eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Vasculitis and Thrombophlebitis: Follow-up

Author: Christine Hom, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College
Contributor Information and Disclosures

Updated: Nov 23, 2009

Follow-up

Further Outpatient Care

  • A pediatric rheumatologist should provide follow-up care for patients with vasculitis to monitor the appearance of new manifestations that may indicate a different diagnosis.
  • Patients with Henoch-Schönlein purpura should have urinalysis performed monthly for 6-12 months as a screen for late-onset nephritis.13 A meta-analysis examined therapies used to prevent or treat kidney disease in Henoch-Schönlein purpura. The results of 10 randomized controlled trials (1230 children) were examined. No significant difference was noted in the risk of persistent kidney disease at 6 months and 12 months in children given prednisone for 14-28 days after presentation compared with placebo or supportive treatment. In children with severe kidney disease, no significant difference was observed between cyclophosphamide and supportive treatment. No difference was noted between children given cyclosporin and children given methylprednisolone. The authors concluded that trials for interventions in kidney outcomes in children with Henoch-Schönlein purpura are sparse and no benefit was noted with prednisone.
  • CBC count and erythrocyte sedimentation rate (ESR) at a minimum may be used as markers of disease activity. For patients with elevated cytoplasmic pattern ANCA (c-ANCA), titers may normalize during periods of disease control and increase with disease activity. Serial ANCA titers have been used to measure disease activity, with limited success. An acute rise in ANCA titer suggests clinical activity; persistent high titers are less helpful in identifying patients with active disease.

Inpatient & Outpatient Medications

  • Nonsteroidal anti-inflammatory drugs for myalgias
  • Corticosteroid in tapering doses as tolerated, once adequate control of disease achieved
  • Maintenance daily oral or monthly intravenous Cytoxan for renal, CNS, or pulmonary vasculitis
  • Tumor necrosis factor (TNF) inhibition or anti–B-cell therapies for patients refractory to or intolerant of cyclophosphamide: TNF inhibition with infliximab has been helpful in treating patients with Takayasu arteritis; however, TNF inhibition with etanercept was no better than placebo for patients with Wegener granulomatosis
  • Antihypertensive therapy as needed
  • Oral anticoagulant therapy as maintenance with documented thrombus and evidence for underlying hypercoagulable state

Transfer

  • Transfer patients to a tertiary care center for undiagnosed systemic vasculitis with life-threatening complications.
  • Transfer patients with a clinical picture consistent with pulmonary, renal, or CNS involvement.

Deterrence/Prevention

  • Prevention of complications of vasculitis is achieved by early diagnosis and aggressive treatment, where indicated, of both systemic (idiopathic) and secondary vasculitis. A high index of suspicion is essential for the diagnosis of this group of diseases.
  • Immunization against hepatitis B is the current standard of care in the United States, and it may decrease the prevalence of hepatitis B–associated vasculitis over time.
  • Minimize catheter instrumentation.
  • Treat patients with a hypercoagulable state and a history of arterial or venous thrombosis with lifelong anticoagulation.

Complications

  • Renal insufficiency
  • Digital gangrene
  • Pulmonary hemorrhage
  • CNS infarction
  • Arterial or venous thrombosis
  • Subglottic stenosis
  • Death

Prognosis

  • Prognosis is directly related to the degree of end-organ involvement.

Patient Education

  • Advise patients requiring corticosteroids of Cushingoid symptoms, including, but not limited to, hypertension, glucose intolerance, weight gain, hirsutism, mood swings, osteonecrosis, and growth disturbance.
  • Advise patients requiring immunosuppression of the increased risk of infections, myelosuppression, and teratogenic and oncogenic potential, as well as any hepatic, pulmonary, cardiac, or renal toxicities.
  • Discuss use of adequate low-estrogen birth control with patients of childbearing age if warfarin is used; enoxaparin may be considered, but increased risk of osteoporosis is associated with long-term use.
  • Patients taking anticoagulants should not participate in contact sports.
  • For excellent patient education resources, visit eMedicine's Circulatory Problems Center. Also, see eMedicine's patient education article Leg Blood Clot.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose specific vasculitides requiring aggressive therapy (eg, Wegener granulomatosis)
  • Failure to diagnose vascular thrombosis: These patients should be on anticoagulation therapy early to prevent postphlebitic syndrome with incompetent venous return.

Special Concerns

  • Long-term outcome for most patients with Kawasaki disease is unknown; numerous reports detail patients experiencing early myocardial infarction, possibly due to residual endothelial injury.
  • Patients with Henoch-Schönlein purpura may be at increased risk for hypertension during later pregnancies.
 


