Published online July 31, 2006
PEDIATRICS Vol. 118 No. 3 September 2006, pp. e904-e906 (doi:10.1542/10.1542/peds.2005-3111)

This Article Abstract Full Text (PDF) Submit a response View responses Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Preud'Homme, D. L. Articles by Mezoff, A. G. Search for Related Content PubMed PubMed Citation Articles by Preud'Homme, D. L. Articles by Mezoff, A. G. Related Collections Gastrointestinal Tract Social Bookmarking                         What's this?

EXPERIENCE & REASON

Use of Wireless Capsule Endoscopy in the Management of Severe Henoch-Schonlein Purpura

Daniel L. Preud'Homme, MD^a,b, Sonia Michail, MD^a,b, Cathy Hodges, RN^a, Tonya Milliken, RN^a and Adam G. Mezoff, MD^a,b

^a Gastroenterology and Nutrition, Dayton Children's Hospital, Dayton, Ohio
^b Department of Pediatrics, Boonshoft School of Medicine, Wright State University, Dayton, Ohio

ABSTRACT

Henoch-Schonlein purpura is a multisystem vasculitis that primarily affects children. Characteristic symptoms include purpura of the lower extremities and buttocks, abdominal pain, arthralgias, and hematuria. Gastrointestinal bleeding occurs in 50% of children and, although often self-limiting, can be significant. Wireless capsule endoscopy has been found to be safe and effective for children over 10 years of age. It is now the preferred imaging modality for evaluating gastrointestinal bleeding from the small intestine. Here we report an unusual case of chronic Henoch-Schonlein purpura vasculitis, primarily affecting the gastrointestinal tract, in which wireless capsule endoscopy was helpful in evaluating and directing treatment options.

---

Key Words: gastrointestinal bleeding • Henoch-Schonlein syndrome

Abbreviations: HSP, Henoch-Schonlein purpura • WCE, wireless capsule endoscopy

Henoch-Schonlein purpura (HSP) is a multisystem vasculitis that primarily affects children. Characteristic symptoms include purpura of the lower extremities and buttocks, abdominal pain, arthralgias, and hematuria. Gastrointestinal bleeding occurs in 50% of children and, although often self-limiting, can be significant. Wireless capsule endoscopy (WCE) has been found to be safe and effective for children over 10 years of age.¹ It is now the preferred imaging modality for evaluating gastrointestinal bleeding from the small intestine. Here we report an unusual case of chronic HSP vasculitis, which primarily affects the gastrointestinal tract, in which WCE was helpful in evaluating and directing treatment options.

CASE REPORT

A 12-year-old boy presented with a 3-month history of intermittent purpuric skin rash on the lower and upper extremities, joint pain, and abdominal pain. The patient developed hematochezia and was hospitalized for additional evaluation. Physical examination revealed a purpuric rash on the arms, buttocks, knees, legs, and feet. His ankles and knees were swollen and tender.

Urinalysis demonstrated microscopic hematuria. His hemoglobin (12.6 g/dL), creatinine/serum urea nitrogen (12 mg/dL/0.6 mg/dL), albumin (4.8 g/dL), and C3/C4 levels and the erythrocyte sedimentation rate (10 mm/hour) were normal. Methyl-prednisolone (20 mg every 6 hours) was begun for 3 days and then switched to oral prednisone (80 mg per day). The patient's hematochezia and abdominal pain persisted. A series of abdominal radiographs and computed tomography scan showed ileal and cecal thickening without evidence of intussusception or perforation. Esophagogastroduodenoscopy and colonoscopy showed edematous and hyperemic gastric mucosa and punctated hemorrhages in the duodenal bulb. Diffuse areas of severe edema, ulceration, and necrosis were noted in the rest of the duodenum, jejunum, ileum, and colon. Skin biopsy demonstrated the presence of a vasculitis and was consistent with a diagnosis of HSP. Skin rash and abdominal pain improved, and the patient was subsequently discharged from the hospital 1 week after admission on 80 mg of oral prednisone.

WCE was performed 3 weeks after hospitalization to evaluate the extent of the small intestinal involvement of HSP in hopes of weaning his steroids. This study demonstrated diffuse and extensive ulcerations throughout (Fig 1). Therefore, the current steroid dose was maintained; however, 3 weeks after the WCE was obtained, the patient developed severe complications of his steroid therapy, including vertebral fractures, cushingoid appearance, and hypertension. Cyclophosphamide was then added to his daily prednisone regime, with improved control of his gastrointestinal symptoms.

View larger version (58K): [in this window] [in a new window]   FIGURE 1 WCE of the jejunum in a patient with HSP demonstrating severe erythema and edema of intestinal folds.

 
A second WCE was performed 4 months after the first study to evaluate treatment efficacy with cyclophosphamide and the feasibility of discontinuing steroid therapy. The second WCE (Fig 2) showed complete resolution of the previously identified lesions. The patient was subsequently able to tolerate a slow tapering of his steroid therapy, although he continued to have intermittent flares of HSP.

View larger version (92K): [in this window] [in a new window]   FIGURE 2 Follow-up WCE of the jejunum demonstrating resolution of the previous erythema and edema.

