatous gastritis. Other causes include noninfectious etiologies (eg, Crohn disease, mucosa-associated lymphoid tissue lymphoma, chronic gastritis), infectious etiologies (Helicobacter pylori, tuberculosis, cryptococcosis, anisakiasis, schistosomiasis, syphilis), and idiopathic granulomatous gastritis. (See "Granulomatous gastritis".)
●Clinical manifestations – Gastric sarcoidosis is often a clinically silent finding: in an old series of 60 patients with sarcoidosis, 10 percent had asymptomatic lesions found incidentally on endoscopy [12]. When symptomatic, manifestations of gastric sarcoidosis are usually related either to the presence of peptic ulcerations or to narrowing of the gastric lumen due to granulomatous inflammation and associated fibrosis of the gastric wall; in the latter case, diminished peristalsis often results [13]. Epigastric pain is the predominant symptom in either circumstance and is dull, burning, or cramping in nature and often postprandial [14]. Nausea, vomiting, anorexia, heartburn, generalized abdominal discomfort, and diarrhea may also be reported, and symptom complexes may be influenced by concomitant intestinal or rectal involvement. Nausea and vomiting may occur in the presence of pyloric obstruction. Weight loss is common and can be severe, often raising a suspicion of malignancy [15,16]. Upper GI bleeding, although rare, can be the initial presentation and may occasionally be massive and fatal [17,18].
●Endoscopy – Upper GI endoscopy is typically performed in patients with sarcoidosis who have persistent epigastric symptoms or upper GI bleeding. Endoscopic findings in gastric sarcoidosis include nodular changes, erosive gastritis, thickened mucosa, greater or lesser curvature deformities, and benign or malignant-appearing ulcers [12]. Nodular mucosal irregularities are common, and mucosal polyps may occasionally be present [19]. As these findings are nonspecific, mucosal biopsies are essential to document the presence of non-necrotizing granulomas and to exclude H. pylori and other infections. (See "Granulomatous gastritis", section on 'Histopathologic features'.)
●Imaging – Typically, upper endoscopy is performed in preference to imaging studies [20]. The most common abnormalities on upper GI series are segmental mucosal thickening and nondistensibility that mimics the linitis plastica variety of gastric cancer. Deformity of the antrum may also be seen [21]. (See "Clinical features, diagnosis, and staging of gastric cancer" and "Clinical features, diagnosis, and staging of gastric cancer", section on 'Barium studies'.)
●Diagnosis – The diagnosis of gastric sarcoidosis rests upon histologic evidence of non-necrotizing granulomas in the biopsy specimens, exclusion of other causes of granulomatous inflammation, and clinical and pathologic evidence of sarcoidosis affecting other organ systems. Gastric biopsy specimens should be interpreted with care because Crohn disease, Whipple disease, tuberculosis, fungal infections, syphilis, and foreign body reactions may yield similar histopathology. The diagnosis of gastric sarcoidosis is difficult to establish in the absence of multisystem involvement. (See "Granulomatous gastritis", section on 'Diagnosis'.)
SMALL INTESTINAL — Very few cases of small intestine sarcoidosis have been reported and post-mortem involvement in one series of 320 autopsies was only 0.03 percent [22]. Duodenal involvement is reported with greater frequency than other portions of the small intestine as tissue biopsy of other regions is performed less often [2,20,23-25]. Most cases have occurred in the fifth or sixth decade of life, and evidence of multisystem sarcoidosis is present in approximately one-half of patients.
●Clinical manifestations – The clinical manifestations of small intestine sarcoidosis are non-specific and can mimic Crohn disease. Nonbloody diarrhea generally is the most common symptom, with colicky, epigastric and/or periumbilical abdominal pain occurring in approximately one-half of cases. Weight loss, anorexia, low-grade fever, and weakness may be present. Rare manifestations of small intestine sarcoidosis are protein-losing enteropathy [26,27], mesenteric venous insufficiency caused by enlarged sarcoid lymphadenopathy [28], and megaloblastic anemia caused by granulomatous infiltration of the terminal ileum [29].
