Skip to main content

Advertisement

SpringerLink
Log in

Hypocomplementemic urticarial vasculitis syndrome

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon immune complex-mediated entity characterized by urticaria with persistent acquired hypocomplementemia. First described in 1973, HUVS is associated with several systemic findings including leukocytoclastic vasculitis, severe angioedema, laryngeal edema, pulmonary involvement, arthritis, arthralgia, glomerulonephritis, and uveitis. These manifestations should be present for at least 6 months. Laboratory findings include low complement levels of classical pathway, namely C1q, C2, C3, and C4. The disease marker is the serum presence of anti-C1q antibodies. Treatment, based on disease severity, involves corticosteroids and other immunosuppressive agents that have demonstrated some success. Patients may have significant morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Wisnieski JJ: Urticarial vasculitis. Curr Opin Rheumatol 2000, 12:24–31.

    Article  CAS  PubMed  Google Scholar 

  2. Venzor J, Lee WL, Huston DP: Urticarial vasculitis. Clin Rev Allergy Immunol 2002, 23:201–216.

    Article  PubMed  Google Scholar 

  3. Khasnis A, Langford CA: Update on vasculitis. J Allergy Clin Immunol 2009, 123:1226–1236.

    Article  PubMed  Google Scholar 

  4. McDuffie FC, Sams WM Jr, Maldonado JE, et al.: Hypocomplementemia with cutaneous vasculitis and arthritis: possible immune complex syndrome. Mayo Clin Proc 1973, 48:340–348.

    CAS  PubMed  Google Scholar 

  5. Davis MD, Brewer JD: Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 2004, 24:183–213.

    Article  PubMed  Google Scholar 

  6. Jamison SC, Brierre S, Sweet J, de Boisblanc B: A case of precocious emphysema and lung cancer in a woman with a history of hypocomplementemic urticarial vasculitis. Chest 2008, 133:787–789.

    Article  PubMed  Google Scholar 

  7. Kallenberg CG: Anti-C1q antibodies. Autoimmunity Rev 2008, 8:612–615.

    Article  Google Scholar 

  8. Zeiss CR, Burch FX, Marder RJ, et al.: A hypocomplementemic vasculitic urticarial syndrome. Report of four new cases and the definition of the disease. Am J Med 1980, 68:867–875.

    Article  CAS  PubMed  Google Scholar 

  9. Wisnieski JJ, Baer AN, Christensen J, et al.: Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore) 1995, 74:24–41.

    Article  CAS  Google Scholar 

  10. Abel G, Agnello B: Complement deficiency and systemic lupus erythematosus. In Systemic Lupus Erythematosus. Edited by Lahita RG. Amsterdam: Elsevier, 2004:173–201.

    Chapter  Google Scholar 

  11. Schwartz HR, McDuffie FC, Black LF, et al.: Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease. Mayo Clin Proc 1982, 57:231–238.

    CAS  PubMed  Google Scholar 

  12. Marder RJ, Burch FX, Schmid FR, et al.: Low molecular weight C1q-precipitins in hypocomplementemic vasculitisurticaria syndrome: partial purification and characterization as immunoglobulin. J Immunol 1978, 121:613–618.

    CAS  PubMed  Google Scholar 

  13. Marder RJ, Potempa LA, Jones JV, et al.: Assay, purification and further characterization of 7S C1q-precipitins (C1q-p) in hypocomplementemic vasculitis urticaria syndrome and systemic lupus erythematosus. Acta Pathol Microbiol Immunol Scand Suppl 1984, 284:25–34.

    CAS  PubMed  Google Scholar 

  14. Sasaki T, Yonemasu K, Matsumoto M, Nagaki K: Characterization of C1q found in a patient with hypocomplementemic vasculitis-urticaria syndrome. Microbiol Immunol 1989, 33:413–422.

    CAS  PubMed  Google Scholar 

  15. Wisnieski JJ, Naff GB: Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome. Arthritis Rheum 1989, 32:1119–1127.

    Article  CAS  PubMed  Google Scholar 

  16. Wisnieski JJ, Jones SM: Comparison of autoantibodies to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome and systemic lupus erythematosus. J Immunol 1992, 148:1396–1403.

    CAS  PubMed  Google Scholar 

  17. Mårtensson U, Sjöholm AG, Sturfelt G, et al.: Western blot analysis of human IgG reactive with the collagenous portion of C1q: evidence of distinct binding specificities. Scand J Immunol 1992, 35:735–744.

    Article  PubMed  Google Scholar 

  18. Kishore U, Reid KB: C1q: structure, function, and receptors. Immunopharmacology 2000, 49:159–170.

    Article  CAS  PubMed  Google Scholar 

  19. Sontheimer RD, Racila E, Racila DM: C1q: its functions within the innate and adaptive immune responses and its role in lupus autoimmunity. J Invest Dermatol 2005, 125:14–25.

    Article  CAS  PubMed  Google Scholar 

  20. Jiang K, Chen Y, Xu CS, Jarvis JN: T cell activation by soluble C1q-bearing immune complexes: implications for the pathogenesis of rheumatoid arthritis. Clin Exp Immunol 2003, 131:61–67.

    Article  CAS  PubMed  Google Scholar 

  21. Dincy CV, George R, Jacob M, et al.: Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India. J Eur Acad Dermatol Venereol 2008, 22:789–794.

