Volume 6 Supplement 1

15thPaediatric Rheumatology European Society (PreS) Congress

Open Access

Gastroparesis associated with Juvenile Dermatomyositis

  • N Martin1,
  • J Davidson1,
  • H Harris2 and
  • P Gillett1
Pediatric Rheumatology20086(Suppl 1):P217

DOI: 10.1186/1546-0096-6-S1-P217

Published: 15 September 2008

Background

Gastrointestinal involvement is well recognised in juvenile dermatomyositis (JDM) Dysphagia due to pharyngeal and upper oesophageal dysmotility occurs in 33% of UK patients [1]. Gastrointestinal vasculitis with ulceration and intestinal perforation is also described. There is only one previous report highlighting gastric dysmotility associated with juvenile dermatomyositis [2].

Cases

We present two cases where gastroparesis was associated with JDM and improved with immunosuppression. Clinical features and investigations are summarised in table 1.
Table 1

Clinical features and investigations

 

Case 1

Case 2

Diagnosis

Juvenile dermatomyositis with overlap features

Juvenile dermatomyositis

Age at onset of gastric dysmotility symptoms

Seventeen

Thirteen

Age at diagnosis of connective tissue disorder

Eleven

Sixteen

Features of dermatomyositis

Rash, proximal weakness, raised muscle enzymes (CK 6926 units/l, ALT 126 units/l)

Rash, muscle pain, raised muscle enzymes (ALT 143 units/l LDH 1097 units); abnormal muscle MRI

Other clinical features

Sialadenitis

Pulmonary fibrosis

Polyarthritis

Gastrointestinal features

Vomiting

Abdominal pain

Weight loss

Required NJ feeding

Recurrent vomiting and abdominal pain

Autoantibodies

ANA, 1/640

RF, Anti Ro, La, Sm, RNP positive

ANA 1/640.

Gastrointestinal investigations

Gastric emptying study grossly delayed

H. Pylori negative

barium normal

Normal endoscopy

Coeliac screen negative

H. Pylori negative

Treatment

Prednisolone

Mycophenolate Mofetil, Intravenous immunoglobulin

Methotrexate

Response to treatment

Normal gastric emptying study

Reduced frequency and severity of symptoms

Conclusion

Gastric dysmotility may complicate JDM. In both these cases it presented with intractable vomiting and otherwise normal gastrointestinal investigations. Neither had evidence of otherwise active JDM at the time of onset of their vomiting but both responded to immunosuppression.

Authors’ Affiliations

(1)
Royal Hospital for Sick Children
(2)
Victoria Hospital

References

  1. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ, Pilkington CA: Juvenile Dermatomyositis Research Group. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)–clinical characteristics of children recruited within the first 5 yr. Rheumatology. 2006, 45 (10): 1255-1260. 10.1093/rheumatology/kel099.View ArticlePubMedGoogle Scholar
  2. Laskin BL, Choyke P, Keenan GF, Miller FW, Rider LG: Novel gastrointestinal tract manifestations in juvenile dermatomyositis. Journal of Pediatrics. 1999, 135 (3): 371-374. 10.1016/S0022-3476(99)70137-X.View ArticlePubMedGoogle Scholar

Copyright

© Martin et al; licensee BioMed Central Ltd. 2008

This article is published under license to BioMed Central Ltd.

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