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

Khairullah, Shasha; Raja, Jasmin; Yahya, Fariz; Cheah, Tien Eang; Ng, Chin Teck; Sockalingam, Sargunan

doi:10.1177/0961203313492873

Cited by 36 publications

(18 citation statements)

References 10 publications

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“…CIP is considered to be caused by the autoimmune inflammatory involvement of the visceral smooth muscle and enteric nervous system (10). Nonsurgical treatments that are often effective against CIP include high doses of corticosteroids, fasting, nasogastric drainage, total parenteral nutrition and administration of gastromotor or anti-infective drugs (11). In the present case, CIP was diagnosed and, although the patient had previously been treated by conservative therapy, the disease reappeared three more times.…”

Section: Discussionmentioning

confidence: 57%

Systemic lupus erythematosus with intestinal perforation: A case report

Zhu

Wang

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease, which can affect almost all systems and organs. Gastrointestinal disorder is one of the most noteworthy complications of patients with SLE. However, gastrointestinal disorder with intestinal perforation is rare, but potentially life-threatening if not treated promptly. The present study reported a case of SLE with intestinal perforation, where surgical intervention was performed and a crevasse (~3 cm in diameter) was detected in the ileum, ~60 cm from the ileocecal valve. Following surgery, the patient suffered from difficult ventilator weaning, septic shock and intestinal obstruction. The patient was successfully treated and discharged from the hospital after ~4 months of treatment. Intestinal perforation in SLE patients is potentially life-threatening; early diagnosis and prompt treatment are crucial to the management of this rare complication of SLE.

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Section: Discussionmentioning

confidence: 57%

Systemic lupus erythematosus with intestinal perforation: A case report

Zhu

Wang

et al. 2015

Experimental and Therapeutic Medicine

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“…[2][3][4] Patients usually present with symptoms of abdominal pain, bloating, and vomiting; less commonly constipation and diarrhea; and often have weight loss secondary to malnutrition. 3,5,6 Clinical diagnosis is based on these nonspecific, protracted symptoms suggestive of obstruction; however, no identifiable lesion or evidence of mechanical obstruction can be found on endoscopic and radiologic evaluation. 6,7 Small bowel manometry can help differentiate mechanical from functional obstruction and suggest myopathic versus neurogenic type; nevertheless, manometry is of limited diagnostic value due to its low specificity.…”

Section: Discussionmentioning

confidence: 99%

“…5,[11][12][13][14][15] Most cases, though, have no identified underlying cause and are described as idiopathic or primary. 1,11,15 Although mostly sporadic, familial cases have been reported with varied modes of inheritance and are more frequent in mitochondrial neurogastrointestinal encephalomyopathy.…”

Section: Classificationmentioning

confidence: 99%

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Two Cases of Chronic Intestinal Pseudo-obstruction: A Comparison of Staining Characteristics of Enteric Visceral Myopathy With Hirschsprung Disease

Chaffin

Lee²,

Rao

et al. 2016

Applied Immunohistochemistry &Amp; Molecular Morphology

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Chronic intestinal pseudo-obstruction (CIPO), a rare, debilitating disorder of bowel motility dysfunction, is largely a clinical diagnosis, without any universally accepted diagnostic criteria. Three subgroups are generally acknowledged based on the cell-type affected: enteric visceral myopathy (the most common subgroup), neuropathy, and mesenchymopathy. A fourth subgroup includes abnormalities of neurohormonal peptides. Although immunohistochemical staining is reportedly useful for identifying the mesenchymopathic type, its role in diagnosing enteric visceral myopathy and neuropathy has been fraught with difficulties. We present two cases of chronic intestinal pseudo-obstruction that are clinically and histopathologically suggestive of type III visceral enteric myopathy, aiming to expound upon the diagnostic and pathogenic features. We found that the outer-longitudinal layer of the muscularis propria was more severely affected as compared with the inner circular layer. To investigate the value of this finding, we performed immunostains in the one case in which a paraffin block was available. We found increased peripherin and calretinin immunopositive nerve fibers in the outer layer as compared with inner, but without any significant increase in S-100 positivity or alteration in neuronal morphology of myenteric plexus, a novel finding. This differential staining pattern was completely different from Hirschsprung disease, in which we found rare to absent peripherin and calretinin staining. It is unclear if this increase in the outer layer in visceral myopathy reflects a reactive change or dysfunctional axons. In addition, the history of volvulus in one patient and transmural inflammatory changes in the second raise concerns about the higher propensity of clinical complications secondary to the attenuated outer muscular layer. This study suggests that enteric visceral myopathy has histologic and staining characteristics different from Hirschsprung disease, a finding of diagnostic significance in the differential diagnosis of bowel obstruction. Moreover, these features may have pathogenic value and need further confirmation.

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“…CIPO is idiopathic in most cases. Etiologies causing CIPO include neurologic, endocrine, collagen vascular, paraneoplastic, infectious and genetic disorders (Table 2 ) [ 12 - 14 ]. Histological features of CIPO include myenteric plexus infiltration with plasma cells and lymphocytes associated with axonal and neuronal degeneration [ 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

The great masquerader of malignancy: chronic intestinal pseudo-obstruction

et al. 2014

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Paraneoplastic syndromes can precede the initial manifestation and diagnosis of cancer. Paraneoplastic syndromes are a heterogeneous group of disorders caused by mechanisms other than the local presence of tumor cells. These phenomena are mediated by humoral factors secreted by tumor cells or by tumor mediated immune responses. Among paraneoplastic syndromes, chronic intestinal pseudo-obstruction (CIPO) is rare and represents a particularly difficult clinical challenge. Paraneoplastic CIPO is a highly morbid syndrome characterized by impaired gastrointestinal propulsion with symptoms and signs of mechanical bowel obstruction. Clinical outcomes of paraneoplastic CIPO are often deleterious. The current standard of care for the management of CIPO includes supportive treatment with promotility and anti-secretory agents. However, the majority of patients with CIPO eventually require the resection of the non-functioning gut segment. Here, we present a 62-year-old patient with anti-Hu antibody associated paraneoplastic CIPO and underlying small cell lung cancer who underwent treatment with cisplatin and etoposide. Herein, we discuss diagnosis, prognosis, proposed mechanisms, treatment options, and future potential therapeutic strategies of paraneoplastic CIPO.

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

Cited by 36 publications

References 10 publications

Systemic lupus erythematosus with intestinal perforation: A case report

Systemic lupus erythematosus with intestinal perforation: A case report

Two Cases of Chronic Intestinal Pseudo-obstruction: A Comparison of Staining Characteristics of Enteric Visceral Myopathy With Hirschsprung Disease

The great masquerader of malignancy: chronic intestinal pseudo-obstruction

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