●Evaluation – The order of testing is usually determined by the presenting symptoms and the presence (or absence) of a known diagnosis of sarcoidosis. Endoscopy is the most appropriate initial test for patients presenting with predominant diarrhea, while imaging studies (eg, abdominal computed tomography [CT]) may be a more appropriate initial step for those presenting with abdominal pain.
Endoscopic (either esophagogastroduodenoscopy or enteroscopy, depending upon the location of the lesion) findings include nodular submucosal lesions. Biopsy of any mucosal lesions should be obtained and sent for histopathology and special stains for mycobacterial and fungi.
Abdominal CT may show intra-abdominal adenopathy or hypodense liver lesions that suggest the presence of sarcoidosis outside the small bowel. Barium studies, D-xylose absorption, fecal fat excretion, and Schilling tests are rarely obtained.
●Diagnosis – The differential diagnosis of granulomatous enteritis includes sarcoidosis, Crohn disease, Whipple disease, tuberculosis, leprosy, and Histoplasma capsulatum enteritis. The diagnosis of small bowel sarcoidosis rests upon histologic evidence of non-necrotizing granulomas in the biopsy specimens, negative mycobacterial and fungal stains, and clinical and histopathologic evidence of sarcoidosis affecting other organ systems [23]. As part of the evaluation for possible histoplasmosis, a test for urinary histoplasma antigen is obtained when the patient has lived in endemic areas.
COLONIC AND APPENDICEAL — Sarcoidosis rarely involves the colon and rectum and usually does not produce symptoms. In the experience of the authors, granulomas are occasionally noted incidentally in colonic polyps resected at the time of colonoscopy in patients with systemic sarcoidosis [2]. Approximately 30 definite cases have been reported in the literature [2,7,23,30]. Abdominal pain is the most common symptom followed by diarrhea and weight loss [7,23]; stool occult blood was present in only one patient [31]. Reported findings on colonoscopy include multiple nodules, polyps, stenosis, obstructive lesions, aphthous erosions, and small punctuate bleeding sites [23].
Appendiceal sarcoidosis occurs rarely. As examples, one pathologic series of 71,000 surgically removed appendices found only one case consistent with sarcoidosis [32,33], and fewer than 10 cases of appendiceal sarcoidosis have been reported in the English language literature. All patients have been less than 30 years of age, and most were men. Two patients had no evidence of systemic sarcoidosis, and their disease would be classified as a "local sarcoid reaction" [2,34].
Patients with appendiceal sarcoidosis may present with acute right lower quadrant abdominal pain or subacute to chronic pain in association with a right lower quadrant mass. An appendiceal abscess is unusual. Histologic examination of the appendix reveals inflammatory changes and non-necrotizing granulomas [35]. The differential diagnosis of granulomatous involvement of the appendix includes bacterial (Yersinia, actinomycosis), mycobacterial, or parasitic (schistosomiasis, Enterobius) infection, Crohn disease, and appendicitis with a delayed appendectomy after initial medical management (interval appendectomy). Granulomas associated with parasitic infection typically have associated eosinophilic inflammation [36]. Negative mycobacterial and fungal stains and documented sarcoid involvement of another organ are essential to confirming the diagnosis [23].
Coexistence with other gastrointestinal immune disorders — Sarcoidosis is occasionally reported in association with either Crohn disease or ulcerative colitis. Furthermore, sarcoidosis has been reported in rare patients being treated with infliximab, adalimumab, or natalizumab for Crohn disease [37-39] or infliximab for ulcerative colitis [40].
Crohn disease — A shared pathogenic mechanism for sarcoidosis and Crohn disease was first proposed in the early 1970s based on the observation that the Kveim test was positive in 48 percent of patients with Crohn disease (see "Clinical manifestations and diagnosis of sarcoidosis", section on 'Proposed activity tests, including angiotensin converting enzyme (ACE) level') [41]. Bronchoalveolar lavage fluid lymphocytosis with elevated CD4/CD8 lymphocyte ratios [42-45] and non-necrotizing granulomas in the lungs can be seen in both disorders suggesting the possibility of a common pathogenic mechanism between Crohn disease and sarcoidosis. Reports of familial occurrences of sarcoidosis and Crohn disease [46-48] and genome wide association studies [49] also suggest the two diseases do share susceptibility loci. However, more definitive evidence in support of a causal relationship is lacking.
