The Structure and Function of the Human Small Intestinal Microbiota: Current Understanding and Future Directions.

Despite growing literature characterizing the fecal microbiome and its association with health and disease, few studies have analyzed the microbiome of the small intestine. Here, we examine what is known about the human small intestinal microbiota in terms of community structure and functional properties. We examine temporal dynamics of select bacterial populations in the small intestine, and the effects of dietary carbohydrates and fats on shaping these populations. We then evaluate dysbiosis in the small intestine in several human disease models, including small intestinal bacterial overgrowth, short-bowel syndrome, pouchitis, environmental enteric dysfunction, and irritable bowel syndrome. What is clear is that the bacterial biology, and mechanisms of bacteria-induced pathophysiology, are enormously broad and elegant in the small intestine. Studying the small intestinal microbiota is challenged by rapidly fluctuating environmental conditions in these intestinal segments, as well as the complexity of sample collection and bioinformatic analysis. Because the functionality of the digestive tract is determined primarily by the small intestine, efforts must be made to better characterize this unique and important microbial ecosystem.

Is small intestinal bacterial overgrowth a cause of hyperdynamic circulation in cirrhosis?

BACKGROUND/AIMS: Small intestinal bacterial overgrowth (SIBO) and hemodynamic changes are common in cirrhosis. We wanted to examine our hypothesis whether SIBO leads to hemodynamic changes in cirrhosis. MATERIALS AND METHODS: A total of 50 patients with cirrhosis and 15 healthy controls were enrolled in a pilot prospective study. All participants underwent the lactulose hydrogen breath test for SIBO and echocardiography with a simultaneous assessment of blood pressure and heart rate. Cardiac output and systemic vascular resistance were calculated. RESULTS: Study participants with SIBO had a lower systolic blood pressure and systemic vascular resistance compared to those without SIBO and to healthy controls (110.2±12.3 mmHg vs. 126.2±21.0 mmHg and 121.2±9.8 mmHg; p=0.005 and p=0.011, respectively; 1312±352 dyn•s•cm-5 vs. 1704±424 dyn•s•cm-5 and 1648±272 dyn•s•cm-5; p=0.001 and p=0.006, respectively), but a higher cardiac output (5.38±1.41 l/min vs. 4.52±1.03 l/min and 4.40±0.68 l/min; p=0.034 and p=0.041, respectively) and C-reactive protein (10.5[1.2-16.5] mg/l vs. 2.8[0.6-9.1] mg/l; p=0.028; no comparison with healthy controls). There were no significant differences between patients without SIBO and healthy controls with regard to systolic blood pressure (p=0.554), systemic vascular resistance (p=0.874), and cardiac output (p=0.795). SIBO was associated with vasodilation and hyperdynamic circulation in decompensated cirrhosis (p=0.002; p=0.012), but not in compensated cirrhosis (p=1.000; p=0.474). CONCLUSIONS: SIBO is associated with hyperdynamic circulation and other hemodynamic changes in cirrhosis and may be a principal factor causing these through systemic inflammation.

Small intestinal bacterial overgrowth (SIBO) and vitamin K-responsive coagulopathy: a previously unrecorded association.

A 17-year-old woman, with a history of three operations on the upper gut in early life and intermittent diarrhoea, presented with a history of epistaxis and leg ecchymosis for the previous 3 months. Initial investigation revealed mild anaemia, low serum albumin, moderately elevated aminotransferases and an exceedingly prolonged prothrombin time (PT) which was promptly shortened to normal by intravenous vitamin K. Additional investigations revealed a grossly abnormal glucose hydrogen breath test, a dilated duodenum and deficiencies of vitamins A, D and E. Repeated courses of antimicrobial agents caused prompt but transient shortening of PT and eventually a duodenal-jejunal anastomosis was performed. Since then, up to 36 months later, the patient has been in good general health and PT has been consistently normal with no vitamin K supplementation. Small intestinal bacterial overgrowth has previously been associated with several conditions but this is the first description of its association with vitamin K-responsive coagulopathy.

Brain fogginess, gas and bloating: a link between SIBO, probiotics and metabolic acidosis.

