Document Detail


Generalized megaviscera of lupus: refractory intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus.
MedLine Citation:
PMID:  19630114     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Dilated dysfunction involving multiple visceral organs has been reported in patients with systemic lupus erythematosus (SLE). Chronic intestinal pseudo-obstruction (CIPO) resulting from intestinal smooth muscle damage has presented in conjunction with ureterohydronephrosis and, more rarely, biliary dilatation (megacholedochus). While the molecular pathogenesis is largely unknown, observed histopathologic features include widespread myositis, myocyte necrosis in the intestinal muscularis propria with subsequent atrophy and fibrosis, preserved myenteric innervations and little vasculitis. High dose immunosuppression usually results in resolution of symptoms with recovery of smooth muscle function, indicative of an autoimmune etiology. We report a patient with SLE who presented with intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus, and present images that illustrate megaviscera simultaneously involving all 3 visceral organs. Since the co-manifestation of all 3 is unusual and has been reported only once previously, we have termed this rare clinical syndrome generalized megaviscera of lupus (GML). Although the SLE disease-activity parameters responded to aggressive immunomodulative therapy in our patient, clinical evidence of peristaltic dysfunction persisted in all involved viscera. This is a variation from the favorable outcomes reported previously in SLE patients with GML and we attribute this poor clinical outcome to disease severity and, most importantly, delayed clinical presentation. Since inflammation followed by atrophy and fibrosis are key aspects in the pathogenesis and natural history of GML, the poor response in our patient who presented late in the clinical course may be the result of 'burnt out' inflammation with irreversible end-stage fibrosis. Thus, early recognition and timely initiation of treatment may be the key to recover visceral peristaltic function in patients with GML.
Authors:
Frederick-D Park; Jeffrey-K Lee; Ganga-D Madduri; Pradipta Ghosh
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  World journal of gastroenterology : WJG     Volume:  15     ISSN:  2219-2840     ISO Abbreviation:  World J. Gastroenterol.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-24     Completed Date:  2009-09-28     Revised Date:  2014-05-20    
Medline Journal Info:
Nlm Unique ID:  100883448     Medline TA:  World J Gastroenterol     Country:  China    
Other Details:
Languages:  eng     Pagination:  3555-9     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Common Bile Duct / pathology*
Dilatation, Pathologic / etiology*,  pathology
Humans
Hydronephrosis / etiology*,  pathology
Immunosuppressive Agents / therapeutic use
Intestinal Pseudo-Obstruction / etiology*,  pathology
Lupus Erythematosus, Systemic / complications*,  drug therapy,  pathology
Middle Aged
Syndrome
Ureteral Obstruction / etiology*,  pathology
Grant Support
ID/Acronym/Agency:
DK080506/DK/NIDDK NIH HHS; T32 DK07202/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/Immunosuppressive Agents
Comments/Corrections

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