Berard, Roberta

Chédeville, Gaëlle

Saint-Martin, Christine

Scuccimarri, Rosie

English

ROBERTA BERARD

GAËLLE CHÉDEVILLE

CHRISTINE SAINT-MARTIN

ROSIE SCUCCIMARRI

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The Journal of Rheumatology

Benign Pneumatosis Intestinalis in a Patient with Juvenile Dermatomyositis

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Western University Scholarship@Western Paediatrics Publications Paediatrics Department 11-1-2010 Benign pneumatosis intestinalis in a patient with juvenile dermatomyositis Roberta Berard Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants, roberta.berard@lhsc.on.ca Gaëlle Chédeville Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants Christine Saint-Martin Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants Rosie Scuccimarri Centre Universitaire de Santé McGill, Hôpital de Montreal Pour Enfants Follow this and additional works at: https://ir.lib.uwo.ca/paedpub Citation of this paper: Berard, Roberta; Chédeville, Gaëlle; Saint-Martin, Christine; and Scuccimarri, Rosie, "Benign pneumatosis intestinalis in a patient with juvenile dermatomyositis" (2010). Paediatrics Publications. 801. https://ir.lib.uwo.ca/paedpub/801 2442 The Journal of Rheumatology 2010; 37:11Personal non-commercial use only. The Journal of Rheumatology Copyright © 2010. All rights reserved.Benign Pneumatosis Intestinalis in a Patient with JuvenileDermatomyositisTo the Editor:Juvenile dermatomyositis (JDM) is an autoimmune vasculopathy involvingprimarily the proximal muscles and skin but can involve other organ sys-tems, particularly the gastrointestinal (GI) tract1. In the presence of cuta-neous vasculitic ulcerations, one needs to monitor for signs and symptomssuggestive of GI vasculopathy2, which can lead to bowel ischemia, necro-sis, ulceration, and perforation1,2,3,4,5. The presence of gas in the bowelwall, also referred to as pneumatosis intestinalis (PI), is an ominous find-ing when it is due to a GI vasculopathy3. PI is a radiographic finding thatis associated with both benign and life-threatening causes. The benign vari-ant of PI has been described in relation to immunosuppressive therapy6 andto JDM itself7. We describe a child with JDM complicated by cutaneousulcerations and benign PI.An 8-year-old girl, following a 1-month history of rash and fatigue, wasdiagnosed with JDM based on the presence of Gottron sign, heliotroperash, symmetric proximal muscle weakness, elevated serum muscleenzymes [creatine kinase (CK) 533 U/l (normal 44–189); lactate dehydro-genase 442 U/l (normal 142–261); aldolase 9.3 U/l (normal 0–7.6)] andfindings consistent with myositis on magnetic resonance imaging of theproximal hip and shoulder girdle muscles. Antinuclear and anti-Jo1 anti-bodies were negative. The initial Childhood Myositis Assessment Scale8(CMAS) was 44/52. Her nailfold capillaries were normal. Initial treatmentincluded high-dose oral corticosteroids and methotrexate (MTX).Three weeks after starting therapy, she presented with increased weak-ness, a vasculitic ulcer on the left upper eyelid and in the left nare associ-ated with an increase in CK (1170 U/l); however, the other muscle enzymesremained unchanged. She was treated with intravenous pulse methylpred-nisolone once daily for 3 days. When reassessed a few days later for acough, a chest radiograph showed free air under the right hemidiaphragm(Figure 1). She was asymptomatic. The eyelid and nare ulcers were heal-ing, with no new cutaneous ulcerations. Her muscle strength (CMAS46/52) had improved. Her abdomen was slightly distended but soft, non-tender with present bowel sounds. A pneumoperitoneum and extensive PI,primarily involving the large bowel, were confirmed by computed tomo-graphy (CT) of the abdomen (Figures 2 and 3). No associated signs ofbowel wall thickening, fat stranding, or abdominal free fluid were present.The patient was admitted to hospital for intravenous antibiotics and ashort period of bowel rest. Additional investigations included a normalcomplete blood cell count, blood gas, lactic acid, fibrinogen, and coagula-tion profile. The muscle enzymes had improved with normalization of CK(52 U/l). D-dimers were negative; however, the vonWillebrand factor anti-gen was elevated at 4.9 U/ml (normal 0.5–1.6). Anticardiolipin antibodiesand lupus anticoagulant were negative. Family history was negative forvascular diseases. There was no escalation in immunosuppressive therapy.She remained asymptomatic and was discharged after 10 days withoutadverse sequelae. An abdominal radiograph performed 6 weeks laterdemonstrated persistence of PI with resolution of the pneumoperitoneum.The rest of her course was uncomplicated. Prednisone was discontinued 14months from diagnosis and she continues on a tapering dose of MTX.PI was first described in 17549. It is defined as the presence of gas with-in the bowel wall10 and is a radiographic finding that can be seen withmany diagnostic entities11. The prevalence of PI is not known; however,this seems to be increasing with more frequent use of CT and immunosup-pressive therapy10,12.Benign causes of PI have been described in relation to systemic illness(collagen vascular disease, chronic pulmonary disease), medications, andGI procedures10,12. In 1973, Borns and Johnston suggested a link betweenPI and immunosuppressive therapy, particularly corticosteroids6. Onpathologic specimens, air was discovered in the Peyer patches of the intes-tine13. This led to the hypothesis that steroids or stress cause lymphoiddepletion and thus atrophy of the Peyer patches, resulting in alteration ofthe submucosal structural