Gut 1998;43:117–122 117 Chronic intestinal pseudo-obstruction in systemic lupus erythematosus Gut: first published as 10.1136/gut.43.1.117 on 1 July 1998. Downloaded from G Perlemuter, S Chaussade, B Wechsler, P Cacoub, M Dapoigny, A Kahan, P Godeau, D Couturier Abstract enteric nerves, or the visceral autonomic nerv- Background/Aims—Chronic intestinal ous system. It may be the primary disease or it pseudo-obstruction (CIPO) reflects a dys- may be secondary to a recognised underlying function of the visceral smooth muscle or disease and then defined as secondary CIPO. the enteric nervous system. Gastrointesti- The causes of secondary CIPO are numerous nal manifestations are common in systemic and involve the nervous, endocrine, and meta- lupus erythematosus (SLE) but CIPO has bolic systems, intra-abdominal inflammation, not been reported. Features of CIPO are infiltrative and connective tissue diseases, and reported in five patients with SLE. drug induced states.2 Mild gastrointestinal Methods—From 1988 to 1993, five patients manifestations are common in systemic lupus with SLE or SLE-like syndrome were hos- erythematosus (SLE). Nausea, vomiting, diar- pitalised for gastrointestinal manometric rhoea, and abdominal pain are found in more studies. CIPO was the onset feature in two than 50% of these patients.3 Motility disorders cases. Antroduodenal manometry (three of the gastrointestinal tract are less frequent. hours fasting, two hours fed) was per- CIPO is rare in SLE. Since our first published formed in all patients, and oesophageal case,4 four other patients with SLE have been manometry in four. hospitalised in our department with a diagnosis Results—Intestinal hypomotility associ- of CIPO, confirmed by manometry. We report ated with reduced bladder capacity and here their clinical, biological, and manographic bilateral ureteral distension was found in features. four patients and aperistalsis of the oesophagus in three. Treatment, which consisted of high dose corticosteroids, Materials and methods parenteral nutrition, promotility agents, From 1988 to 1993, five women, aged 19 to 39 and antibiotics, led to remission of both (mean 33), were hospitalised for gastrointesti- http://gut.bmj.com/ CIPO and urinary abnormalities in all nal manometric studies. Three were sent from Service cases. Antroduodenal manometry per- a department of internal medicine because of d’Hépatogastro- food intolerance during SLE. The two other entérologie, Hôpital formed in two patients after remission showed increased intestinal motility. One patients were hospitalised in a gastroenterology Cochin, 27 rue du department for food intolerance, but no Faubourg Saint patient died, and postmortem examin- underlying disease was known to be present. Jacques, 75674 Paris ation showed intestinal vasculitis. Cedex 14, France All the patients had had the symptoms for at Conclusions—CIPO in SLE is a life on September 24, 2021 by guest. Protected copyright. G Perlemuter threatening situation that can be reversed least six months before the manometric S Chaussade investigation. Between 1988 and 1993, 270 by treatment. It may be: (a) a complica- D Couturier other antroduodenal or duodenojejunal man- tion or onset feature of the disease; (b) secondary to smooth muscle involvement; ometries were performed in our department. Service de Médecine All the patients had abdominal pain, vomit- interne, Hôpital (c) associated with ureteral and vesical ing, abdominal distension, diarrhoea, constipa- Pitié-Salpétrière, 83 involvement; (d) the result of intestinal boulevard de l’Hôpital, vasculitis. tion, and weight loss (table 1). In four cases, 75651 Paris Cedex 13 (Gut 1998;43:117–122) plain abdominal x ray films showed gaseous B Wechsler distension of the small bowel and, in the P Cacoub Keywords: chronic intestinal pseudo-obstruction; upright films, the presence of fluid in the bowel P Godeau systemic lupus erythematosus loops (fig 1). Gastroscopy, colonoscopy, and Service small bowel series did not show any mechanical d’Hépatogastro- Chronic intestinal pseudo-obstruction (CIPO) obstruction. The large loss of weight and the entérologie, Hôpital is a clinical syndrome characterised by ineVec- extradigestive features did not support a Hôtel-Dieu, boulevard tive intestinal propulsion.1 It can be caused by diagnosis of irritable bowel syndrome. A Léon-Malfreyt, 63003 involvement of the visceral smooth muscle, the laparotomy was performed in patient 3 because Clermont-Ferrand Cedex, France Table 1 Clinical and immunological features of SLE M Dapoigny Prior Extradigestive features Service de course ACR Weight Anti-dsDNA Anti-ENA Rhumatologie, Hôpital Case Disease Age (y) (years) criteria loss (kg) RCNUANA 1/ antibody antibody Cochin A Kahan 1 SLE-like 19 0 3 10 − − − + 320 − anti-RNP 2 SLE 28 7 5 5 + + + + 4000 1000 anti-Sm 3 SLE 29 0 3 25 + − − − 100 − anti-RNP Correspondence to: 4 SLE 34 1 6 40 + − + + 400 − − Professor S Chaussade. 