Overall, the simultaneous occurrence of Crohn disease and sarcoidosis is quite rare [50] and for practical purposes the clinical presentations of the two diseases are easy to differentiate. As an example, Crohn disease of the upper gastrointestinal tract is uncommon in the absence of disease beyond the ligament of Treitz (at division of duodenum and jejunum): one-third of patients with proximal Crohn disease may not have distal disease at diagnosis, but distal involvement develops with time in virtually all cases based on a series of 72 patients [51]. The occurrence of pyoderma gangrenosum, rectal and perianal lesions and enterocutaneous fistulas would favor Crohn disease over intestinal sarcoidosis. Detection of granulomas outside the gastrointestinal tract would favor sarcoidosis. It can be difficult to separate the two diseases based on detection of granulomas on biopsy, but intestinal sarcoidosis usually involves only the mucosa, whereas the inflammation in Crohn disease can be transmural. Crypt inflammation, aphthae, and ulcers would also favor a diagnosis of Crohn disease [24]. (See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults" and "Pathology and pathogenesis of sarcoidosis" and "Pulmonary complications of inflammatory bowel disease", section on 'Sarcoidosis'.)
Ulcerative colitis — The concurrence of sarcoidosis and ulcerative colitis has been reported, but is rare [52]. The onset of either condition bears no clear relationship to the activity or the extent of the other, suggesting that the two diseases are independent. (See "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults".)
Other — As many as 40 percent of patients with sarcoidosis have serological evidence of gastric autoimmunity and gluten-associated immune reactivity; however, the incidence of pernicious anemia and celiac disease in patients with sarcoidosis remains low [53,54]. (See "Diagnosis of celiac disease in adults" and "Causes and pathophysiology of vitamin B12 and folate deficiencies", section on 'Pernicious anemia'.)
PANCREATIC — Clinically apparent pancreatic sarcoidosis is rare [23,55-57]. One autopsy series of 92 patients with sarcoidosis documented incidental pancreatic involvement in five [4]. A different report reviewed nine major series and found pancreatic granulomas in three (1 percent) of 287 postmortem examinations [3]. A literature review in 2006 demonstrated 25 cases of surgically-proven pancreatic sarcoidosis with 12 presenting as a pancreatic head mass but only four cases were symptomatic [58]. Sarcoidosis may manifest as hypercalcemic pancreatitis [59].
An older literature review in 1996 found only 13 patients with biopsy-proven granulomas in the pancreas or peripancreatic nodes [55]. They added six of their own cases, one involving the body of the pancreas and five infiltrating the peripancreatic nodes. A clinically helpful profile of pancreatic sarcoidosis emerged from their limited review of 18 patients:
●Clinically apparent pancreatic sarcoidosis appears to be more common in women
●Bilateral hilar adenopathy was present on chest radiograph in three-fourths
●Two-thirds of patients have acute abdominal pain, but other typical findings of acute pancreatitis are uncommon
Laboratory, radiographic, and nuclear imaging features are not definitive. Serum amylase and lipase elevation may or may not be present [60,61]. Computed tomography (CT) and ultrasound of the abdomen are useful in demonstrating a pancreatic "mass," which is in the head of the pancreas in about half the cases [23,57]. A diffusely nodular pancreas is noted in the other half. However, imaging tests are unable to distinguish the granulomatous involvement from focal pancreatic inflammation or a carcinomatous mass. Thus, a CT-guided, ultrasound-guided, or laparoscopic biopsy is needed to identify non-necrotizing granulomas and exclude cancer and mycobacterial or fungal infection [23,60].
The prognosis of pancreatic sarcoidosis is good; 80 percent of patients improve either spontaneously or with glucocorticoids [55-57].
HEPATIC — The liver is involved in the majority of patients with sarcoidosis, although the clinical consequences of the involvement are variable [23,62-64]. Approximately, 50 to 65 percent of patients with sarcoidosis have granulomas on liver biopsy, but symptomatic hepatic sarcoid occurs in 5 to 15 percent [63,65,66]. Hepatic sarcoidosis is approximately twice as common in Black individuals as White individuals [64,65,67].
