Gastric sarcoidosis: a case report and review of the literature.Abstract: Sarcoidosis Sarcoidosis Definition
Sarcoidosis is a disease which can affect many organs within the body. It causes the development of granulomas. Granulomas are masses resembling little tumors. They are made up of clumps of cells from the immune system. is a systemic granulomatous disease of unknown etiology that is characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) tract involvement in sarcoidosis is rare. Gastric sarcoidosis, particularly involving the antrum, affects approximately 10% of patients with systemic disease. (1) GI sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1 to 0.9% of patients with the disease.
This is a rare case report of an individual with symptomatic gastric sarcoidosis. The patient presented with weight loss, nausea, and early satiety satiety
being in a state of satiation; in experimental animals used with reference to eating and drinking.
located in the ventromedial hypothalamic nucleus. . An EGD Esophagogastroduodenoscopy (EGD)
An imaging test that involves visually examining the lining of the esophagus, stomach, and upper duodenum with a flexible fiberoptic endoscope.
Mentioned in: Bleeding Varices
esophagogastroduodenoscopy. and colonoscopy were performed and were grossly normal. However, biopsies of the gastric antrum revealed noncaseating granulomatous inflammation involving the gastric mucosa. Corticosteroid therapy was started and the symptoms abated almost immediately. We also offer a review of the literature.
Key Words: gastric sarcoidosis, corticosteroid therapy, noncaseating granulomas
Sarcoidosis is a systemic granulomatous disease of unknown etiology that is characterized by the formation of noncaseating granulomas. Although it affects multiple systems, the disease has a predilection for intrathoracic structures. Gastrointestinal (GI) tract involvement in sarcoidosis is rare. Gastric sarcoidosis, particularly involving the antrum, affects approximately 10% of patients with systemic disease. (1) GI tract sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1 to 0.9% of patients with the disease. This is a rare case report of an individual with symptomatic gastric sarcoidosis. Weight loss, nausea, and early satiety were reported and abated almost immediately with corticosteroid treatment.
A 35-year-old woman, with a past medical history significant for genital herpes, presented to the emergency room (ER) complaining of 2 to 3 years of intermittent, nonexertional, substernal chest pain. During the week before presentation, the chest pain worsened in severity with exertion and deep inspiration. The patient denied any rashes or ocular, respiratory and central nervous system symptoms. Vital signs on presentation revealed a heart rate of 127, a blood pressure of 117/68, an oxygen saturation of 98% on room air, a temperature of 98.8[degrees] and a respiratory rate of 12. Physical examination was unremarkable: there was no palpable adenopathy; breath sounds were clear to auscultation auscultation
Procedure for detecting certain defects or conditions by listening for normal and abnormal heart, breath, bowel, fetal, and other sounds in the body. The invention of the stethoscope in 1819 improved and expanded this practice, still very useful despite the bilaterally in all lung fields; there were no appreciable murmurs, rubs, or gallops; there was no organomegaly or tenderness on abdominal examination; there were no appreciable rashes on examination of the skin; and there were no sensory or motor deficits.
An EKG EKG: see electrocardiography. done on presentation showed sinus tachycardia. The values on the comprehensive metabolic panel comprehensive metabolic panel Lab medicine A battery of analytes–albumin, alk phos, AST, BUN, calcium, chloride, glucose, potassium, sodium, total protein–which are measured to establish a baseline and detect metabolic disorders. See Panel. and complete blood count were all within normal limits. Liver-associated enzymes were also within normal limits. Serum aminotransferases (AST (AST Computer, Irvine, CA) A PC manufacturer founded in 1980 by Albert Wong, Safi Quershey and Tom Yuen (A, S and T). It offered a complete line of PCs that sold through its dealer channel. and ALT) were 15 and 12, respectively, and the alkaline phosphatase was 68. A computed tomography (CT) scan of the chest was done in the ER and ruled out a pulmonary embolism. However, there was significant bilateral adenopathy in the hilum hilum /hi·lum/ (hi´lum) pl. hi´la [L.] a depression or pit on an organ, giving entrance and exit to vessels and nerves.hi´lar
n. pl. and porta hepatis suggestive of sarcoidosis. There were also multiple, small hypodense lesions in the spleen seen on the CT but no lesions evident in the parenchyma Parenchyma
A ground tissue of plants chiefly concerned with the manufacture and storage of food. The primary functions of plants, such as photosynthesis, assimilation, respiration, storage, secretion, and excretion—those associated with living of the lungs or the liver. Mediastinoscopy and lymph node biopsy Lymph Node Biopsy Definition
A lymph node biopsy is a procedure in which all or part of a lymph node is removed and examined to determine if there is cancer within the node. were performed and revealed noncaseating granulomas. There was no evidence of lymphoma on biopsy. Lymph node cultures grew out no organisms and had only rare neutrophils. Special stains for mycobacterium and fungi (AFB AFB
AFB Acid-fast bacillus, also 1. Aflatoxin B 2. Aorto-femoral bypass and GMS) were negative. The absence of abdominal pain, an abdominal mass, perianal perianal
around the anus.
under the skin outside the anal canal. Causes sufficient pain to inhibit defecation. disease, fistulas, or strictures excluded Crohn disease. A diagnosis of systemic sarcoidosis was made. Pulmonary function tests were done shortly after the ER visit and showed normal lung volume and flow, but a reduced diffusion capacity to 61% of the predicted value. An angiotensin converting enzyme Noun 1. angiotensin converting enzyme - proteolytic enzyme that converts angiotensin I into angiotensin II
angiotensin-converting enzyme, ACE
peptidase, protease, proteinase, proteolytic enzyme - any enzyme that catalyzes the splitting of proteins into level was also drawn and found to be 19. Steroid therapy was not offered at that time.