    Article  CAS  PubMed  Google Scholar 

  22. Amano H, Furuhata N, Tamura N, et al.: Hypocomplementemic urticarial vasculitis with Jaccoud’s arthropathy and valvular heart disease: case report and review of the literature. Lupus 2008, 17:837–841.

    Article  CAS  PubMed  Google Scholar 

  23. Chew GY, Gatenby PA: Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression. Clin Rheumatol 2007, 26:1370–1372.

    Article  PubMed  Google Scholar 

  24. Ghamra Z, Stoller JK: Basilar hyperlucency in a patient with emphysema due to hypocomplementemic urticarial vasculitis syndrome. Respir Care 2003, 48:697–699.

    PubMed  Google Scholar 

  25. Balsam L, Karim M, Miller F, Rubinstein F: Crescentic glomerulonephritis associated with hypocomplementemic urticarial vasculitis syndrome. Am J Kidney Dis 2008, 52:1168–1173.

    Article  PubMed  Google Scholar 

  26. Ramirez G, Saba SR, Espinoza L: Hypocomplementemic vasculitis and renal involvement. Nephron 1987, 45:147–150.

    Article  CAS  PubMed  Google Scholar 

  27. Grimbert P, Schulte K, Buisson C, et al.: Renal transplantation in a patient with hypocomplementemic urticarial vasculitis syndrome. Am J Kidney Dis 2001, 37:144–148.

    Article  CAS  PubMed  Google Scholar 

  28. Renard M, Wouters C, Proesmans W: Rapidly progressive glomerulonephritis in a boy with hypocomplementaemic urticarial vasculitis. Eur J Pediatr 1998, 157:243–247.

    Article  CAS  PubMed  Google Scholar 

  29. Filosto M, Cavallaro T, Pasolini G, et al.: Idiopathic hypocomplementemic urticarial vasculitis-linked neuropathy. J Neurol Sci 2009 Apr 15 (Epub ahead of print).

  30. Her MY, Song JY, Kim DY: Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus. J Korean Med Sci 2009, 24:184–186.

    Article  PubMed  Google Scholar 

  31. Calvo-Romero JM: Diffuse large B cell lymphoma in a patient with hypocomplementemic urticarial vasculitis. J Postgrad Med 2003, 49:252–253.

    CAS  PubMed  Google Scholar 

  32. Callen JP, Kalbfleisch S: Urticarial vasculitis: a report of nine cases and review of the literature. Br J Dermatol 1982, 107:87–93.

    Article  CAS  PubMed  Google Scholar 

  33. Fortson JS, Zone JJ, Hammond ME, Groggel GC: Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol 1986, 15:1137–1142.

    Article  CAS  PubMed  Google Scholar 

  34. Eiser AR, Singh P, Shanies HM: Sustained dapsone-induced remission of hypocomplementemic urticarial vasculitis—a case report. Angiology 1997, 48:1019–1022.

    Article  CAS  PubMed  Google Scholar 

  35. Nürnberg W, Grabbe J, Czarnetzki BM: Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline. Acta Derm Venereol 1995, 75:54–6l.

    PubMed  Google Scholar 

  36. Lopez LR, Davis KC, Kohler PF, Schocket AL: The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine. J Allergy Clin Immunol 1984, 73:600–603.

    Article  CAS  PubMed  Google Scholar 

  37. Werder M, Truniger B: Hypocomplementaemic urticarial vasculitis. Nephrol Dial Transplant 1997, 12:1278–1279.

    Article  CAS  PubMed  Google Scholar 

  38. Worm M, Muche M, Schulze P, et al.: Hypocomplementaemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy. Br J Dermatol 1998, 139:704–707.

    Article  CAS  PubMed  Google Scholar 

  39. Worm M, Sterry W, Kolde G: Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Br J Dermatol 2000, 143:1324–1326.

    Article  CAS  PubMed  Google Scholar 

  40. Saigal K, Valencia IC, Cohen J, Kerdel FA: Hypocomplementemic urticarial vasculitis with angioedema, a rare presentation of systemic lupus erythematosus: rapid response to rituximab. J Am Acad Dermatol 2003, 49:S283–S285.

    Article  PubMed  Google Scholar 

  41. Kristen AV, Borst MM, Andrassy K, et al.: Anti-oxidant and anti-inflammatory therapy in hypocomplementemic urticarial vasculitis with pulmonary emphysema. Resp Med Extra 2007, 3:147–151.

    Article  Google Scholar 

  42. Staubach-Renz P, von Stebut E, Bräuninger W, et al.: Hypocomplementemic urticarial vasculitis syndrome. Successful therapy with intravenous immunoglobulins. Hautarzt 2007, 58:693–697.

    Article  CAS  PubMed  Google Scholar 

  43. Wisnieski JJ, Emancipator SN, Korman NJ, et al.: Hypocomplementemic urticarial vasculitis syndrome in identical twins. Arthritis Rheum 1994, 37:1105–1111.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Hospital de Especialidades Centro Medico La Raza, Seris/Zaachila S/N Colonia La Raza, ZP 02990, Mexico City, Mexico

    Luis J. Jara

Authors
  1. Luis J. Jara
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Carmen Navarro
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gabriela Medina
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Olga Vera-Lastra
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Miguel A. Saavedra
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Luis J. Jara.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Jara, L.J., Navarro, C., Medina, G. et al. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep 11, 410–415 (2009). https://doi.org/10.1007/s11926-009-0060-y

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-009-0060-y

Keywords

Search

Navigation