Most patients with hepatic sarcoid are asymptomatic and have only biochemical abnormalities (usually an elevated alkaline phosphatase and gamma glutamyl transpeptidase). Abdominal pain and pruritus are noted in approximately 15 percent of affected patients, and hepatomegaly in 5 to 15 percent [65,67]. Fever, weight loss, and jaundice are present in less than 5 percent [67]. Rare patients develop cirrhosis (6 percent), cholestatic liver disease with diffuse intrahepatic biliary strictures resembling sclerosing cholangitis, obstructive jaundice due to hepatic hilar lymphadenopathy, or hepatic vein thrombosis [13,65,67-76]. Portal hypertension, estimated to occur in 3 percent of patients with hepatic sarcoidosis, may develop as a result of biliary fibrosis or cirrhosis, or rarely due to periportal granulomas that restrict portal flow [62,65,67].
Among patients with clinical evidence of hepatic sarcoidosis, serum aminotransferases are increased in 50 to 70 percent, although the degree of increase is less than the serum alkaline phosphatase [65,67]. Hyperbilirubinemia and hypoalbuminemia are rare and associated with cirrhosis. The serum angiotensin converting enzyme level may be elevated, but is normal in approximately 25 percent of untreated patients, and is not useful diagnostically [77]. (See "Clinical manifestations and diagnosis of sarcoidosis", section on 'Proposed activity tests, including angiotensin converting enzyme (ACE) level'.)
Asymptomatic hepatic lesions may be first identified on the liver images of thoracic computed tomography (CT). On contrast-enhanced abdominal CT, the typical findings are hepatomegaly and numerous hypodense nodular lesions ranging in size from 1 mm to 3 cm (image 1) [78,79]. On magnetic resonance imaging, T1-weighted images show hypointense nodular lesions, while on T2-weighted images the nodules are hyperintense relative to the surrounding liver parenchyma [80]. Lesions tend to be located around the portal veins.
Liver biopsy is recommended for patients with moderate or severe liver-test abnormalities (eg, more than three times the upper limit of normal) [81]. Typically, the majority of biopsy specimens greater than 2 cm in length will have non-necrotizing granulomas that are often located along the portal tract. As conditions other than sarcoidosis can cause granulomatous lesions in the liver (eg, tuberculosis, fungal infections, brucellosis, Q fever, primary biliary cholangitis, Hodgkin disease, drug toxicity), additional steps are needed to make a confident diagnosis of sarcoidosis. These steps include identifying characteristic extrahepatic manifestations of sarcoidosis (eg, cutaneous manifestations and/or chest imaging abnormalities), characterization of the granulomas within the liver (location and appearance), examination of special stains/cultures for infectious organisms, and exclusion of malignancy and drug-induced granulomas. (See "Evaluation of the adult patient with hepatic granuloma", section on 'Causes'.)
PERITONEAL — Peritoneal involvement is an uncommon manifestation of sarcoidosis, especially in the absence of splenic, hepatic, adnexal, or small intestinal involvement [82]. It can be particularly difficult to separate peritoneal sarcoidosis clinically and radiographically from peritoneal carcinomatosis or peritoneal tuberculosis [83-85]. Commonly used cancer biomarkers, particularly CA-125, have been found to be elevated in peritoneal sarcoidosis [86]. Thus, cancer biomarker elevations cannot separate the diagnosis of peritoneal sarcoidosis from peritoneal carcinomatosis. Tissue sampling is recommended in this situation to document non-necrotizing granulomas and exclude malignancy and infection.
Symptomatic patients most commonly complain of abdominal pain. Ascitic fluid is typically identified by computed tomography or peritoneal studding and ascites are noted at the time of laparoscopy. The peritoneal fluid is usually a lymphocyte predominant, low serum-ascites albumin gradient fluid (ie, SAAG <1.1 g/dL) [87]. In contrast, when sarcoidosis causes ascites due to cor pulmonale or portal hypertension, the fluid has a high serum-ascites albumin gradient (ie, SAAG ≥1.1 g/dL). (See "Evaluation of adults with ascites", section on 'Serum-to-ascites albumin gradient'.)