5 SLE 29 1 5 12 + + − + 5000 80 anti-RNP Accepted for publication ACR, American College of Rheumatology; R, rheumatological; C, cardiological; N, neurological; U, urological (reduced bladder 19 January 1998 capacity and bilateral ureteral distension); SLE, systemic lupus erythematosus. 118 Perlemuter, Chaussade, Wechsler, et al Gut: first published as 10.1136/gut.43.1.117 on 1 July 1998. Downloaded from Figure 1 Plain abdominal radiograph (upright film). Figure 2 Urinary involvement during systemic lupus Gaseous distension of the small bowel with the presence of erythematosus and chronic intestinal pseudo-obstruction fluid in the bowel loops is observed. (intravenous urography). There is an increase in the thickness of the bladder wall with marked reduction in capacity associated with bilateral ureteral distension of the extensive vomiting despite normal x ray (patient 1). films. Everything was found to be normal. The association of symptoms and signs of mechani- the aid of a steerable catheter on the day before cal bowel obstruction in the absence of an recording. In two patients, the perfusion tube occluding lesion of the intestinal lumen are in consisted of four catheters which were posi- http://gut.bmj.com/ accordance with the criteria of SchuZer et al,2 tioned in the duodenum and the proximal jeju- and Christensen et al,5 for CIPO. num 10 cm from each other. In the other three The diagnosis of SLE was made in three patients, the perfusion tube consisted of eight patients because of the presence of at least four perfusion catheters (Arndorfer Instruments, of the criteria of the American College of Milwaukee, WI, USA). One side opening was Rheumatology (ACR). One had only three of made in each catheter. Three of these openings the criteria, but the diagnosis of SLE was were 10 cm apart, with the distal port at 30 cm highly suggestive because of the association of into the proximal jejunum. The remaining five on September 24, 2021 by guest. Protected copyright. arthralgia,positiveantinuclearantibodies,hypo- openings were placed at 1 cm intervals and complementaemia, positive lupus band test on positioned across the antroduodenal junction. normal unexposed skin, and IgG, C1q, IgA, The eight catheters comprising a single mano- and C3 deposits in the mesangial areas of the metric assembly were each perfused with glomeruli.6 A diagnosis of Sjögren’s syndrome distilled water via a low compliance pneumo- was suggested for one patient because of the hydraulic pump (perfusion rate 0.1 ml/min; presence of five of the six criteria of the perfusion pressure 96.6 kPa) and attached to a European Community7: ocular symptoms, oral strain gauge transducer chart recorder (As- symptoms, ocular signs, focus score of 2 on tromed 2000) with a paper speed of 0.25 minor salivary gland biopsy specimen, pres- mm/second.8 A radiograph of the abdomen was ence of serum antibodies to SS-A antigens, and obtained on the day before and immediately presence of antinuclear antibodies. Never- before the recording to localise the position of theless, the clinical picture of this patient was the catheters. Recordings were obtained for very close to SLE and can be considered as an three hours with the patient in the fasting state SLE-like syndrome with three strong ACR cri- after a 12 hour fast and for two hours after teria of SLE: persistent proteinuria greater ingestion of a standard mixed solid and liquid than 0.5 g per day, haemolytic anaemia with meal consisting of chicken, potatoes, butter, reticulocytosis, and antinuclear antibodies. water (24.3% protein, 55.5% carbohydrate, Urinary echography and/or intravenous 20.2% fat, and 190 ml water, total energy 511 urography were carried out for all patients. kcal). Manometric studies consisted of antroduo- Intestinal manometric tracings were ana- denal or small bowel manometry in all patients lysed visually as described previously.8 Abnor- and oesophageal manometry in four. Gastroin- malities were identified by comparison with a testinal manometry was performed in each large pool of similar manometric studies by the patient after a 12 hour fast. A multilumen per- same experienced examiner (SC). Fasting trac- fusion tube was placed fluoroscopically with ings were evaluated for the presence of aberrant Intestinal pseudo-obstruction in systemic lupus erythematosus 119 Table 2 Oesophageal manometry in two patients and over the entire length in one. The lower oesophageal sphincter pressure Lower oesophageal Propagated wave sphincter pressure amplitude was decreased in all cases. Quantitative solid Case (N=14–65 cm H2O) (N >50 cm H2O) Per cent non-propagated waves (N <10%) phase gastric emptying studies were performed Gut: first published as 10.1136/gut.43.1.117 on 1 July 1998. Downloaded from 1ND ND ND in two patients using technetium-99m and 2 10,5 16 Aperistalsis in lower third of oesophagus showed delayed gastric emptying. 3 12 40 <10 4 6 20 DiVuse aperistalsis in oesophagus 5 0 0 Aperistalsis in lower third of oesophagus MOTILITY OF THE STOMACH AND PROXIMAL N, normal; ND, not done. SMALL BOWEL (TABLE 3) During the three hour fasting period, only one patient exhibited normal amplitude and dura- tion of the propagated phase III of the MMC.