More on Vasculitis and Thrombophlebitis

Overview: Vasculitis and Thrombophlebitis
Differential Diagnoses & Workup: Vasculitis and Thrombophlebitis
Treatment & Medication: Vasculitis and Thrombophlebitis
Follow-up: Vasculitis and Thrombophlebitis
Multimedia: Vasculitis and Thrombophlebitis
References
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References

  1. Jennette JC, Falk RJ, Andrassy K. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].

  2. Lie JT. Nomenclature and classification of vasculitis: plus ca change, plus c'est la meme chose. Arthritis Rheum. Feb 1994;37(2):181-6. [Medline].

  3. Esper F, Shapiro ED, Weibel C. Association between a novel human coronavirus and Kawasaki disease. J Infect Dis. Feb 15 2005;191(4):499-502. [Medline].

  4. Valentini RP, Smoyer WE, Sedman AB, et al. Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience. J Pediatr. Feb 1998;132(2):325-8. [Medline].

  5. [Guideline] Arend WP, Michel BA, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. Aug 1990;33(8):1129-34. [Medline].

  6. [Guideline] Fries JF, Hunder GG, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. Aug 1990;33(8):1135-6. [Medline].

  7. [Guideline] Hunder GG, Arend WP, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. Aug 1990;33(8):1065-7. [Medline].

  8. [Guideline] Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. Aug 1990;33(8):1101-7. [Medline].

  9. [Guideline] Lightfoot RW Jr, Michel BA, Bloch DA. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. Aug 1990;33(8):1088-93. [Medline].

  10. Ozen S, Besbas N, Saatci U. Diagnostic criteria for polyarteritis nodosa in childhood. J Pediatr. Feb 1992;120(2 Pt 1):206-9. [Medline].

  11. Andrew M, David M, Adams M. Venous thromboembolic complications (VTE) in children: first analyses of the Canadian Registry of VTE. Blood. Mar 1 1994;83(5):1251-7. [Medline].

  12. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med. Jan 27 2005;352(4):351-61. [Medline].

  13. [Best Evidence] Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev. Jul 8 2009;CD005128. [Medline].

  14. Abdulahad WH, Stegeman CA, van der Geld YM, Doornbos-van der Meer B, Limburg PC, Kallenberg CG. Functional defect of circulating regulatory CD4+ T cells in patients with Wegener's granulomatosis in remission. Arthritis Rheum. Jun 2007;56(6):2080-91. [Medline].

  15. [Best Evidence] Akikusa JD, Schneider R, Harvey EA, Hebert D, Thorner PS, Laxer RM. Clinical features and outcome of pediatric Wegener's granulomatosis. Arthritis Rheum. Jun 15 2007;57(5):837-44. [Medline].

  16. Bayrakci US, Topaloglu R, Soylemezoglu O, Kalyoncu M, Ozen S, Besbas N. Effect of early corticosteroid therapy on development of Henoch-Schönlein nephritis. J Nephrol. Jul-Aug 2007;20(4):406-9. [Medline].

  17. Bosch X, Guilabert A, Espinosa G, Mirapeix E. Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. JAMA. Aug 8 2007;298(6):655-69. [Medline].

  18. Breunis WB, Biezeveld MH, Geissler J, Kuipers IM, Lam J, Ottenkamp J. Polymorphisms in chemokine receptor genes and susceptibility to Kawasaki disease. Clin Exp Immunol. Oct 2007;150(1):83-90. [Medline].

  19. Butani L, Morgenstern BZ. Long-term outcome in children after Henoch-Schonlein purpura nephritis. Clin Pediatr (Phila). Jul 2007;46(6):505-11. [Medline].

  20. Dedeoglu F, Sundel RP. Vasculitis in children. Pediatr Clin North Am. Apr 2005;52(2):547-75, vii. [Medline].

  21. Dillon MJ. Childhood vasculitis. Lupus. 1998;7(4):259-65. [Medline].

  22. Dillon MJ, Ansell BM. Vasculitis in children and adolescents. Rheum Dis Clin North Am. Nov 1995;21(4):1115-36. [Medline].

  23. Ehrenforth S, Junker R, Koch HG. Multicentre evaluation of combined prothrombotic defects associated with thrombophilia in childhood. Childhood Thrombophilia Study Group. Eur J Pediatr. Dec 1999;158 Suppl 3:S97-104. [Medline].

  24. Guilpain P, Servettaz A, Goulvestre C, Barrieu S, Borderie D, Chereau C. Pathogenic effects of antimyeloperoxidase antibodies in patients with microscopic polyangiitis. Arthritis Rheum. Jul 2007;56(7):2455-63. [Medline].

  25. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. Nov 20 1997;337(21):1512-23. [Medline].

  26. Kandarpa K. Catheter-directed thrombolysis of peripheral arterial occlusions and deep vein thrombosis. Thromb Haemost. Aug 1999;82(2):987-96. [Medline].

  27. Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med. Jan 15 2006;173(2):180-7. [Medline].

