Top

Pediatric Rheumatology

Published in:

Open Access 01-12-2018 | Case Report

A case of Henoch-Schonlein Purpura with dilated coronary arteries

Authors: Jessica L. Bloom, Jeffrey R. Darst, Lori Prok, Jennifer B. Soep

Published in: Pediatric Rheumatology | Issue 1/2018

Abstract

Background

Henoch-Schonlein Purpura (HSP) is one of the most common vasculitides of childhood, with 10–20 cases per 100,000 children. It frequently occurs following an infectious trigger and involves IgA and C3 deposition in small vessel walls. HSP is characterized by palpable purpura plus IgA deposition on biopsy, arthritis/arthralgia, renal involvement (hematuria and/or proteinuria), and/or abdominal pain. It is not generally recognized as a cause of dilated coronary arteries.

Case presentation

We describe the first reported case of HSP presenting with dilated coronary arteries. This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP. An echocardiogram revealed coronary dilation, including the left main (5.32 mm, Z score + 4.25) and left anterior descending (LAD) (3.51 mm, Z score + 2.64) coronary arteries. He received high dose aspirin, IVIG, and infliximab with normalization of the LAD. Skin biopsy revealed leukocytoclastic vasculitis with positive IgA staining. He was Rhinovirus/Enterovirus positive with Group A Streptococcus on throat culture.

Conclusion

Cardiac findings, while rare, can exist in HSP. Coronary dilation appeared to respond to our hospital protocol’s Kawasaki Disease (KD) therapy, possibly indicating an overlap in HSP and KD pathophysiology. This case, along with prior reports of dilated coronaries in systemic juvenile idiopathic arthritis (SJIA), highlights the importance of considering other sources of systemic inflammation, in addition to KD, when coronary dilation is identified. The appropriate therapy, follow-up, and prognosis for our patient are not clear, as further studies are needed to determine the natural course of these findings.

Petty RE, Lindsley CB, et al. Cassidys textbook of pediatric rheumatology. 7th ed. Philadelphia: Elsevier; 2016.

Tizard EJ, Hamilton-Ayres MJJ. Henoch Schonlein purpura. Arch Dis Child Educ Pract. 2008;93(1):1–8. https://doi.org/10.1136/adc.2004.066035.CrossRef

Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. 2007;369(9566):976–8. https://doi.org/10.1016/s0140-6736(07)60474-7.CrossRefPubMed

Ozen S. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2005;65(7):936–41. https://doi.org/10.1136/ard.2005.046300.CrossRefPubMedPubMedCentral

Soliman M, Laxer R, et al. Imaging of systemic vasculitis in childhood. Pediatr Radiol. 2015;45(8):1110–25. https://doi.org/10.1007/s00247-015-3339-3.CrossRefPubMed

Kang Y, Park J-S, et al. Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein Purpura. J Korean Med Sci. 2014;29(2):198. https://doi.org/10.3346/jkms.2014.29.2.198.CrossRefPubMedPubMedCentral

Pillebout E. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13(5):1271–8. https://doi.org/10.1097/01.asn.0000013883.99976.22.CrossRefPubMed

Kereiakes D, Ports T, et al. Endomyocardial biopsy in Henouch-Schonlein purpura. Am Heart J. 1984;107(2):382–5.CrossRefPubMed

Carmichael P, Brun E, et al. A fatal case of bowel and cardiac involvement in Henoch-Schonlein purpura. Nephrol Dial Transplant. 2002;17:497–9.CrossRefPubMed

10.

Satoh M, Mikuniya A, et al. A case of Schonlein-Henoch Purpura with myocardial complications. Kokyu To Junkan. 1991;39(3):273–7.PubMed

11.

Agraharkar M, Gokahle S, et al. Cardiopulmonary Manifestations of Henoch-Schonlein Purpura. Am J Kidney Dis. 2000;35(2):319–22.CrossRefPubMed

12.