Six months after the ER visit, the patient presented to her primary care doctor complaining of generalized fatigue, early satiety, and an unintentional weight loss of 20 pounds. Physical examination was significant for a palpable spleen and liver edge and small axillary lymphadenopathy. Routine labs were drawn and revealed a microcytic anemia and an elevated alkaline phosphatase to 189. The total serum bilirubin was normal at 0.3. An HIV test was performed because of the constellation of symptoms and was nonreactive. To assess the elevated alkaline phosphatase, a CT scan of the chest, abdomen, and thorax was repeated and showed multiple low density liver and splenic splenic /splen·ic/ (splen´ik) pertaining to the spleen.
Of, in, near, or relating to the spleen.
pertaining to the spleen. lesions, as well as significant hepatosplenomegaly. Viral and autoimmune hepatitis panels were then checked and were negative. Referral was made to a gastroenterologist for investigation of the liver lesions, anemia, and early satiety.
An esophagogastroduodenoscopy (EGD) and colonoscopy were performed and were grossly normal. Biopsies of the gastric antrum revealed noncaseating granulomatous inflammation involving the gastric mucosa. There was no evidence of H pylori, acid-fast bacilli, or fungi. Liver biopsy of the hypodense lesions also revealed noncaseating granulomas. A capsule endoscopy was performed to complete the anemia workup work·up
n. Abbr. w/u
A thorough medical examination for diagnostic purposes. . It was normal, and the anemia was thought to be secondary to the chronic sarcoidosis, as small bowel sarcoidosis can cause protein-losing enteropathy and malabsorption. (2) A gastric emptying scan Gastric Emptying Scan Definition
A gastric emptying scan (GES) is an x-ray exam using special radioactive material that allows physicians to identify abnormalities related to emptying of the stomach. was abnormal, with a prolonged lag phase before the initiation of gastric emptying. The overall half-time of gastric emptying was borderline high at 85.5 minutes.
The patient was started on prednisone prednisone (prĕd`nĭsōn): see corticosteroid drug. 20 mg twice a day and noted weight gain and improvement in her energy level. However, as the steroids were slowly tapered off, the patient started to have recurrence of her GI symptoms. She was again having early satiety and weight loss and also reported nausea and vomiting Nausea and Vomiting Definition
Nausea is the sensation of being about to vomit. Vomiting, or emesis, is the expelling of undigested food through the mouth. . A repeat EGD was performed. The entire gastric mucosa, as well as the mucosa of the lower one third of her esophagus, was erythematous. Biopsies of the antrum again showed noncaseating granuloma granuloma /gran·u·lo·ma/ (gran?u-lo´mah) pl. granulomas, granulo´mata an imprecise term for (1) any small nodular delimited aggregation of mononuclear inflammatory cells, or (2) such a collection of modified macrophages , and biopsies of the fundus fundus /fun·dus/ (fun´dus) pl. fun´di [L.] the bottom or base of anything; the bottom or base of an organ, or the part of a hollow organ farthest from its mouth. showed chronic gastritis with multinucleated multinucleated
characterized by having more than one nucleus per cell.
multinucleated giant cell
see giant cell. giant cells. Biopsies of the esophagus showed squamous mucosa with chronic inflammation consistent with reflux esophagitis. Prednisone 20 mg daily was restarted, and the patient responded immediately with resolution of nausea, vomiting and early satiety. Pantoprazole 40 mg daily was also started to control the patient's reflux disease.
Gastrointestinal tract sarcoidosis is rare and may present in the context of generalized disease or as an isolated finding. It may mimic other gastric processes, including peptic ulcer disease Peptic ulcer disease (PUD)
A stomach disorder marked by corrosion of the stomach lining due to the acid in the digestive juices.
Mentioned in: Indigestion
peptic ulcer disease See Duodenal ulcer, Gastric ulcer, GERD. , Menetrier disease, localized hypertrophic gastritis, Crohn disease, and carcinoma, both clinically and radiographically. (3) The diagnosis of gastric sarcoidosis is dependent on histologic evidence of sarcoid sarcoid /sar·coid/ (sahr´koid)
2. a sarcoma-like tumor.
Of or resembling flesh.
