A Familiar Face in the Treatment of Immunoglobulin A Vasculitis: Colchicine

Abstract

Objective: The aim of the this study was to evaluate immunoglobulin A vasculitis (IgAV) patients on colchicine therapy and determine the causative factors leading to the initiation and maintenance of colchicine. Material and Methods: This retrospective study was conducted receiving IgAV patients on colchicine at our clinic between January 2013 and June 2023. Demographic, clinical, and laboratory data of IgAV patients were obtained from their electronic medical records. Results: A total of 33 IgAV patients receiving colchicine, 20 (60.6%) males and 13 (39.4%) females, were included in the study. The mean age at diagnosis of IgAV was 111.6±45.5 months. Colchicine was initiated in 13 (39.4%) patients with persistent rash, 8 (24.2%) with severe gastrointestinal tract involvement, seven (21.2%) with generalized/severe rash, and five (15.2%) with recurrent IgAV. The median elapsed time to colchicine initiation was 30 (10.5-60) days. The median duration of colchicine usage was ten (IQR, 6-54) months. Colchicine treatment was discontinued in 14 patients after a median 6.5 (IQR, 3-11.2) months. At the last visit, 13 (39.4%) patients were receiving colchicine for familial Mediterranean fever (FMF), four (12.1%) for recurrent IgAV, and two (6.1%) for persistent rash. Conclusion: Colchicine should be kept in mind especially in the presence of severe, persistent or recurrent rash. Early initiation of colchicine treatment should be considered in IgAV patients with severe GI tract disease, especially in countries where FMF is common.

Keywords

• Colchicine
• Immunoglobulin A vasculitis
• Leukocytoclastic vasculitis
• Recurrence

References

1. Du L, Wang P, Liu C, Li S, Yue S, Yang Y. Multisystemic manifestations of IgA vasculitis. Clin Rheumatol 2021;40:43-52.
2. Pillebout E, Sunderkötter C. IgA vasculitis. Semin Immunopathol 2021;43:729-38.
3. Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford) 2019;58:1607-16.
4. Poddighe D, Romano M, Garcia-Bournissen F, Demirkaya E. Conventional and novel therapeutic options in children with familial Mediterranean fever: A rare autoinflammatory disease. Br J Clin Pharmacol. 2022;88:2484-99.
5. Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med 2021;16:831-41.
6. Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol 2008;9:71-92.
7. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798-806.
8. Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, et al. Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis 2019;78:1025-32.

9. Yalçinkaya F, Ozen S, Ozçakar ZB, Aktay N, Cakar N, Düzova A, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford) 2009;48:395-8.
10. Dolezalova P, Price-Kuehne FE, Özen S, Benseler SM, Cabral DA, Anton J, et al. Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). Ann Rheum Dis 2013;72:1628-33.
11. Nothhaft M, Klepper J, Kneitz H, Meyer T, Hamm H, Morbach H. Hemorrhagic Bullous Henoch-Schonlein¨ Purpura: case Report and Review of the Literature. Front Pediatr 2019;6:413.
12. Ramelli V, Lava SA, Simonetti GD, Bianchetti MG, Ramelli GP, Milani GP. Blistering eruptions in childhood Henoch-Schonlein¨ syndrome: systematic review of the literature. Eur J Pediatr 2017;176:487–92.
13. Sestan M, Kifer N, Sozeri B, Demir F, Ulu K, Silva CA, et al. Vasculitis Working Party of the Pediatric Rheumatology European Society (PReS). Clinical features, treatment and outcome of pediatric patients with severe cutaneous manifestations in IgA vasculitis: Multicenter international study. Semin Arthritis Rheum 2023;61:152209.
14. Li Y, Zhang X, Liu H, Li G, Guan W, Zhang T, et al. Severe gastrointestinal involvement in pediatric IgA vasculitis: a retrospective single-center cohort study in China. Front Pediatr 2023;11:1194214.
15. Den Boer SL, Pasmans S, Wulffraat NM, Ramakers-Van Woerden NL, Bousema MT. Bullous lesions in Henoch Schonlein Purpura as indication to start systemic prednisone. Acta Paediatr 2010; 99:781–3.
16. Fraticelli P, Benfaremo D, Gabrielli A. Diagnosis and management of leukocytoclastic vasculitis. Intern Emerg Med 2021;16:831-41.
17. Chan KH, Tang WY, Lo KK. Bullous lesions in Henoch-Schönlein purpura. Pediatr Dermatol 2007;24:325-6.
18. Saulsbury FT. Successful treatment of prolonged Henoch-Schönlein purpura with colchicine. Clin Pediatr (Phila) 2009;48:866-8.
19. Allali S, Fraitag S, Terrier B, Bodemer C, Chalumeau M. Efficacy of colchicine in a child with relapsing bullous Henoch-Schönlein purpura. Eur J Pediatr 2016; 175:147-9.
20. Ekinci RMK, Balci S, Bisgin A, Atmis B, Dogruel D, Altintas DU, et al. MEFV gene variants in children with Henoch-Schönlein purpura and association with clinical manifestations: a single-center Mediterranean experience. Postgrad Med 2019;131:68-72.
21. Cakici EK, Kurt Şükür ED, Özlü SG, Yazılıtaş F, Özdel S, Gür G, et al. MEFV gene mutations in children with Henoch-Schönlein purpura and their correlations-do mutations matter? Clin Rheumatol 2019;38:1947-52.