Generalized megaviscera of lupus: Refractory intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus

Park, Frederick D.; Lee, Jeffrey K.; Madduri, Ganga D; Ghosh, Pradipta

doi:10.3748/wjg.15.3555

Cited by 35 publications

(39 citation statements)

References 11 publications

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“…A possible explanation for the poor response to therapy in this patient was the delay in commencement of immunosuppressants, which resulted in non-resolution of symptoms in a previous case report. 14 In 12 of the 18 patients reviewed by Mok et al and isolated case reports, 14,15 associated bilateral ureterohydronephrosis and megacholedochus were noted, which was absent in our patient. The classical histological feature of gastroparesis is intestinal leiomyocyte damage, 13 thus the normal gastric mucosal biopsy in this patient does not assist with the differential.…”

Section: Discussionsupporting

confidence: 48%

Gastroparesis in pregnancy: case report and literature review

et al. 2011

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Summary: Gastroparesis is a syndrome characterized by delayed gastric emptying in the absence of mechanical obstruction. The most common underlying aetiology is diabetes mellitus; however, many cases are idiopathic. Pregnancy per se is associated with gastrointestinal neuromuscular dysfunction; however, reports of gastroparesis arising during pregnancy are rare. We report a case of severe gastroparesis and proximal small bowel paresis presenting during pregnancy.Keywords: gastroenterology, high-risk pregnancy CASE REPORTA 24-year-old primigravida, at 10 weeks gestation, presented with nausea, vomiting, colicky right lower quadrant pain and an elevated white cell count. There was no history of fevers. She underwent uncomplicated laparoscopic appendicectomy; however, she was found to have congenital malrotation of the bowel, and the appendix, located in the right upper quadrant, was normal on histology. No cause of the right lower quadrant pain was identified.Post appendicectomy, the patient had ongoing symptoms and developed intractable large volume vomitus, initially containing 'old' food, and absence of bowel motions with minimal flatus. Two weeks later the patient presented to the emergency department with ongoing symptoms and 7 kg weight loss (8.1% of initial body weight, body mass index 31.6). The vital signs showed a blood pressure of 110/70 ( postural drop of 20 mmHg in systolic blood pressure), heart rate 96 beats per minute and temperature 37.18C. On examination the patient appeared unwell with clinical signs of dehydration and right lower quadrant abdominal tenderness with abdominal distension. There were no clinical features consistent with an autonomic or peripheral neuropathy. Laboratory tests were notable for the presence of a marked electrolyte disturbance, acute renal impairment with an elevated urea/ creatinine ratio (in keeping with dehydration) and deranged liver function tests (Table 1). The lipase and lactate were normal.The patient was prescribed antiemetic medication (metoclopramide, prochlorperazine and ondansetron), vitamin supplementation (thiamine and multivitamin), intravenous fluid therapy and kept nil by mouth. With hydration there was resolution of the postural drop, normalization of the renal and electrolyte disturbances, and improvement of the liver function tests. There was little change in symptomatology.An abdominal ultrasound showed a normal gallbladder, pancreas, spleen, kidneys and ureters with diffuse fatty infiltration of the liver (likely secondary to rapid weight loss). On day 8, an upper endoscopy performed showed a dilated stomach with a large amount of fluid residue. No focal abnormality was detected. The first and second parts of the duodenum were grossly dilated with an apparent transition point between segments D2 and D3 with no mucosal abnormality visualized. The distal D3 segment appeared normal. Gastric mucosal biopsy was normal. A diagnostic laparoscopy on day 9 revealed a dilated stomach with inflammatory adhesions at the duodenum, thought to be a point of obs...

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Section: Discussionsupporting

confidence: 48%

Gastroparesis in pregnancy: case report and literature review

et al. 2011

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“…However, restoration of bowel function, resolution of symptoms and prevention of complications like recurrent intra-peritoneal adhesions and irreparable tissue damage can only be achieved if there is early recognition and timely initiation of treatment. 3,10 Early detection also avoids unnecessary invasive procedures and other surgical intervention.…”

Section: Discussionmentioning

confidence: 99%

Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus

Khairullah

Raja

Yahya

et al. 2013

Lupus

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Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome of ineffective intestinal motility characterised by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. CIPO can either be idiopathic or secondary to a systemic disease, like systemic lupus erythematosus (SLE). Fewer than 30 cases of CIPO secondary to SLE have been reported so far. Here we describe a case of SLE with the initial presentation of CIPO. In SLE-related CIPO, treatment includes a combination of high-dose intravenous corticosteroids, immunosuppressants and supportive care. With awareness of this condition, unnecessary surgical intervention and repeated invasive procedures could be avoided. Early initiation of treatment would avoid complications and bring about resolution of symptoms.

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“…This rare triad of gastrointestinal, genitourinary, and hepatobiliary hollow viscera dilatation has been described in lupus only twice previously but never been distinguished in its early stage [4,5]. The pathophysiology of IPO secondary to SLE remains unclear [3], though several hypotheses have been proposed [1,4,[6][7][8].…”

Section: Discussionmentioning

confidence: 99%

“…The pathophysiology of IPO secondary to SLE remains unclear [3], though several hypotheses have been proposed [1,4,[6][7][8]. The high association between IPO and ureterohydronephrosis suggests a smooth muscle dysmotility due to a primary neuropathy or myopathy, or secondary to either an immune complexmediated vasculitis or common autoantibody against the smooth muscles [4] Generalized vasculitis involving inXammatory Wbrinoid deposits or smooth muscle muscularis propria dysmotility may also aVect the smooth muscle of the intestine and lead to small bowel obstruction. MoK MY et al [1] reported six cases and reviewed 12 cases presented with IPO in SLE, most of whom were Asia descent.…”

Section: Discussionmentioning

confidence: 99%

“…On the contrary, delayed recognition of this life-threatening problem may result in potentially fatal bowel infarction and perforation [6], recurrent bowel obstruction due to chronic Wbrosis, and pancreatitis secondary to biliary tract disease [5]. Moreover, chronic hydronephrosis without opportune therapy may lead to renal failure that eventually requires hemodialysis [4].…”

Section: Discussionmentioning

confidence: 99%

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Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

2011

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Intestinal pseudo-obstruction (IPO) is not uncommon in systemic lupus erythematosus (SLE), and IPO in SLE has an apparent association with ureterohydronephrosis. However, hepatobiliary dilatation without mechanical obstruction presenting together with IPO and ureterohydronephrosis is much more scarce in SLE. Here, we named this rare triad of IPO, ureterohydronephrosis, and biliary tract dilatation as visceral muscle dysmotility syndrome (VMDS). It always imitates an acute abdomen and is even life-threatening if treated incorrectly. To diagnose a VMDS, infections and mechanical obstructions should be ruled out carefully. Here, we report a 24-year-old Chinese woman with SLE who presented of VMDS that associated with corticoids tapering induced SLE flare. In this case, early vigorous immunosuppressive treatment conquered the triad timely and thus yielded a good outcome.

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Generalized megaviscera of lupus: Refractory intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus

Abstract: Dilated dysfunction involving multiple visceral organs

Cited by 35 publications

References 11 publications

Gastroparesis in pregnancy: case report and literature review

Gastroparesis in pregnancy: case report and literature review

Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus

Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

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