BACKGROUND: D-lactic acidosis is characterized by brain fogginess (BF) and elevated D-lactate and occurs in short bowel syndrome. Whether it occurs in patients with an intact gut and unexplained gas and bloating is unknown. We aimed to determine if BF, gas and bloating is associated with D-lactic acidosis and small intestinal bacterial overgrowth (SIBO). METHODS: Patients with gas, bloating, BF, intact gut, and negative endoscopic and radiological tests, and those without BF were evaluated. SIBO was assessed with glucose breath test (GBT) and duodenal aspiration/culture. Metabolic assessments included urinary D-lactic acid and blood L-lactic acid, and ammonia levels. Bowel symptoms, and gastrointestinal transit were assessed. RESULTS: Thirty patients with BF and 8 without BF were evaluated. Abdominal bloating, pain, distension and gas were the most severe symptoms and their prevalence was similar between groups. In BF group, all consumed probiotics. SIBO was more prevalent in BF than non-BF group (68 vs. 28%, p = 0.05). D-lactic acidosis was more prevalent in BF compared to non-BF group (77 vs. 25%, p = 0.006). BF was reproduced in 20/30 (66%) patients. Gastrointestinal transit was slow in 10/30 (33%) patients with BF and 2/8 (25%) without. Other metabolic tests were unremarkable. After discontinuation of probiotics and a course of antibiotics, BF resolved and gastrointestinal symptoms improved significantly (p = 0.005) in 23/30 (77%). CONCLUSIONS: We describe a syndrome of BF, gas and bloating, possibly related to probiotic use, SIBO, and D-lactic acidosis in a cohort without short bowel. Patients with BF exhibited higher prevalence of SIBO and D-lactic acidosis. Symptoms improved with antibiotics and stopping probiotics. Clinicians should recognize and treat this condition.

Small Intestinal Bacterial Overgrowth: Nutritional Implications, Diagnosis, and Management.

Small intestinal bacterial overgrowth (SIBO), characterized by the presence of excessive bacteria in the small intestine, is typically described as a malabsorptive syndrome occurring in the context of gut stasis syndromes. SIBO is now considered to be a disorder associated with diverse clinical conditions without classic risk factors for SIBO and a cause of several nonspecific gastrointestinal and nongastrointestinal symptoms. Because there is currently no gold standard for diagnosing SIBO, its prevalence and role in the pathogenesis of other diseases remain uncertain; as does optimal treatment of patients with relapsing symptoms.

[The prevalence of bacterial overgrowth syndrome and its associated factors in patients with inflammatory bowel diseases (according to the data of the Novosibirsk registry)]. / Rasprostranennost' sindroma izbytochnogo bakterial'-nogo rosta i assotsiirovannye s nim faktory u bol'nykh vospalitel'nymi zabolevaniyami kishechnika (po dannym Novosibirskogo registra).

AIM: To assess the prevalence of bacterial overgrowth syndrome (BOS) and its risk factors in patients with inflammatory bowel diseases (IBD). SUBJECTS AND METHODS: The patients from the Novosibirsk IBD registry, who had undergone a hydrogen breath test (HBT) using a Gastro+ device, were examined. RESULTS: In 93 IBD patients who had undergone a HBD, the prevalence of BOS was 48% (46.2% for ulcerative colitis and 51.2% for Crohn's disease). There was a strong correlation between abdominal bloating, abdominal rumbling, and positive HBT results in both patient groups. During the HBT, the patients with BOS frequently complained of diarrhea, borborygmi, belching, and anxiety. CONCLUSION: The findings suggest that BOS is highly prevalent among patients with IBD. BOS is associated with clinical symptoms, such as abdominal bloating, abdominal rumbling, tearfulness, and irritability.

Levothyroxine therapy and impaired clearance are the strongest contributors to small intestinal bacterial overgrowth: Results of a retrospective cohort study.