 
Fifteen months after the original presentation the patient is tolerating a 1-mg daily dose of prednisone, with maintenance therapy of mycophenolate mofetil (Cellcept) in place of cyclophosphamide.

DISCUSSION

HSP is a systemic immunoglobulin A–mediated leukocytoclastic vasculitis that affects mostly children.² Characteristic symptoms of HSP include purpura of the lower extremities and buttocks, abdominal pain, arthralgias, and hematuria.^3,4 Gastrointestinal complications of HSP include appendicitis,⁵ intussusceptions,⁶ bowel ischemia, perforation,³ and gastrointestinal bleeding.⁷ Gastrointestinal bleeding occurs in 50% of children affected by HSP, and although usually self-limiting, massive bleeding may occur.⁸ The systemic vasculitis of HSP also affects the kidney and can occasionally cause chronic nephritis, requiring long-term steroid or immunosuppressive therapy with cyclophosphamide,⁹ although the long-term benefits of steroid therapy for this condition is unclear.¹⁰ Although in our patient HSP resulted in a protracted vasculitis, most cases reported resolve without the need for endoscopic evaluation.⁶ Our patient presented a complex clinical case because of (1) protracted course, (2) multiple recurrences of symptoms including microscopic and gross hematochezia or melena, (3) steroid dependency, and (4) severe steroid-related complications. WCE first identified the severity of the small intestinal disease, explaining symptoms of abdominal pain as well as significant bleeding. The discovery of these problems led to treatment with cyclophosphamide despite mild evidence of kidney involvement (normal albumin, minimal proteinuria, and microscopic hematuria), the usual indication for this aggressive mode of treatment.⁸ After 3 months of treatment with cyclophosphamide and then mycophenolate mofetil, steroids were almost completely weaned.

CONCLUSIONS

The use of WCE in this severe case of HSP helped to identify the intensity and extent of the gastrointestinal involvement and confirmed the importance of cyclophosphamide therapy in improving the gastrointestinal lesions. WCE must be used judiciously to avoid capsule retention in any disease process that can lead to bowel stricture or edema; however, it can be a helpful tool for evaluating severe cases of HSP with gastrointestinal involvement. Although a previous adult study demonstrates HSP lesions with WCE,¹¹ our case is the first report of WCE facilitating treatment decisions with this disorder.

FOOTNOTES

Accepted Apr 10, 2006.

Address correspondence to Adam G. Mezoff, MD, Gastroenterology and Nutrition, Dayton Children’s Hospital, One Children’s Plaza, Dayton, OH 45404. E-mail: amezoff{at}aol.com

The authors have indicated they have no financial relationships relevant to this article to disclose.

REFERENCES

1. Guilhon de Araujo Sant'Anna AM, Dubois J, Miron MC, Seidman EG. Wireless capsule endoscopy for obscure small-bowel disorders: final results of the first pediatric controlled trial. Clin Gastroenterol Hepatol. 2005;3 :264 –270[CrossRef][Web of Science][Medline]
2. Gunasekaran TS. Henoch-Schonlein purpura: what does the "rash" look like in the gastrointestinal mucosa? Pediatr Dermatol. 1997;14 :437 –440[Web of Science][Medline]
3. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337 :1512 –1523[Free Full Text]
4. Saulsbury FT. Henoch-Schonlein purpura. Curr Opin Rheumatol. 2001;13 :35 –40[CrossRef][Web of Science][Medline]
5. Binstadt BA, Fleegler EW. Perforated appendicitis in a child with Henoch-Schonlein purpura. J Pediatr Surg. 2005;40 :E24 –E27[CrossRef][Medline]
6. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schonlein purpura. J Paediatr Child Health. 1998;34 :405 –409[CrossRef][Web of Science][Medline]
7. Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schonlein purpura: a review of 261 patients. Acta Paediatr. 2004;93 :1427 –1431[CrossRef][Web of Science][Medline]
8. Katz S, Borst M, Seekri I, Grosfeld J. Surgical evaluation of Henoch-Schonlein purpura: experience with 110 children. Arch Surg. 1991;126 :849 –854[Abstract/Free Full Text]
9. Flynn J, Smoyer W, Bunchman T, Kershaw D, Sedman A. Treatment of Henoch-Schonlein purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol. 2001;21 :128 –133[CrossRef][Web of Science][Medline]
10. Haroon M. Should children with Henoch-Schonlein purpura and abdominal pain be treated with steroids? Arch Dis Child. 2005;90 :1196 –1198[Free Full Text]
11. Skogestad E. Capsule endoscopy in Henoch-Schonlein purpura. Endoscopy. 2005;37 :189[CrossRef][Medline]

---

PEDIATRICS (ISSN 1098-4275). ©2006 by the American Academy of Pediatrics

CiteULike    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

eLetters:

Read all eLetters

Is capsule endoscopy necessary in the evaluation of gastrointestinal Henoch-Schonlein vasculitis?

Jae Il Shin, et al.

Pediatrics Online, 23 Aug 2006 [Full text]

This Article Abstract Full Text (PDF) Submit a response View responses Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Preud'Homme, D. L. Articles by Mezoff, A. G. Search for Related Content PubMed PubMed Citation Articles by Preud'Homme, D. L. Articles by Mezoff, A. G. Related Collections Gastrointestinal Tract Social Bookmarking                         What's this?