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Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy (TMA), but pathogenic mutations in complement genes have been reported in patients with hypertension-induced TMA. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. A cohort of 110 patients with malignant hypertension caused by diseases other than aHUS served as control. Thirty-six patients with aHUS presented Grade 2 or Grade 3 hypertension and funduscopic examination showed malignant hypertension in 19. Genetic abnormalities in complement were found in 19 patients (37% among patients with malignant hypertension). Plasmapheresis was performed in 46 patients and 26 received eculizumab. Renal and hematological responses were significantly lower after plasmapheresis (24%) than after eculizumab (81%). Renal survival was significantly higher in patients treated with eculizumab (85% at one, three and five years) compared to patients who did not receive this treatment (54%, 46% and 41%), respectively. Response to eculizumab was independent of hypertension severity and the presence of complement genetic abnormalities. Among patients with malignant hypertension caused by other diseases the prevalence of TMA was very low (5%). Thus, severe and malignant hypertension are common among patients with aHUS and eculizumab treatment leads to a higher renal survival when compared to plasmapheresis. However, TMA is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.

Francisco Rivera*, Sara Anaya*Sección de Nefrología, Hospital General Universitario de Ciudad Real, Ciudad Real, Spain*These authors contributed equally to this workPurpose: Adherence 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 1: A 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 2: A 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.Conclusion: We 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 nee