AIM: To identify a set of contributors, and weight and rank them on a pathophysiological basis. METHODS: Patients who have undergone a lactulose or glucose hydrogen breath test to rule out small intestinal bacterial overgrowth (SIBO) for various clinical symptoms, including diarrhoea, weight loss, abdominal pain, cramping or bloating, were seen as eligible for inclusion in a retrospective single-centre study. Clinical data such as co-morbidities, medication, laboratory parameters and other possible risk factors have been identified from the electronic data system. Cases lacking or with substantially incomplete clinical data were excluded from the analysis. Suspected contributors were summarised under four different pathophysiological pathways (impaired gastric acid barrier, impaired intestinal clearance, immunosuppression and miscellaneous factors including thyroid gland variables) and investigated using the χ2 test, Student's t-test and logistic regression models. RESULTS: A total of 1809 patients who had undergone hydrogen breath testing were analysed. Impairment of the gastric acid barrier (gastrectomy, odds ratio: OR = 3.5, PPI therapy OR = 1.4), impairment of intestinal clearance (any resecting gastric surgery OR = 2.6, any colonic resection OR = 1.9, stenosis OR = 3.4, gastroparesis OR = 3.4, neuropathy 2.2), immunological factors (any drug-induced immunosuppression OR = 1.8), altered thyroid gland metabolism (hypothyroidism OR = 2.6, levothyroxine therapy OR = 3.0) and diabetes mellitus (OR = 1.9) were associated significantly to SIBO. Any abdominal surgery, ileocecal resection, vagotomy or IgA-deficiency did not have any influence, and a history of appendectomy decreased the risk of SIBO. Multivariate analysis revealed gastric surgery, stenoses, medical immunosuppression and levothyroxine to be the strongest predictors. Levothyroxine therapy was the strongest contributor in a simplified model (OR = 3.0). CONCLUSION: The most important contributors for the development of SIBO in ascending order are immunosuppression, impairment of intestinal clearance and levothyroxine use, but they do not sufficiently explain its emergence.

Assessment of intestinal malabsorption.

Significant efforts have been made in the last decade to either standardize the available tests for intestinal malabsorption or to develop new, more simple and reliable techniques. The quest is still on and, unfortunately, clinical practice has not dramatically changed. The investigation of intestinal malabsorption is directed by the patient's history and baseline tests. Endoscopy and small bowel biopsies play a major role although non-invasive tests are favored and often performed early on the diagnostic algorithm, especially in paediatric and fragile elderly patients. The current clinically available methods and research tools are summarized in this review article.

Additional Value of CH4 Measurement in a Combined (13)C/H2 Lactose Malabsorption Breath Test: A Retrospective Analysis.

The lactose hydrogen breath test is a commonly used, non-invasive method for the detection of lactose malabsorption and is based on an abnormal increase in breath hydrogen (H2) excretion after an oral dose of lactose. We use a combined (13)C/H2 lactose breath test that measures breath (13)CO2 as a measure of lactose digestion in addition to H2 and that has a better sensitivity and specificity than the standard test. The present retrospective study evaluated the results of 1051 (13)C/H2 lactose breath tests to assess the impact on the diagnostic accuracy of measuring breath CH4 in addition to H2 and (13)CO2. Based on the (13)C/H2 breath test, 314 patients were diagnosed with lactase deficiency, 138 with lactose malabsorption or small bowel bacterial overgrowth (SIBO), and 599 with normal lactose digestion. Additional measurement of CH4 further improved the accuracy of the test as 16% subjects with normal lactose digestion and no H2-excretion were found to excrete CH4. These subjects should have been classified as subjects with lactose malabsorption or SIBO. In conclusion, measuring CH4-concentrations has an added value to the (13)C/H2 breath test to identify methanogenic subjects with lactose malabsorption or SIBO.

Small Intestinal Transit Time Is Delayed in Small Intestinal Bacterial Overgrowth.

