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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.
PurposeAdherence is a challenging issue in the treatment of systemic lupus erythematosus. Nonadherence has been widely addressed in patients with lupus and must be detected quickly to prevent severe complications. The cases we present illustrate the importance of adherence in young adults.Case 1A 23-year-old Spanish woman diagnosed with severe lupus nephritis 8 years previously achieved renal remission after immunosuppressive treatment with corticosteroids and cyclophosphamide. Three years later, she developed a renal flare. Her treatment was intensified, and rituximab and mycophenolate mofetil were added. One year later, she was readmitted for a new renal flare. A blood test revealed no detectable levels of mycophenolic acid, and the patient admitted she had not taken her treatment correctly. Treatment was resumed. Four years later, the patient remains in remission.Case 2A 19-year-old Spanish woman was diagnosed with nephrotic syndrome due to lupus nephritis. She achieved complete remission after treatment with corticosteroids and cyclophosphamide followed by mycophenolate mofetil. Two years later, she developed a severe renal relapse that was treated with corticosteroids, cyclophosphamide, and rituximab. The response to treatment was good. Mycophenolic acid was undetectable in blood. The patient admitted that she had often missed doses before this relapse. The renal response has been maintained since she resumed her previous medications 2 years ago.ConclusionWe conclude that the frequent and severe relapses of lupus nephritis observed in young patients may actually be due to nonadherence rather than to refractory disease. Our cases are typical examples of nonadherence that were discovered after a detailed interview with the patients and their families. We emphasize the need for clinical suspicion of nonadherence when caring for young adults with lupus.
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