1. granulomas on mucosal biopsies. Proper interpretation of the biopsies is crucial because tuberculosis, syphilis, Crohn disease, foreign body reactions, and fungal infections can all present with granulomatous disease. (4) Of the approximately 60 cases of symptomatic gastric sarcoidosis in the literature, only 25 have well-documented histologic evidence of noncaseating granulomas consistent with sarcoidosis. (5)
GI sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1 to 0.9% of patients with the disease. (5) Based on previous case reports, there are two main presentations of gastric sarcoidosis. One is gastric ulcer formation, in which localized mucosa is infiltrated with sarcoid granulomas. The other involves extreme, diffuse infiltration with granulomas and results in a reduced lumen size secondary to fibrosis. (6) This latter type resembles linitis plas-tica. Manifestations of the disease include epigastric epigastric adjective Referring to the body region between the costal margins and the subcostal plane pain, nausea, vomiting, and are usually postprandial. Weight loss is common and can be severe, often raising the suspicion of malignancy. Upper GI bleeding can also be the initial presentation, and evaluation often leads to the diagnosis of sarcoidosis. (7)
Endoscopy may reveal ulcerations Ulcerations
Breaks in skin or mucous membranes that are often accompanied by loss of tissue on the surface.
Mentioned in: Hypersplenism , gastritis, or diffusely erythematous, friable friable /fri·a·ble/ (fri´ah-b'l) easily pulverized or crumbled.
1. Readily crumbled; brittle.
2. Relating to a dry, brittle growth of bacteria. mucosa that is elevated over the surroundings. (8) Nodular nodular
marked with, or resembling, nodules.
see nodular fasciitis (below).
a firm painless nodular swelling, 0. irregularities are also common, and mucosal polyps may be present occasionally. (3) Segmental mucosal thickening and nondistensibility that mimics the linitis plastica variety of gastric cancer is the most common abnormality seen on upper GI series.
The decision to treat gastrointestinal tract sarcoidosis is based upon the activity and extent of the disease. Corticosteroids are the treatment of choice, but alternative agents may be needed in patients who do not respond or cannot tolerate side effects. The recommended starting dose of steroids is 30 to 40 mg of prednisone daily. The dose should be gradually tapered to a maintenance dose of 10 to 15 mg over a period of approximately 6 months. (5) The disease activity is monitored clinically, radiographically, and by serum ACE levels. Antacids and metoclopramide can be used for symptomatic relief of abdominal pain or delayed gastric emptying. Surgery is required for those that develop pyloric stenosis or severe GI bleeding.
We describe a patient with pulmonary, liver, and gastric sarcoidosis. The patient had symptoms of weight loss, nausea, and early satiety, which resolved with corticosteroid therapy. Review of the literature suggests that gastric sarcoidosis is rare. However, because of its subclinical nature, it may actually be much more common than thought. It is therefore essential that histologic confirmation be established so that symptoms may be treated appropriately as they arise.
1. Fireman Z, Sternberg A, Yarchovsky Y, et al. Multiple antral ulcers in gastric sarcoid. J Clin Gastroenterol 1997;24:97-99.
2. Sprague R, Harper P, McClain S, et al. Disseminated gastrointestinal sarcoidosis: case report and review of the literature. Gastroenterology 1984;87:421-425.
3. Farman J, Ramirez G, Rybak B, et al. Gastric sarcoidosis. Abdom Imaging 1997;22:248-252.
4. Akinyemi E, Rohewal U, Tangorra M, et al. Gastric sarcoidosis. J Natl Med Assoc 2006;98:948-949.
5. Sharma A, Kadakia J, Sharma O. Gastrointestinal sarcoidosis. Semin Respir Med 1992;6:442.
6. Kaneki T, Koizumi T, Yamamoto H, et al. Gastric sarcoidosis: a single polypoid appearance in the involvement. Hepatogastroenterology 2001;48:1209-1210.
7. Chinitz M, Brandt L, Frank M, et al. Symptomatic sarcoidosis of the stomach. Dig Dis Sci 1985;30:682-688.
8. Chlumsky J, Krtek V, Chlumska A. Sarcoidosis of the stomach: endoscopic diagnosis and possibilities of conservative treatment. Hepatogastroenterology 1985;32:255-257.
I say, follow your bliss and don't be afraid, and doors will open where you didn't know they were going to be. --Joseph Campbell
Mark Friedman, MD, M. Aamir Ali, MD, and Marie L. Borum, MD, EdD, MPH
From the Division of Gastroenterology and Liver Diseases, George Washington University George Washington University, at Washington, D.C.; coeducational; chartered 1821 as Columbian College (one of the first nonsectarian colleges), opened 1822, became a university in 1873, renamed 1904. , Washington, DC.
Reprint requests to Dr. Mark Friedman, George Washington University, 2150 Pennsylvania Avenue, NW, Suite 3-408, Washington, DC 20037. Email: email@example.com
Accepted November 8, 2006.
RELATED ARTICLE: Key Points
* Symptomatic gastric sarcoidosis is a rare disease.
* The clinical manifestations of gastric sarcoidosis include abdominal pain, weight loss, nausea, vomiting, early satiety, and upper GI bleeding.
* Histological evidence of noncaseating granulomas consistent with sarcoidosis is necessary for diagnosis.
* Treatment with corticosteroid therapy usually causes resolution of symptoms.