BACKGROUND: Altered small intestinal motility is thought to contribute to the development of small intestinal bacterial overgrowth (SIBO). The clinical manifestations of SIBO and consequent malabsorption are wide ranging and include abdominal pain, bloating, diarrhea, weight loss, and nutritional deficiencies. However, due to the nonspecific nature of symptoms, the diagnosis may often be overlooked. To date, few studies have illustrated a direct relationship between impaired small intestinal motility and SIBO. In addition, further study has been limited by the technical challenges and lack of widespread availability of antroduodenal manometry. The development of a wireless motility capsule (WMC) (SmartPill) that evaluates pressure, pH, and temperature throughout the GI tract offers the potential to identify patients with small bowel transit delays who may be at risk for bacterial overgrowth. AIMS: The primary aims of this study were to: (1) characterize the relationship of prolonged small bowel transit time (SBTT) in patients undergoing WMC with SIBO as based on a positive lactulose breath testing (LBT); and (2) to assess the relationship of prolonged gastric, colonic, and whole gut transit times (WGTT) and additional motility parameters with SIBO (positive LBT). We also sought to evaluate the relationship of small bowel motility parameters (SB motility index, contractions per minute, and SB peak amplitudes) with LBT results. METHODS: We performed a retrospective study of consecutive patients who were referred for wireless motility testing at a single, tertiary care institution from April 2009 to December 2012. Of the 72 total patients identified, 34 underwent both WMC and LBT. Gastric, small bowel, colonic, WGTT, and SB motility parameters were measured and correlated with LBT results. Statistical methods utilized for data analysis include ANOVA, 2-sample t tests, nonparametric Kruskal Wallis test, Wilcoxon rank-sum test, and the Fisher exact test. RESULTS: Of the 37 patients who underwent both WMC and LBT, 24 (65%) were LBT positive. The mean SBTT among those who were LBT positive was 6.6 hours as compared with 4.2 hours in those who were LBT negative (P=0.04). Among patients who were LBT positive, 47.6% had prolonged SBTT (≥6 h), whereas only 7.7% of those who were LBT negative had a delay in their SBTT (P=0.01). In addition, patients who were LBT positive were more likely to have prolongation of both colonic and WGTT versus those who were LBT negative (CTT: positive LBT=64.4 h vs. negative LBT=35.5 h, P=0.02; WGTT: positive LBT=70.5 h vs. negative LBT=44.1 h, P=0.02). However, there were no statistical differences observed between the groups for gastric emptying times or other small intestinal motility parameters (SB motility index, contractions per minute, and peak amplitudes) between the 2 groups. CONCLUSIONS: Patients with underlying SIBO have significant delays in SBTT as compared with those without. The association between prolonged SBTT and positive LBT may be useful in identifying those patients with SIBO diagnosed by LBT and potentially target therapeutic options for those refractory to standard therapy. Interestingly, patients with positive LBT did not necessarily have a generalized gastrointestinal motility (similar GETs among groups), suggesting that small bowel transit specifically predisposes to the development of SIBO. Future, prospective studies are needed to further characterize intestinal dysmotility and other contributing pathophysiological mechanisms in SIBO and to investigate the potential benefits of prokinetics in this challenging patient population.

Jejunoileal bypass as the main procedure in the onset of immune-related conditions: the model of BADAS.

Bariatric surgery represents a common approach for the control of severe morbid obesity, reducing caloric intake by modifying the anatomy of the gastrointestinal tract. Following jejunoileal bypass, a large spectrum of complications has been described, with rheumatic manifestation present in up to 20% of cases. Although bowel bypass syndrome, also called blind loop syndrome, is a well-recognized complication of jejunoileal bypass, the same syndrome was recognized in patients who had not had intestinal bypass surgery, and the term the 'bowel-associated dermatosis-arthritis syndrome' (BADAS) was coined. The pathogenesis of BADAS is as yet poorly understood and only few data concerning this issue have been published in the literature. The aim of the present paper is to review the literature and to discuss putative pathogenic mechanisms of BADAS, focusing on the immune system.

Home parenteral nutrition in patients with systemic sclerosis: a retrospective review of 12 cases.

OBJECTIVE: To evaluate the effect of home total parenteral nutrition (HTPN) on the nutritional and functional status of patients with systemic sclerosis (SSc). METHODS: Retrospective review of SSc patients from the HTPN database at the University Health Network, Toronto, Canada, from 1998 to 2010. RESULTS: In total 12 subjects (9 females, 3 males) with SSc were identified. At initiation of HTPN, the age was 48.75 ± 3.94 years and the median body mass index was 16.8 kg/m(2) (range 12.3-21.3). This significantly increased to 18.3 kg/m(2) (17.0-21.7) at 6 months and to 19.7 kg/m(2) (17.0-24.6) at 12 months. The mean Karnofsky score at baseline was 58.00 ± 3.27, and the score at 12 months of 39.00 ± 13.29 did not change significantly. CONCLUSION: HTPN improved the nutritional status of these patients with SSc but it did not improve their functional status.

D-Lactic acidosis 25 years after bariatric surgery due to Salmonella enteritidis.

D-Lactic acidosis is a rare complication that occurs in patients with short bowel syndrome due to surgical intestine resection for treatment of obesity. The clinical presentation is characterized by neurologic symptoms and high anion gap metabolic acidosis. The incidence of this syndrome is unknown, probably because of misdiagnosis and sometimes symptoms may be incorrectly attributed to other causes. Therapy is based on low carbohydrate diet, sodium bicarbonate intravenous, rehydratation, antiobiotics, and probiotics that only produce L-lactate. In the case we describe, D-lactic acidosis encephalopathy occurred 25 y after bypass jejunoileal, due to Salmonella enteriditis infection.

