Henoch-Schönlein nephritis associated with streptococcal infection and persistent hypocomplementemia: a case report

Abstract

IntroductionHenoch-Schönlein purpura is a systemic disease with frequent renal involvement, characterized by IgA mesangial deposits. Streptococcal infection can induce an abnormal IgA immune response like Henoch-Schönlein purpura, quite similar to typical acute post-infectious glomerulonephritis. Indeed, hypocomplementemia that is typical of acute glomerulonephritis has also been described in Henoch-Schönlein purpura.Case presentationWe describe a 14-year-old Caucasian Spanish girl who developed urinary abnormalities and cutaneous purpura after streptococcal infection. Renal biopsy showed typical findings from Henoch-Schönlein purpura nephritis. In addition, she had low serum levels of complement (C4 fraction) that persisted during follow-up, in spite of her clinical evolution. She responded to treatment with enalapril and steroids.ConclusionThe case described has, at least, three points of interest in Henoch-Schönlein purpura: 1) Initial presentation was preceded by streptococcal infection; 2) There was a persistence of low serum levels of complement; and 3) There was response to steroids and angiotensin-converting enzyme inhibitor in the presence of nephrotic syndrome. There are not many cases described in the literature with these characteristics. We conclude that Henoch-Schönlein purpura could appear after streptococcal infection in patients with abnormal complement levels, and that steroids and angiotensin-converting enzyme inhibitor could be successful treatment for the disease.

Henoch–Schönlein purpura with hypocomplementemia

“…Two further case reports also provided evidence of streptococcal infection [7,8]. In our study, 37 patients in the case group had their levels of ASO measured, and elevated titers were found in 29 patients.…”

“…Deposits of IgA selfaggregates or IgA-IgGICs, which may be present in the glomerular mesangium and in the vascular walls, could potentially activate the complement system during HSP [1][2][3]. Serum complement levels are normal in most patients with HSP, but reductions in levels of C3 and C4 can occur [3,[5][6][7][8][9][10]. Complement levels tend to normalize within 4 to 6 weeks according to previous reports [5,6].…”

“…Rivera et al reported a patient with severe renal injury, which was complicated by persistent hypocomplementemia in which a congenial complement deficit could not be excluded [8]. A further paper reported on a patient with HSP with hypocomplementemia who ultimately developed endstage renal failure [7].…”

“…Abnormalities of the complement system in HSP have been previously reported [3,[5][6][7][8][9][10]. However, the total number of HSP patients with hypocomplementemia studied in any single report has only been, to our knowledge, 10 at most [5].…”

Clinical remission of Henoch–Schönlein purpura nephritis after a monotherapeutic tonsillectomy

“…Consistently, there have been several reports demonstrating a potential role of GAS infection in the pathogenesis of HSPN [12][13][14][15]. However, acute poststreptococcal glomerulonephritis (APGN) has been considered to be the most common form of immune-mediated glomerulopathy related to GAS infection [16].…”