Published: 2021-07-27

Clinical significance of anti-phospholipid antibodies in Henoch Schönlein purpura

Anuj Gupta, Joshika Agarwal, Shilpi Gupta, Anurag Singh


Background: Henoch Schönlein purpura also known as IgA vasculitis is described histologically by IgA deposition in the blood vessel walls and presents with kidney involvement, palpable purpura, arthralgia, and abdominal pain. Our study aims to evaluate the association between anti-phospholipid antibody, anti-cardiolipin antibody, anti-beta(2) glycoprotein 1 antibody and Anti-phosphatidylserine/prothrombin antibodies and IgA vasculitis. Treatment response with intravenous steroids and cyclophosphamide was also studied based on resolution of antibody titer.

Methods: We conducted an observational study in three Rheumatology clinics at Ahmedabad, India. Data was collected for a period of 6 months. Diagnosis of IgA vasculitis was determined based on the International Chapel hill consensus conference 2012. Disease activity was assessed based on antibody titer, histological grading and through a pre-determined clinical form to assess objective clinical symptoms. P value of less than 0.05 was considered significant

Results: Study evaluated antibody titer of 178 patients. Sixty one percent of the patient's had positive anti-phospholipid antibody titer with predominant antibody subtype as IgG. Inflammatory markers were significantly higher in patient having anti-phospholipid antibody titer. Anti-phospholipid antibody was present in 100 percent patients who had vascular thrombosis. IgG subtype of anti-cardiolipin antibody were found in 60 percent of the patients with renal complication.

Conclusions: Anti-phospholipid antibody have a close association with IgA vasculitis. Anti-phospholipid antibody has a significant role in mounting inflammatory response and vascular thrombosis. Combination treatment of intravenous steroids and cyclophosphamide found to be more effective in resolution of titer


Vasculitis, antibodies, anti-phospholipid, Henoch Schönlein purpura

Full Text:



Lally L, Sammaritano LR. Vasculitis in antiphospholipid syndrome. Rheum Dis Clin North Am. 2015;41(1):109-23.

Hočevar A, Rotar Ž, Žigon P, Čučnik S, Ostrovršnik J, Tomšič M. Antiphospholipid antibodies in adult IgA vasculitis: observational study. Clin Rheumatol. 2019;38(2):347-51.

Norden DK, Ostrov BE, Shafritz AB, Von-Feldt JM. Vasculitis associated with anti-phospholipid syndrome. In: Seminars in arthritis and rheumatism. USA: WB Saunders; 1995:24(4);273-81.

Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of serum antiphospholipid antibody levels in adult Henoch-Schönlein purpura and cutaneous leukocytoclastic angiitis. Arthritis Rheum. 2008;59(4):561-7.

Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol. 2008;9(2):71-92.

Tajima C, Suzuki Y, Mizushima Y, Ichikawa Y. Clinical significance of immunoglobulin A antiphospholipid antibodies: possible association with skin manifestations and small vessel vasculitis. J Rheumatol. 1998;25(9):1730-6.

Bueno Filho R, Cordeiro AP, Almeida FT, Shaletich C, Costa RS, Roselino AM. Rare association of cutaneous vasculitis, IgA nephropathy and antiphospholipid antibody syndrome with tuberculous lymphadenitis. Clinics (Sao Paulo). 2012;67(12): 1497-500.

Burden AD, Tillman DM, Foley P, Holme E. IgA class anticardiolipin antibodies in cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1996;35(3 Pt 1):411-5.

Lockshin MD, Sammaritano LR, Schwartzman S. Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum. 2000;43(2):440-3.

Filho R, Cordeiro AP, Almeida FT, Shaletich C, Costa RS, Roselino AM. Rare association of cutaneous vasculitis, IgA nephropathy and antiphospholipid antibody syndrome with tuberculous lymphadenitis. Clinics (Sao Paulo). 2012;67(12):1497-500.

Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017; 16(12):1246-53.

Banday AZ, Jindal AK, Kaur A, Saka R, Parwaiz A, Sachdeva MUS, et al. Cutaneous IgA vasculitis-presenting manifestation of a novel mutation in the IKZF1 gene. Rheumatology (Oxford). 2021;60(3): e101-3.

Fujiwara K, Shimizu J, Tsukahara H, Shimada A. Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature. Rheumatol Int. 2019;39(10):1811-9.

Yachoui R, Sehgal R, Amlani B, Goldberg JW. Anti-phospholipid antibodies-associated diffuse alveolar hemorrhage. In: Seminars in arthritis and rheumatism. USA: WB Saunders; 2015:44(6);652-7.

Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14(7):579-85.

Selewski DT, Ambruzs JM, Appel GB, Bomback AS, Matar RB, Cai Y, et al. Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study. Kidney Int Rep. 2018;3(6):1373-84.

González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S. IgA Vasculitis: Genetics and Clinical and Therapeutic Management. Curr Rheumatol Rep. 2018;20(5):24.

Kimball AS, Obi AT, Diaz JA, Henke PK. The Emerging Role of NETs in Venous Thrombosis and Immunothrombosis. Front Immunol. 2016 ;7:236.

Du L, Wang P, Liu C, Li S, Yue S, Yang Y. Multisystemic manifestations of IgA vasculitis. Clin Rheumatol. 2021;40(1):43-52.