Orocecal transit time and small intestinal bacterial overgrowth in type 2 diabetes patients from North India.

BACKGROUND: Diarrhea, constipation, flatulence, and abdominal pain are common complaints in type 2 diabetes patients. Impaired intestinal motility is often followed by small intestinal bacterial overgrowth (SIBO). Therefore, this study was planned to determine the incidence of SIBO and its relation with orocecal transit time (OCTT) in type 2 diabetes patients. SUBJECTS AND METHODS: SIBO and OCTT were measured by using noninvasive glucose and lactulose hydrogen breath tests, respectively. For this study, 84 patients with type 2 diabetes mellitus in the age range 30-65 years and 45 age-matched apparently healthy controls were enrolled. RESULTS: The glucose hydrogen breath test was suggestive of SIBO in 15.5% of patients with type 2 diabetes mellitus but in one (2.2%) of controls. There was a significant increase (P<0.001) in OCTT in type 2 diabetes patients compared with controls. It was also observed that OCTT in type 2 diabetes patients with SIBO was significantly delayed (P<0.001) compared with type 2 diabetes patients without SIBO. CONCLUSION: This study indicates that SIBO in diabetes patients may be due to delayed OCTT.

Small intestinal clustered contractions and bacterial overgrowth: a frequent finding in obese patients.

BACKGROUND: Small intestinal bacterial overgrowth (SIBO) has been observed in several disorders of the gastrointestinal tract. Studies have shown abnormalities of motor function in obese patients, and there is indirect evidence suggesting that SIBO is present in them. AIMS: To study small intestinal motility and the prevalence of SIBO in obese patients and to determine whether there was any relationship between both parameters. METHODS: Thirty-nine patients scheduled for bariatric surgery were subjected to hydrogen breath test with lactulose and to a stationary small intestinal motility study with perfused catheters. RESULTS: SIBO was observed in 41% of obese patients and was not related to body mass index. Small intestinal manometry showed a marked increase of clustered contractions in obese patients with SIBO compared to obese subjects without SIBO, whereas all the other parameters of fasting cyclic activity were not different. CONCLUSIONS: SIBO was a frequent finding in obese patients and was associated with an increased pattern of clustered contractions, which was not observed in absence of SIBO.

Small intestinal bacterial overgrowth syndrome.

Human intestinal microbiota create a complex polymicrobial ecology. This is characterised by its high population density, wide diversity and complexity of interaction. Any dysbalance of this complex intestinal microbiome, both qualitative and quantitative, might have serious health consequence for a macro-organism, including small intestinal bacterial overgrowth syndrome (SIBO). SIBO is defined as an increase in the number and/or alteration in the type of bacteria in the upper gastrointestinal tract. There are several endogenous defence mechanisms for preventing bacterial overgrowth: gastric acid secretion, intestinal motility, intact ileo-caecal valve, immunoglobulins within intestinal secretion and bacteriostatic properties of pancreatic and biliary secretion. Aetiology of SIBO is usually complex, associated with disorders of protective antibacterial mechanisms (e.g. achlorhydria, pancreatic exocrine insufficiency, immunodeficiency syndromes), anatomical abnormalities (e.g. small intestinal obstruction, diverticula, fistulae, surgical blind loop, previous ileo-caecal resections) and/or motility disorders (e.g. scleroderma, autonomic neuropathy in diabetes mellitus, post-radiation enteropathy, small intestinal pseudo-obstruction). In some patients more than one factor may be involved. Symptoms related to SIBO are bloating, diarrhoea, malabsorption, weight loss and malnutrition. The gold standard for diagnosing SIBO is still microbial investigation of jejunal aspirates. Non-invasive hydrogen and methane breath tests are most commonly used for the diagnosis of SIBO using glucose or lactulose. Therapy for SIBO must be complex, addressing all causes, symptoms and complications, and fully individualised. It should include treatment of the underlying disease, nutritional support and cyclical gastro-intestinal selective antibiotics. Prognosis is usually serious, determined mostly by the underlying disease that led to SIBO.

Small intestinal bacterial overgrowth in systemic sclerosis.