Osman A, Mccreery CJ. Cardiac Vasculitis in Henoch-Schonlein Purpura. Circulation. 2000;101(5) https://doi.org/10.1161/01.cir.101.5.e69.

13.

Lecutier MA. A case of the Schonlein-Henoch syndrome with Mycocardial necrosis. J Clin Path. 1952;5:336–8.CrossRefPubMed

14.

Polizzotto MN, Gibbs SDJ, et al. Cardiac involvement in Henoch-Schonlein purpura. Intern Med J. 2006;36:328–31.CrossRefPubMed

15.

Lutz H, Ackermann T, et al. Henoch-Schonlein Purpura complicated by cardiac involvement: case report and review of the literature. Am J Kidney Dis. 2009;54(5):e9–e15.CrossRefPubMed

16.

Imai T, Matsumoto S. Anaphylacatoid Purpura with cardiac involvement. Arch Dis Child. 1970;45(243):727–9.CrossRefPubMedPubMedCentral

17.

Bellantoni A, Presti P, et al. Un caso pediatrico di porpora di Schoenlein-Henoch con segni clinici, sierologici ed elettrocardiografici di danno miocardico. G Ital Cardiol. 2013;14(9):622–5.

18.

Hayakawa K, Shiohara T. Two cases of Henoch-Schonlein Purpura with transient myocardial ischemia. Acta Derm Venereol. 2003;83:393–4.CrossRefPubMed

19.

Abdel-Hadi O, Greenstone MA, et al. Myocardial infarction - a rare complication in Henoch-Schonlein purpura. Postgrad Med J. 1981;57(668):390–2. https://doi.org/10.1136/pgmj.57.668.390.CrossRefPubMedPubMedCentral

20.

Eleftheriadis D. Severe coronary artery disease in the setting of Henoch–Schoenlein purpura. Int J Cardiol. 2007;118(2):262–3. https://doi.org/10.1016/j.ijcard.2006.07.023.CrossRefPubMed

21.

Canpolat U, Yorgun H, et al. Myocardial infarction due to coronary thrombosis in a patient with Henoch–Schönlein purpura. Herz. 2012;37(7):801–3. https://doi.org/10.1007/s00059-012-3597-x.CrossRefPubMed

22.

Michas G, Grigoriou K, et al. A rare cause of myocarditis resulting in acute heart failure in the setting of Henoch–Schönlein purpura. Hell J Cardiol. 2017; https://doi.org/10.1016/j.hjc.2017.05.002.

23.

Mattoo TK, al-Mutair A, et al. Group A B-haemolitic streptococcal infection and Henoch-Schonlein Purpura with cardiac, renal and neurological complications. Ann Trop Paediatr. 1997;17(4):381–6.CrossRefPubMed

24.

Güven H, Özhan B, et al. A case of Henoch-Schönlein purpura and rheumatic carditis with complete atrioventricular block. Eur J Pediatr. 2006;165(6):395–7. https://doi.org/10.1007/s00431-006-0094-3.CrossRefPubMed

25.

Shin JI, Kim JH, et al. Cardiac manifestations of Henoch-Schoenlein purpura: IgA mediated vasculitis or rheumatic fever? Eur J Pediatr. 2006;166(6):627. https://doi.org/10.1007/s00431-006-0273-2.CrossRefPubMed

26.

Ocal B, Karademir S, et al. Acute rheumatic carditis in Henoch-Schonlein purpura. Int J Cardiol. 2000;74:97–8.CrossRefPubMed

27.

Eisenstein EM, Navon-Elkan P. Acute rheumatic fever associated with Henoch-Schonlein purpura: report of three cases and review of the literature. Acta Paediatr. 2002;91:1265–7.CrossRefPubMed

28.

Gairdner D. The Schonlein-Henoch syndrome (anaphylactois purpura). Q J Med. 1948;17(66):95–122.PubMed

29.

Hung JT, Yang YH, et al. Leukocytoclastic vasculitis with severe cardiac involvement in an infant: a case report. Clin Rheumatol. 2008;27:945–7.CrossRefPubMed

30.