  28. Knofler R, Siegert E, Lauterbach I, et al. Clinical importance of prothrombotic risk factors in pediatric patients with malignancy--impact of central venous lines. Eur J Pediatr. Dec 1999;158 Suppl 3:S147-50. [Medline].

  29. Lamprecht P, Till A, Steinmann J, Aries PM, Gross WL. Current State of Biologicals in the Management of Systemic Vasculitis. Ann N Y Acad Sci. Sep 2007;1110:261-270. [Medline].

  30. Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K, D'Cruz D. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford). Oct 2007;46(10):1615-6. [Medline].

  31. Laupland KB, Dele Davies H. Epidemiology, etiology, and management of Kawasaki disease: state of the art. Pediatr Cardiol. May-Jun 1999;20(3):177-83. [Medline].

  32. Manco-Johnson MJ. Disorders of hemostasis in childhood: risk factors for venous thromboembolism. Thromb Haemost. Jul 1997;78(1):710-4. [Medline].

  33. McCrindle BW, McIntyre S, Kim C, Lin T, Adeli K. Are patients after Kawasaki disease at increased risk for accelerated atherosclerosis?. J Pediatr. Sep 2007;151(3):244-8, 248.e1. [Medline].

  34. Mitchell L, Hoogendoorn H, Giles AR, Vegh P, Andrew M. Increased endogenous thrombin generation in children with acute lymphoblastic leukemia: risk of thrombotic complications in L'Asparaginase-induced antithrombin III deficiency. Blood. Jan 15 1994;83(2):386-91. [Medline].

  35. Mukhtyar C, Flossmann O, Hellmich B, Bacon PA, Cid MC, Cohen Tervaert JW. Outcomes from studies of Antineutrophil Cytoplasm Antibody Associated Vasculitis: a systematic review by the EULAR Systemic Vasculitis Task Force. Ann Rheum Dis. Oct 2 2007;[Medline].

  36. [Best Evidence] Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med. Feb 15 2007;356(7):663-75. [Medline].

  37. Newburger JW, Takahashi M, Gerber MA. 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. Oct 26 2004;110(17):2747-71. [Medline].

  38. Nowak-Gottl U, Dubbers A, Kececioglu D, et al. Factor V Leiden, protein C, and lipoprotein (a) in catheter-related thrombosis in childhood: a prospective study. J Pediatr. Oct 1997;131(4):608-12. [Medline].

  39. Nuss R, Hays T, Manco-Johnson M. Childhood thrombosis. Pediatrics. Aug 1995;96(2 Pt 1):291-4. [Medline].

  40. Peco-Antic A, Bonaci-Nikolic B, Basta-Jovanovic G. Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol. Oct 27 2005;[Medline].

  41. Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol. Feb 2007;13(1):25-8. [Medline].

  42. Ronkainen J, Ala-Houhala M, Autio-Harmainen H, Jahnukainen T, Koskimies O, Merenmies J. Long-term outcome 19 years after childhood IgA nephritis: a retrospective cohort study. Pediatr Nephrol. Sep 2006;21(9):1266-73. [Medline].

  43. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. Oct 9 2007;[Medline].

  44. Shimizu C, Shike H, Baker SC. Human Coronavirus NL63 Is Not Detected in the Respiratory Tracts of Children with Acute Kawasaki Disease. J Infect Dis. Nov 15 2005;192(10):1767-71. [Medline].

  45. Streif W, Andrew ME. Venous thromboembolic events in pediatric patients. Diagnosis and management. Hematol Oncol Clin North Am. Dec 1998;12(6):1283-312, vii. [Medline].

  46. Villa-Forte A, Clark TM, Gomes M, Carey J, Mascha E, Karafa MT. Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore). Sep 2007;86(5):269-77. [Medline].

  47. Walsh M, Jayne D. Rituximab in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis and systemic lupus erythematosus: past, present and future. Kidney Int. Sep 2007;72(6):676-82. [Medline].

  48. Williams A, Nagy M, Wingate J, et al. Lemierre syndrome: a complication of acute pharyngitis. Int J Pediatr Otorhinolaryngol. Sep 15 1998;45(1):51-7. [Medline].

  49. Zoller B, Garcia de Frutos P, Hillarp A. Thrombophilia as a multigenic disease. Haematologica. Jan 1999;84(1):59-70. [Medline].

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Further Reading

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Keywords

vasculitis, thrombophlebitis, superficial venous thrombosis, Henoch-Schönlein purpura, Kawasaki disease, infantile polyarteritis nodosa, polyarteritis nodosa, Takayasu's arteritis, Takayasu arteritis, temporal arteritis, Wegener granulomatosis, treatment, diagnosis, symptoms

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Contributor Information and Disclosures

Author

Christine Hom, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Rheumatology, New York Medical College
Christine Hom, MD is a member of the following medical societies: American College of Rheumatology, American Medical Association, and Arthritis Foundation
Disclosure: Nothing to disclose.

Medical Editor

Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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