Clinical significance of anti-phospholipid antibodies in Henoch Schönlein purpura

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 significantResults: 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

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DISORDERS OF HEMOSTASIS IN IgA VASCULITIS (HENOCH– SCHÖNLEIN PURPURA) IN CHILDREN AND THEIR CORRECTION

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2019-98-4-71-77 ◽

2019 ◽

Vol 98 (4) ◽

pp. 71-77

Author(s):

M.A. Kudryashova ◽

◽

N.S. Podchernyaeva ◽

E.V. Frolkova ◽

◽

...

Keyword(s):

Iga Vasculitis ◽

Henoch Schonlein Purpura ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

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Biopsy-proven Henoch-Schönlein purpura nephritis: a single center experience

Pediatric Nephrology ◽

10.1007/s00467-020-04809-8 ◽

2020 ◽

Author(s):

Eda Didem Kurt-Şükür ◽

Thivya Sekar ◽

Kjell Tullus

Keyword(s):

Angiotensin Receptor Blockers ◽

Laboratory Data ◽

Treatment Modalities ◽

Oxford Classification ◽

Henoch Schonlein Purpura ◽

Nephritic Syndrome ◽

Kidney Involvement ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura ◽

Purpura Nephritis

Abstract Background Knowledge on normal progress and treatment of Henoch-Schönlein purpura nephritis (HSPN) is limited. This study reviews outcome, clinical, pathological, and therapeutic factors affecting the prognosis of HSPN patients. Methods Forty-nine children with biopsy-confirmed HSPN diagnosed between September 2008 and 2018 were included. Demographics, clinical and laboratory data, treatment, and outcome were recorded at the time of biopsy, 3, 6, 12, and 24 months and at last visit. Clinical outcome was graded according to Meadow’s criteria. Results The median age at time of biopsy was 10.1 years (IQR:5.7) and female/male ratio 24/25. At presentation, 40.8% of patients had nonnephrotic proteinuria, 18.4% nephrotic syndrome (NS), 4.1% nephritic syndrome (NephrS), and 36.7% NephrS+NS. There were 11 patients with an estimated glomerular filtration rate below 90 ml/min/1.73 m2. Biopsy specimens were classified according to International Study of Kidney Diseases in Children (ISKDC) and Oxford Classification MEST-C scoring systems. Forty-one patients received angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, 37 patients steroids, and 35 patients other immunosuppressive medications. At last visit, 24 patients had stage 1 chronic kidney disease (CKD), three stage 2 CKD, and two had stage 5 CKD. Neither clinical parameters nor ISKDC biopsy grade or treatment modalities effected the final outcome. The Oxford classification showed significantly increased segmental glomerulosclerosis in patients with unfavorable outcome. Favorable outcome was associated with shorter time from kidney involvement to biopsy and start of treatment. Conclusion A large proportion of patients continued to show signs of CKD at last follow-up while only a small proportion developed stage 5 CKD.

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