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Rheumatology 2001; 40: 1072-1073
© 2001 British Society for Rheumatology
Letters to the Editor |
Intravenous immunoglobulins and octreotide as treatment of intestinal pseudo-obstruction revealing a recurrence of polymyositis
Department of Internal Medicine and
1 Department of Gastroenterology, Centre Hospitalier Universitaire de Rouen, 76031 Rouen Cedex, France
SIR, Polymyositis (PM) and dermatomyositis (DM) are systemic inflammatory disorders affecting skeletal muscles and other organs, especially the digestive tract [1, 2]. Although the prevalence of oesophageal impairment in PM/DM patients has been reported to be as high as 60% [1], gastrointestinal involvement is less recognized in PM/DM, leading to dramatic complications, i.e. haemorrhage and perforation related to vasculitis, spontaneous abdominal haematoma, pneumatosis cystoides intestinalis [3–6] and more rarely intestinal pseudo-obstruction [7–9]. We recently observed a new case which is of particular interest, as the patient developed intestinal pseudo-obstruction, revealing a recurrence of steroid-resistant PM, with favourable outcome after initiation of therapy associating intravenous immunoglobulins (IVIG) and octreotide.
A 55-yr-old man had PM evolving from November 1998. PM diagnosis was made by Bohan and Peter criteria [1], i.e. (1) symmetric muscle weakness; (2) increased serum muscle enzymes; (3) myopathic changes on electromyography; and (4) muscle damage on histological examination. Muscle power was gauged for eight proximal muscles (neck flexors, trapezius, deltoid, biceps, psoas, maximus and medius gluteus, and quadriceps) by a modification of the British Medical Research Council grading system [1], resulting in scores ranging from 0 to 11 (theoretical maximum score: 88 points); muscle power was 63 points. Autoantibody screening was positive for antinuclear antibodies (ANA) (1:160). Other investigations, notably abdominal ultrasound and oesophageal manometry, were within normal limits. Treatment with prednisone was initiated at a dose of 1 mg/kg daily. As both clinical and biochemical status continued to deteriorate gradually, therapy of combined IVIG (at a dose of 1 g/kg for 2 consecutive days monthly for 6 months) and 30 mg weekly methotrexate was started, resulting in improvement of muscle clinical symptoms and biochemical tests. In November 1999, the patient received methotrexate 30 mg weekly and prednisone 27 mg per day; his muscle power was 81 points. In February 2000, the patient presented with a 3-month history of diffuse abdominal pain, nausea and severe constipation. On admission, his general condition was poor with a 5-kg weight loss. Physical examination revealed abdominal distention and tender abdomen upon palpation. General examination further showed muscle weakness involving upper and lower limbs; muscle power was 72 points. Laboratory findings were as follows: creatine kinase 2600 IU/l, aldolase 31.5 IU/l. Autoantibody screen was positive for ANA. Electromyography showed evidence of a myositic process. Other investigations, including salivary gland histology, were normal. Abdominal radiograph revealed gaseous distention of the small intestine. Further tests, i.e. gastroscopy, colonoscopy, small bowel barium meal and abdominal computed tomography (CT) scan, did not demonstrate mechanical obstruction. Small bowel manometry was performed; intestinal motility was continuously recorded for 24 h and the patient received 50 µg of octreotide subcutaneously at the end of the study. During the fasting period, the patient exhibited no spontaneous duodenal and jejunal phase III migrating motor complex (MMC); abnormal bursts of uncoordinated contractile activity were observed. The meals induced a post-prandial pattern, which was abnormal because of duodenal and jejunal contractions of decreased amplitude and frequency, and uncoordinated bursts of phasic pressure activity; 50 µg of subcutaneous octreotide stimulated a high-amplitude phase III-like activity within 2 min following infusion. The diagnosis of intestinal pseudo-obstruction, revealing a recurrence of steroid-resistant PM, was suspected. It was confirmed as stool cultures detected no bacteria, parasites or fungi, which excluded intestinal motor impairment related to bacterial overgrowth. Prokinetic therapy with cisapride was started in association with dietary measures (small and frequent liquid meals, low in fat and fibre). As digestive clinical symptoms continued to deteriorate, the patient was treated with combined IVIG at a dose of 1 g/kg for 2 consecutive days monthly for 6 months and octreotide at a daily dose of 50 µg subcutaneously. Prednisone was simultaneously decreased gradually to 2.5 mg every 3 weeks and methotrexate was discontinued. Intestinal clinical symptoms improved and completely healed after the third IVIG infusion; muscle manifestations concomitantly improved. In July 2000, the octreotide regimen was reduced to 50 µg three times a week. In August 2000, the patient remains free of digestive clinical features, receiving therapy of combined IVIG (0.5 g/kg for 2 consecutive days monthly for 6 additional months) and octreotide (50 µg three times a week).
Only a few authors have previously reported intestinal pseudo-obstruction in PM/DM patients resulting in intestinal atonia and dilatation on barium studies [7–9]. Our case report is original in that the patient presented with intestinal pseudo-obstruction revealing a recurrence of steroid-resistant PM. In this instance, the motor disorders could be attributed to intestinal pseudo-obstruction related to PM/DM, as: (1) all digestive tests demonstrated no mechanical obstruction; (2) stool cultures were negative, excluding underlying bacterial overgrowth; (3) the extensive search for other conditions associated with intestinal pseudo-obstruction, e.g. other connective tissue disorders, diabetes, hypothyroidism, amyloidosis, proved negative; and (4) dramatic improvement in both PM intestinal and muscle features took place after initiation of IVIG and octreotide. Our findings underscore the importance of recognizing such gastrointestinal complication due to PM/DM at an early stage, resulting in accurate management. Moreover, our case is, to our knowledge, the first to evaluate both duodenal and jejunal manometric disturbances during a 24-h period in a PM patient with intestinal pseudo-obstruction. Small bowel manometry was helpful in accurately demonstrating characteristic disturbances of intestinal pseudo-obstruction, i.e. absence of duodeno-jejunal phase III MMC in the fasting period, marked post-cibal duodeno-jejunal hypomotility, and bursts of uncoordinated contractions in fasting and post-prandial periods [10]. Our observation further highlights that intestinal manometry may be useful in PM patients with intestinal pseudo-obstruction, by accurately assessing the severity of motor disturbances and thereby assisting in therapy selection. Our PM patient with severe intestinal atony failed to improve after institution of cisapride, whereas he was successfully treated with combined IVIG and octreotide. Our data are therefore in accordance with those of Di Lorenzo [10] who mentioned a correlation between intestinal atony (i.e. absent phase III MMC on manometry) and unfavourable response of pseudo-obstruction symptoms to prokinetic drugs, which may be due to both reduced delivery and absorption of drugs; Di Lorenzo also noted that motor response to octreotide injection was predictive of pseudo-obstruction clinical improvement. Finally, our findings indicate that IVIG and octreotide should be considered in severe intestinal pseudo-obstruction complicating steroid-resistant PM, such a therapy further offering the advantages of short-term efficacy and good tolerance.
Notes
Correspondence to: I. Marie, 16 rue Arthur Duval, 76300 Sotteville lès Rouen, France. 
References
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[Abstract/Free Full Text] - Patterson M, Riss G. Disturbed gastrointestinal motility—an unusual manifestation of a systemic muscular disorder: polymyositis or progressive muscular dystrophy. Gastroenterology 1959;36:261–8.[Web of Science][Medline]
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