integrity, which permits intraluminal gas to dis-sect into the noninflamed bowel wall6,10,11,12.The life-threatening causes of PI are those associated with bowelnecrosis. In JDM, bowel necrosis occurs as a consequence of the vascu-lopathy1. A review in 1998 identified 6 patients in the literature with PIassociated with JDM4; 4 had a benign clinical course similar to our patient,1 had a life-threatening course, and 1 patient died4. In JDM, it is impera-tive to promptly identify a GI vasculopathy, as this can be fatal.Cutaneous ulcerative disease in JDM may be associated with morewidespread vasculitis and thus PI seen in this context would more com-Figure 1. Free air under the right hemidiaphragm (arrow). www.jrheum.orgDownloaded on August 8, 2022 from 2443Lettermonly be secondary to bowel necrosis. Despite the cutaneous ulcerationsin our patient, she did not have life-threatening PI. Pneumoperitoneum, asobserved in our patient, can also occur in benign PI, which in this contextis usually the consequence of the rupture of a subserosal cyst rather than atrue perforation10,12. Worrisome features on imaging include signs ofbowel wall inflammation or ischemia manifesting as bowel wall thicken-ing, free fluid, or portal gas12, which were absent in our case.The management of PI depends on the underlying etiology and mostimportantly on the clinical condition of the child. In the absence of sepsisor peritonitis, many children can be managed conservatively with antibiot-ic coverage and bowel rest10. The presence of PI alone is not an indicationfor surgical intervention or escalation of medical therapy. However, in chil-dren with a clinical deterioration, surgical intervention may be warranted10.Our patient was asymptomatic and did not have the more worrisomefeatures on imaging. Her presentation and clinical course, in our opinion,were consistent with benign PI, which we presumed was secondary toimmunosuppressive therapy. However, we could not exclude the presenceof a mild vasculopathy resulting in loss of mucosal integrity, given the ele-vated von Willebrand factor antigen. Both the benign and life-threateningvariants of PI should be considered, particularly in patients at high risk forbowel necrosis.Increased awareness of the benign form of PI in combination with clin-ical history, physical examination, and laboratory and radiographic evalu-ations will allow proper identification of this entity, which may preventunnecessary escalation in medical therapy and/or surgical intervention.ROBERTA BERARD, MD; GAËLLE CHÉDEVILLE, MD, Department ofPediatrics, Division of Rheumatology; CHRISTINE SAINT-MARTIN,MD, Department of Medical Imaging; ROSIE SCUCCIMARRI, MD,Department of Pediatrics, Division of Rheumatology, Montreal Children’sHospital, McGill University Health Centre, Montreal, Quebec, Canada.Address correspondence to Dr. Scuccimarri;E-mail: rosie.scuccimarri@muhc.mcgill.caREFERENCES1. Feldman BM, Rider LG, Reed AM, Pachman LM. Juveniledermatomyositis and other idiopathic inflammatory myopathies ofchildhood. Lancet 2008;371:2201-12.2. Meneghel A, Zulian F, Martini G, Guariso G. Ischemic ulcerativecolitis in juvenile dermatomyositis. J Pediatr Gastroenterol Nutr2009;49:549.3. Fischer TJ, Cipel L, Stiehm ER. Pneumatosis intestinalis associatedwith fatal childhood dermatomyositis. Pediatrics 1978;61:127-30.4. Stefanski JC, Shetty AK. Abdominal pain in a girl with juveniledermatomyositis. Clin Pediatr 1998;37:561-4.5. Mamyrova G, Kleiner DE, James-Newton L, Shaham B, MillerFW, Riser LG. Late-onset gastrointestinal pain in juveniledermatomyositis as a manifestation of ischemic ulceration fromchronic endarteropathy. Arthritis Rheum 2007;57:881-4.6. Borns PF, Johnston TA. Indolent pneumatosis of the bowel wallassociated with immune suppressive therapy. Ann Radiol1973;16:163-6.7. Braunstein EM, White SJ. Pneumatosis intestinalis indermatomyositis. Br J Radiol 1980;53:1011-2.8. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, LindsleyCB, Perez MD, et al. Validation and clinical significance of theChildhood Myositis Assessment Scale for assessment of musclefunction in the juvenile idiopathic inflammatory myopathies.Arthritis Rheum 2004;50:1595-603.9. Koss LG. Abdominal gas cysts (pneumatosis cystoidesintestinorum hominis): an analysis with a report of a case and acritical review of the literature. Arch Pathol 1952;53:523-49.10. Fenton LZ, Buonomo C. Benign pneumatosis in children. PediatrRadiol 2000;30:786-93.11. Pear BL. Pneumatosis intestinalis: a review. RadiologyFigure 2. Infused CT scan of the abdomen, coronal view, showing exten-sive pneumatosis intestinalis in the large colon.Figure 3. Infused CT scan of the abdomen, axial view, showing circum-ferential pneumatosis intestinalis of the transverse colon.Personal non-commercial use only. The Journal of Rheumatology Copyright © 2010. All rights reserved. www.jrheum.orgDownloaded on August 8, 2022 from 2444 The Journal of Rheumatology 2010; 37:11Personal non-commercial use only. The Journal of Rheumatology Copyright © 2010. All rights reserved.1998;207:13-9.12. Ho LM, Paulson EK, Thompson WM. Pneumatosis intestinalis inthe adult: benign to life-threatening causes. AJR Am J Roentgenol2007;188:1604-13.13. Smith BH, Welter LH. Pneumatosis intestinalis. Am J Clin Pathol1967;48:455-65.J Rheumatol 2010;37:11; doi:10.3899/jrheum.100537 www.jrheum.orgDownloaded on August 8, 2022 from 

Benign pneumatosis intestinalis in a patient with juvenile dermatomyositis

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Benign pneumatosis intestinalis in a patient with juvenile dermatomyositis

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