James CA, Gonzalez I, et al. Severe mitral regurgitation in a child with Henoch-Schönlein Purpura and pulmonary hemorrhage. Glob Pediatr Health. 2017;4:1–4.

31.

Mao Y, Yin L, et al. Incidence and clinical features of paediatric vasculitis in eastern China: 14-year retrospective study, 1999–2013. J Int Med Res. 2016;44(3):710–7. https://doi.org/10.1177/0300060515621446.CrossRefPubMedPubMedCentral

32.

Binstadt BA. Coronary Artery Dilation Among Patients Presenting With Systemic-Onset Juvenile Idiopathic Arthritis. Pediatrics. 2005;116(1) https://doi.org/10.1542/peds.2004-2190.

33.

Newburger JW. Diagnosis, treatment, and long-term Management of Kawasaki Disease: a statement for health professionals from the committee on rheumatic fever, endocarditis and Kawasaki disease, council on cardiovascular disease in the young, american heart association. Circulation. 2004;110(17):2747–71. https://doi.org/10.1161/01.cir.0000145143.19711.78.CrossRefPubMed

34.

Miura M, Mochizuki T, et al. A case of Kawasaki disease accompanied by Henoch-Schönlein purpura. Clin Exp Rheumatol. 2004;22:377–8.PubMed

35.

Vedagiriswaran VV, Amperayani S, et al. A case of Henoch–Schönlein Purpura with Kawasaki disease. Indian J Pediatr. 2014;81(4):408–9. https://doi.org/10.1007/s12098-014-1395-6.CrossRefPubMed

36.

Heldrich FJ, Jodorkovsky RA, et al. Kawasaki syndrome: HUS and HSP complicating its course and management. Md Med J. 1987;36(9):764–6.PubMed

37.

Gámez-González LB, Rodríguez-Lozano A, et al. Enfermedad de Kawasaki y púrpura de Henoch-Schönlein: vasculitis frecuentes en una asociación infrecuente. Rev Alerg Mex. 2012;59(1):37–40.PubMed

38.

Porrello M, Gesu M, et al. Porpora di Schoenlein-Henoch dopo sindrome di Kawasaki: una predisposizione alle vasculiti? Ped Med Chir. 1998;20:71–3.

39.

Weiler T, Chelliah A, et al. Novel finding of coronary ectasia in a case of acute rheumatic fever. Case Rep Pediatr. 2013;2013:1–4. https://doi.org/10.1155/2013/674174.CrossRef

40.

Shin JI, Lee JS. Severe coronary artery disease in Henoch–Schoenlein purpura: superimposition of antiphospholipid or polyangitis overlap syndrome? Int J Cardiol. 2007;122(2):182–3. https://doi.org/10.1016/j.ijcard.2006.11.229.CrossRefPubMed

41.

Watanabe K, Abe H, et al. Polyangitis overlap syndrome: a fatal case combined with adult Henoch-Schnonlein purpura and polyarteritis nodosa. Pathol Int. 2003;53:569–73.CrossRefPubMed

Title: A case of Henoch-Schonlein Purpura with dilated coronary arteries
Authors: Jessica L. Bloom
Jeffrey R. Darst
Lori Prok
Jennifer B. Soep
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Pediatric Rheumatology / Issue 1/2018
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-018-0270-9

At a glance: The STEP trials

Springer Medicine

A case of Henoch-Schonlein Purpura with dilated coronary arteries

Abstract

Background

Case presentation

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Case presentation

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2018

Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany

Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database

Self-management needs of Irish adolescents with Juvenile Idiopathic Arthritis (JIA): how can a Canadian web-based programme meet these needs?

Disease burden and social impact of pediatric chronic nonbacterial osteomyelitis from the patient and family perspective

Pilot study comparing the childhood arthritis and rheumatology research alliance consensus treatment plans for induction therapy of juvenile proliferative lupus nephritis

Successful implementation of a clinical transition pathway for adolescents with juvenile-onset rheumatic and musculoskeletal diseases