TREATMENT — The decision to treat gastrointestinal (GI) sarcoidosis is based upon the activity and extent of disease. Asymptomatic patients generally do not require treatment.
For patients who are symptomatic and have a substantial amount of granulomatous inflammation on tissue biopsy, glucocorticoids are the treatment of choice, based on extrapolation from the treatment of pulmonary sarcoidosis [23].
●Immunosuppressive therapy ─ For most forms of GI sarcoidosis, when therapy is indicated, we initiate prednisone up to 0.5 mg/kg per day (eg, approximately 30 to 40 mg), as a single daily dose, and then gradually taper to a maintenance dose of 10 to 15 mg daily, guided by clinical response, over a period of approximately six months. The proper length of therapy for those who respond to treatment is not known. We usually aim for a duration of therapy of at least one year. (See "Treatment of pulmonary sarcoidosis: Initial approach", section on 'Stopping glucocorticoid therapy' and "Treatment of pulmonary sarcoidosis: Initial approach", section on 'Initial and maintenance dosing'.).
Steps to mitigate the potential adverse effects of glucocorticoid therapy are discussed separately. (See "Major adverse effects of systemic glucocorticoids".)
Alternative or additional agents may be required in patients who do not respond to glucocorticoids alone or cannot tolerate the side effects. (See "Treatment of pulmonary sarcoidosis refractory to initial therapy".)
●Esophageal sarcoid ─ In a case report, a cricopharyngeal myotomy was needed for severe dysphagia due to localized sarcoidosis [88]. (See 'Esophageal' above and "Oropharyngeal dysphagia: Clinical features, diagnosis, and management", section on 'Cricopharyngeal myotomy'.)
●Gastric sarcoid ─ Although evidence from clinical trials is lacking, antisecretory therapy or promotility agents may be useful in managing dyspepsia or symptoms related to delayed gastric emptying, respectively, particularly if the disease is minimally active. Surgery may be required in patients with pyloric stenosis or gastrointestinal hemorrhage. (See "Approach to the adult with dyspepsia" and "Treatment of gastroparesis".)
●Hepatic sarcoid ─ An optimal therapeutic regimen for hepatic sarcoidosis remains ill-defined. It has been proposed that patients with symptomatic hepatic sarcoidosis receive prednisone, following the above approach. This includes patients with diffuse intrahepatic biliary strictures due to sarcoid [81]. In a series of 63 patients with hepatic sarcoid treated with prednisone, approximately one-third had a complete clinical response, one-third a partial response, and one-third no response [89]. Even in patients with advanced disease (ie, portal hypertension or cirrhosis), our practice is to try an initial course of prednisone. (See "Treatment of pulmonary sarcoidosis refractory to initial therapy" and "Evaluation of the adult patient with hepatic granuloma", section on 'Sarcoidosis'.)
Of alternative agents used in hepatic sarcoidosis, methotrexate deserves special mention [90]. Despite the potential risk of hepatic toxicity, methotrexate has been shown to reduce abnormal liver function test abnormalities and to be glucocorticoid sparing in this setting [65,89,91]. (See "Treatment of pulmonary sarcoidosis refractory to initial therapy".)
Although hepatic involvement is common, end-stage liver disease and other hepatic complications were rare indications for orthotopic liver transplantation (OLT) in the United States from October 1987 to December 2007 and accounted for only 0.12 percent of OLT recipients [92]. The one and five-year survival rates of the 102 patients that were transplanted for hepatic sarcoidosis were 78 and 61 percent respectively [92]. Sarcoidosis can recur in the transplanted liver [92-95].
Interventions for other hepatic complications may include:
•Liver transplantation for the rare occurrence of hepatic vein thrombosis due to compression of the hepatic veins by sarcoid granulomas [68-70]. (See "Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis".)
•Treatment for portal hypertension (presumably presinusoidal due to the presence of periportal granulomas), which is similar to that for portal hypertension of other etiologies and includes liver transplantation [65,96,97]. (See "Portal hypertension in adults".)
•Ursodeoxycholic acid may be of benefit for patients with a syndrome of intrahepatic cholestatic jaundice with pruritus and hypercholesterolemia resembling primary biliary cholangitis [81,98,99].