OBJECTIVES: The aims of this study were to: (i) determine the prevalence of small intestinal bacterial overgrowth (SIBO) in unselected patients with SSc; (ii) assess both clinical presentation and outcome of SIBO; and (iii) make predictions about which SSc patients are at risk for SIBO. METHODS: Fifty-one consecutive patients with SSc underwent glucose hydrogen and methane (H(2)/CH(4)) breath test. All SSc patients also completed a questionnaire for intestinal symptoms, and a global symptomatic score (GSS) was calculated. SSc patients with SIBO were given rotating courses of antibiotics (norfloxacin/metronidazole) for 3 months; glucose H(2)/CH(4) breath test was performed at 3-month follow-up. RESULTS: The prevalence of SIBO was 43.1% in our SSc patients. After logistic regression, we identified the following risk factors for SIBO: presence of diarrhoea and constipation. Interestingly, we observed a marked correlation between values of GSS of digestive symptoms (> or =5) and the presence of SIBO (P = 10(-6)); indeed, both sensitivity and specificity of GSS > or =5 to predict SIBO were as high as 0.909 and 0.862, respectively. Finally, eradication of SIBO was obtained in 52.4% of the SSc patients with a significant improvement of intestinal symptoms. CONCLUSION: Our study underscores that SIBO often occurs in SSc patients. We further suggest that GSS may be systematically performed in SSc patients; since we found a correlation between GSS of digestive symptoms > or =5 and SIBO, we suggest that glucose H(2)/CH(4) breath test may be performed in the subgroup of SSc patients exhibiting GSS > or =5.

Small intestinal bacterial overgrowth mimicking acute flare as a pitfall in patients with Crohn's Disease.

BACKGROUND: Small intestinal bacterial overgrowth (SIBO) is characterized by excessive proliferation of colonic bacterial species in the small bowel. Potential causes of SIBO include fistulae, strictures or motility disturbances. Hence, patients with Crohn's Disease (CD) are especially predisposed to develop SIBO. As result, CD patients may experience malabsorption and report symptoms such as weight loss, watery diarrhea, meteorism, flatulence and abdominal pain, mimicking acute flare in these patients. METHODS: One-hundred-fifty patients with CD reporting increased stool frequency, meteorism and/or abdominal pain were prospectively evaluated for SIBO with the Hydrogen Glucose Breath Test (HGBT). RESULTS: Thirty-eight patients (25.3%) were diagnosed with SIBO based on positive findings at HGBT. SIBO patients reported a higher rate of abdominal complaints and exhibited increased stool frequency (5.9 vs. 3.7 bowel movements/day, p = 0.003) and lower body weight (63.6 vs 70.4 kg, p = 0.014). There was no correlation with the Crohn's Disease Activity Index. SIBO was significantly more frequent in patients with partial resection of the colon or multiple intestinal surgeries; there was also a clear trend in patients with ileocecal resection that did not reach statistical significance. SIBO rate was also higher in patients with affection of both the colon and small bowel, while inflammation of the (neo)terminal ileum again showed only tendential association with the development of SIBO. CONCLUSION: SIBO represents a frequently ignored yet clinically relevant complication in CD, often mimicking acute flare. Because symptoms of SIBO are often difficult to differentiate from those caused by the underlying disease, targeted work-up is recommended in patients with corresponding clinical signs and predisposing factors.

[Gastrointestinal involvement in systemic sclerosis. An underestimated complication]. / Gastrointestinale Beteiligung bei systemischer Sklerose. Eine häufig verharmloste Komplikation.

Systemic sclerosis (SSc) belongs to the family of autoimmune connective tissue diseases and is still a challenge to every practicing physician. The disorder is characterized by progressing fibrosis of the skin and internal organs, abnormal activation of the immune system, and distinct changes in microcirculation. Although it is rare--with a prevalence of about 20:100000--patients need to be cared for in a daily setting. In general thickening of the skin is the first sign of the disease, so dermatologists are most frequently consulted first. Two subtypes exist, limited and diffuse forms. Both entities usually involve internal organs, and therefore interdisciplinary cooperation is mandatory. The increased morbidity and mortality depend predominantly on the grade of involvement of the affected organs. Therefore it is essential to diagnose systemic sclerosis early and to identify and monitor all complications closely. In this respect gastrointestinal involvement is frequently neglected, owing to its primarily non-life-threatening character, resulting in substantially delayed therapy.