ABSTRACT
Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. 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 thrombotic microangiopathy 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, thrombotic microangiopathy is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.
Subject(s)
Atypical Hemolytic Uremic Syndrome/complications , Complement System Proteins/genetics , Hypertension, Malignant/epidemiology , Severity of Illness Index , Adult , Antibodies, Monoclonal, Humanized/therapeutic use , Atypical Hemolytic Uremic Syndrome/genetics , Atypical Hemolytic Uremic Syndrome/therapy , Complement Inactivating Agents/therapeutic use , Female , Humans , Hypertension, Malignant/diagnosis , Hypertension, Malignant/genetics , Hypertension, Malignant/therapy , Incidence , Male , Middle Aged , Plasmapheresis , Retrospective Studies , Young AdultABSTRACT
No disponible
Subject(s)
Humans , Polycystic Kidney, Autosomal Recessive/diagnosis , Renal Insufficiency, Chronic/complications , Caroli Disease/diagnosis , Muscle Cramp/etiologySubject(s)
Kidney Failure, Chronic/etiology , Muscle Cramp/etiology , Polycystic Kidney, Autosomal Recessive/diagnosis , Adult , Caroli Disease/complications , Caroli Disease/diagnostic imaging , Cholangiopancreatography, Magnetic Resonance , Disease Progression , Female , Humans , Kidney Failure, Chronic/therapy , Peritoneal Dialysis , Polycystic Kidney, Autosomal Recessive/complications , Polycystic Kidney, Autosomal Recessive/diagnostic imaging , Ultrasonography , Weight LossABSTRACT
La amiloidosis secundaria o AA es una grave complicación de la enfermedad de Crohn (EC) evolucionada para la que no se dispone de un tratamiento eficaz. Presentamos un caso excepcional de un varón de 33 años afecto de insuficiencia renal moderada y proteinuria, que fue diagnosticado simultáneamente de nefropatía amiloide AA y de EC oligosintomática. Fue tratado con infliximab, 5 mg/kg/8 semanas durante 4 años, azatioprina 1-1,5 mg/kg/día (primer año) y un bloqueante del sistema renina-angiotensina-aldosterona, sin complicaciones. El tratamiento se siguió de una reducción de la proteinuria, mejoría de la función renal y de los parámetros inflamatorios a lo largo del tiempo. A propósito de este caso revisamos la literatura médica y concluimos que infliximab puede ser de utilidad para el tratamiento precoz de la amiloidosis secundaria en la EC (AU)
Secondary amyloidosis (AA) is a severe complication of progressed Crohn's disease (CD) for which no effective treatment exists. We present the exceptional case of a 33 year-old male with moderate renal failure and proteinuria, who was simultaneously diagnosed with AA amyloid nephropathy and oligosymptomatic CD. He was treated with infliximab at 5mg/kg/8 weeks for 4 years, azathioprine at 1-1.5mg/kg/day (first year) and renin-angiotensin-aldosterone system blockers, with no complications. Treatment caused a decrease in proteinuria, improved renal function, and improved inflammatory parameters over time. Inspired by this case, we performed a review of the medical literature and found that infliximab could be a useful tool in the early treatment of amyloidosis secondary to CD (AU)
Subject(s)
Humans , Male , Adult , Amyloidosis/drug therapy , Crohn Disease/complications , Antibodies, Monoclonal/pharmacokinetics , Proteinuria/complicationsABSTRACT
Secondary amyloidosis (AA) is a severe complication of progressed Crohn’s disease (CD) for which no effective treatment exists. We present the exceptional case of a 33 year-old male with moderate renal failure and proteinuria, who was simultaneously diagnosed with AA amyloid nephropathy and oligosymptomatic CD. He was treated with infliximab at 5mg/kg/8 weeks for 4 years, azathioprine at 1-1.5mg/kg/day (first year) and renin-angiotensin-aldosterone system blockers, with no complications. Treatment caused a decrease in proteinuria, improved renal function, and improved inflammatory parameters over time. Inspired by this case, we performed a review of the medical literature and found that infliximab could be a useful tool in the early treatment of amyloidosis secondary to CD.