MONITORING — Gastrointestinal sarcoidosis is monitored clinically and radiographically. Repeat endoscopy may be needed when the response to therapy remains unclear, particularly when intensification of therapy is being considered for persistent symptoms.
Hepatic sarcoidosis is monitored with repeated serum alkaline phosphatase and transaminase levels. The frequency of testing is not established and primarily dictated by clinical response. General guidelines for monitoring sarcoidosis are provided in the table (table 1).
The role of biomarkers, such as serum angiotensin converting enzyme levels and soluble interleukin-2 receptor (sIL-2R), for monitoring gastrointestinal sarcoidosis remains investigational [100,101].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Sarcoidosis".)
SUMMARY AND RECOMMENDATIONS
●Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of non-necrotizing (“noncaseating on gross pathology”) granulomas. The stomach and oral cavity are the most commonly involved portions of the gastrointestinal (GI) tract, but sarcoidosis of the esophagus, small intestine, appendix, colon, rectum, pancreas, and peritoneum has also been described. (See 'Introduction' above.)
●Gastric involvement with sarcoidosis is typically silent, but can manifest as peptic ulceration or narrowing of the gastric lumen due to granulomatous inflammation and associated fibrosis of the gastric wall. When symptomatic patients usually experience dull, burning, or cramping abdominal pain; nausea and vomiting occur when gastric outlet obstruction is present. (See 'Gastric' above.)
●The differential diagnosis of non-necrotizing granulomas involving the stomach, small bowel, or colon includes sarcoidosis, Whipple disease, Crohn disease, and infection with Mycobacterium tuberculosis, Histoplasmosis capsulatum, or Treponema pallidum. The differential diagnosis of granulomatous involvement of the appendix also includes bacterial infection (Yersinia, actinomycosis), parasitic infection (schistosomiasis, Enterobius), and appendicitis with delayed appendectomy after initial medical management (interval appendectomy). (See 'Esophageal' above and 'Gastric' above and 'Small intestinal' above and 'Colonic and appendiceal' above.)
●The diagnosis of sarcoidosis of the GI tract is based on the presence of non-necrotizing granulomas on biopsy from the affected portion of the GI tract, evidence of sarcoidosis involving extraabdominal organs, negative stains and cultures (when available) from gastrointestinal biopsy specimens, and exclusion of sarcoid-like reactions to malignancy or foreign bodies. (See 'Approach' above.)
●Sarcoidosis is rarely reported in association with either Crohn disease or ulcerative colitis. The concurrence with ulcerative colitis is rare enough to suggest that the two diseases are independent. (See 'Coexistence with other gastrointestinal immune disorders' above.)
●The liver is almost always involved in patients with gastrointestinal sarcoidosis, but may be affected in the absence of other gastrointestinal involvement. Symptomatic hepatic sarcoidosis is less common. The differential diagnosis of granulomatous hepatitis is broad, and the diagnosis and treatment of hepatic sarcoidosis are discussed separately. (See 'Hepatic' above and "Evaluation of the adult patient with hepatic granuloma", section on 'Sarcoidosis'.)
●Asymptomatic patients are typically monitored without initiation of active therapy. For symptomatic patients with substantial organ involvement, we typically initiate therapy with prednisone up to 0.5 mg/kg per day (eg, approximately 30 to 40 mg daily). This dose is continued for six to eight weeks or until a response to therapy is noted and then gradually tapered, over a period of approximately six months, to a maintenance dose of 10 to 15 mg daily for a further six months. (See 'Treatment' above.)
●The optimal duration of glucocorticoid treatment for gastrointestinal and hepatic sarcoidosis is not known, but our practice is to treat the initial manifestation for at least one year. (See 'Treatment' above.)
●Disease activity is monitored clinically and radiographically. The role of serum angiotensin converting enzyme or soluble interleukin-2 receptor levels in monitoring gastrointestinal sarcoidosis is not known, but appears to be limited and not recommended. Repeat endoscopy is performed when the response to therapy remains unclear, particularly when intensification of therapy is being considered for persistent symptoms. (See 'Monitoring' above.)
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