Severe Cutaneous Presentation of Hydralazine-induced ANCA Vasculitis Without Renal or Pulmonary Involvement, Complicated by DIC

Overview

Journal BMJ Case Rep

Specialty General Medicine

Date 2021 May 12

PMID 33975832

Authors

Mae Xintong Huo

Alzira Rocheteau M Avelino

Gurpreet Singh

Affiliations

Soon will be listed here.

Abstract

Hydralazine is a common arterial vasodilator used in the management of congestive heart failure and hypertension. It can be associated with drug-induced lupus and less commonly antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Drug-induced AAV typically has a favourable long-term prognosis. It is not commonly associated with primary skin involvement, as most cases also have notable kidney and lung disease. Cases with isolated skin findings are rare. We present a rare case of a 60-year-old woman on long-term hydralazine who presented with AAV with primary skin and mucosal involvement, in the form of diffuse bullous and ulcerative lesions, which posed a diagnostic challenge. Her hospital course was marked by several complications including disseminated intravascular coagulation. She required intensive therapy with high-dose steroids, plasmapheresis and rituximab. She tolerated immunosuppression well and with multidisciplinary supportive care, she recovered well and was able to be discharged from the hospital.

References

Niles J . Rituximab in induction therapy for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. Clin Exp Immunol. 2011; 164 Suppl 1:27-30. PMC: 3095862. DOI: 10.1111/j.1365-2249.2011.04363.x. View

Mukhtyar C, Guillevin L, Cid M, Dasgupta B, de Groot K, Gross W . EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2008; 68(3):310-7. DOI: 10.1136/ard.2008.088096. View

Pendergraft 3rd W, Niles J . Trojan horses: drug culprits associated with antineutrophil cytoplasmic autoantibody (ANCA) vasculitis. Curr Opin Rheumatol. 2013; 26(1):42-9. DOI: 10.1097/BOR.0000000000000014. View

Ntatsaki E, Watts R, Scott D . Epidemiology of ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010; 36(3):447-61. DOI: 10.1016/j.rdc.2010.04.002. View

Radic M, Martinovic Kaliterna D, Radic J . Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012; 70(1):12-7. View

Yokogawa N, Vivino F . Hydralazine-induced autoimmune disease: comparison to idiopathic lupus and ANCA-positive vasculitis. Mod Rheumatol. 2009; 19(3):338-47. DOI: 10.1007/s10165-009-0168-y. View

Wada H, Thachil J, Di Nisio M, Mathew P, Kurosawa S, Gando S . Guidance for diagnosis and treatment of DIC from harmonization of the recommendations from three guidelines. J Thromb Haemost. 2013; . DOI: 10.1111/jth.12155. View

Bernstein R, Webster J . Hydrallazine-induced cutaneous vasculitis. Br Med J. 1980; 280(6208):156-7. PMC: 1600356. DOI: 10.1136/bmj.280.6208.156-a. View

Goto K, Nakai K, Fujii H, Nishi S . The Effects of Plasma Exchange on Severe Vasculitis with Diffuse Alveolar Hemorrhage. Intern Med. 2017; 56(1):55-59. PMC: 5313425. DOI: 10.2169/internalmedicine.56.7317. View

10.

Walsh M, Merkel P, Peh C, Szpirt W, Guillevin L, Pusey C . Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials. 2013; 14:73. PMC: 3607855. DOI: 10.1186/1745-6215-14-73. View

11.

Nemoto M, Nishioka K, Fukuoka J, Aoshima M . Hepatitis B Virus-associated Vasculitis: Multiple Cavitary Masses in the Lung Mimicking Granulomatous Polyangiitis. Intern Med. 2019; 58(20):3013-3017. PMC: 6859398. DOI: 10.2169/internalmedicine.3012-19. View

12.

Ragab G, Hussein M . Vasculitic syndromes in hepatitis C virus: A review. J Adv Res. 2017; 8(2):99-111. PMC: 5272950. DOI: 10.1016/j.jare.2016.11.002. View

13.

Schrom K, Field H, Chang T, Montanez-Wiscovich M . Antineutrophil cytoplasmic antibody vasculitis induced by hydralazine. Cutis. 2020; 105(1):E32-E35. View

14.

Chanouzas D, McGregor J, Nightingale P, Salama A, Szpirt W, Basu N . Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated Vasculitis: a multi-center retrospective cohort study. BMC Nephrol. 2019; 20(1):58. PMC: 6378728. DOI: 10.1186/s12882-019-1226-0. View

15.

Stone J, Merkel P, Spiera R, Seo P, Langford C, Hoffman G . Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010; 363(3):221-32. PMC: 3137658. DOI: 10.1056/NEJMoa0909905. View

16.

Bolton W, STURGILL B . Methylprednisolone therapy for acute crescentic rapidly progressive glomerulonephritis. Am J Nephrol. 1989; 9(5):368-75. DOI: 10.1159/000167998. View

17.

Saito T, Tsuchiya M, Shikata C, Yamaguchi H, Miyata S, Matsuo S . Microscopic polyangiitis associated with marked systemic bleeding tendency caused by disseminated intravascular coagulation. Intern Med. 2003; 42(9):850-5. DOI: 10.2169/internalmedicine.42.850. View

18.

Kumar B, Strouse J, Swee M, Lenert P, Suneja M . Hydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis. Semin Arthritis Rheum. 2018; 48(2):283-287. DOI: 10.1016/j.semarthrit.2018.01.005. View

19.

Specks U, Merkel P, Seo P, Spiera R, Langford C, Hoffman G . Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013; 369(5):417-27. PMC: 5953195. DOI: 10.1056/NEJMoa1213277. View