Preventive Effect of Tonsillectomy on Recurrence of Henoch-Schönlein Purpura Nephritis after Intravenous Methylprednisolone Pulse Therapy.

Henoch-Schönlein purpura (HSP) is regarded as a benign and self-limiting vasculitis characterized by purpura, arthritis, and gastrointestinal symptoms; however, about one third of the patients develop HSP nephritis (HSPN), the most serious long-term complication. Since 2013, we have proposed that tonsillectomy in addition to intravenous methylprednisolone pulse therapy (IVMP) be performed in all patients with HSPN, similar to immunoglobulin A nephropathy (IgAN) patients because both diseases are considered to a share common pathogenesis. Herein, we retrospectively reviewed the clinical courses of 71 Japanese children with HSPN (34 boys; median age at diagnosis, 6.7 years; median follow-up period, 5.6 years) who had received initial treatment with IVMP (15-20 mg/kg; on 3 consecutive days/week for 3 weeks) followed by oral prednisolone (initially 1 mg/kg; tapered off within 12 months) and achieved clinical remission (i.e., disappearance of both proteinuria and hematuria). The patients were divided into two groups: 31 patients receiving tonsillectomy after IVMP between 2013 and 2017 (tonsillectomy group) and 40 patients receiving IVMP monotherapy between 2003 and 2012 (IVMP group). For the 2 years after IVMP therapy, the rate of HSPN recurrence (i.e., persistent proteinuria combined with hematuria requiring additional treatments) after clinical remission was significantly lower in the tonsillectomy group than the IVMP group (0% vs. 19%, P < 0.05). Despite the short follow-up period in the tonsillectomy group, this study provides the evidence that tonsillectomy may be beneficial for preventing recurrence of HSPN from clinical remission with IVMP therapy in Japanese children.

Blockade of the kinin B1 receptor ameloriates glomerulonephritis.

Severe inflammation characterizes rapidly progressive glomerulonephritides, and expression of the kinin B1 receptor (B1R) associates with inflammation. Delayed B1R blockade reduces renal inflammation in a model of unilateral ureteral obstruction, but whether B1R modulates the pathophysiology of glomerulonephritides is unknown. Here, we observed an association of B1R protein expression and inflammation, in both glomeruli and the renal interstitium, in biopsies of patients with glomerulonephritides, Henoch-Schönlein purpura nephropathy, and ANCA-associated vasculitis. In the nephrotoxic serum-induced glomerulonephritis model, we observed upregulation of the B1R receptor; treatment with a B1R antagonist beginning 2 weeks after the onset of disease reduced both glomerular and tubular lesions and improved renal function. B1R blockade reduced renal chemokine expression and macrophage accumulation. Collectively, our data demonstrate that blockade of the kinin B1R has significant potential for the treatment of glomerulonephritis.

Henoch-Schõnlein purpura: recurrence and chronicity

OBJETIVOS: Descrever um grupo de pacientes com púrpura de Henoch-Schõnlein seguido em nosso serviço, com ênfase nos casos recorrentes e crônicos, e comparar pacientes com doença monocíclica ou recorrente segundo características clínicas e demográficas. MÉTODOS: Foram avaliados dados de 67 pacientes atendidos desde o início da doença. Doze pacientes foram excluídos, pois abandonaram o seguimento antes de 3 meses de acompanhamento, sendo incluídas 55 crianças no estudo. Recorrência foi definida pela presença de novo quadro após um período de 3 meses sem sintomas, e cronicidade como persistência de manifestações cutâneas, abdominais e renais por um período ≥ 12 meses. RESULTADOS: Foi observada recorrência em 8/55 pacientes (14,4 por cento) e cronicidade em quatro (7,2 por cento). Em 29/55 pacientes (52,7 por cento), foi constatada infecção como fator desencadeante. Curso monocíclico esteve presente em 43 pacientes (26 meninas, média de idade de 5,4 anos). Acometimento gastrointestinal e renal foi observado em 55,8 e 20,9 por cento dos pacientes, respectivamente. Dentre os 12 pacientes com púrpura de Henoch-Schõnlein recorrente ou crônica, três apresentaram artrite, quatro tiveram sinais e sintomas de comprometimento abdominal e sete de doença renal: hematúria microscópica em cinco, hematúria macroscópica em um e hematúria com proteinúria em um paciente. Idade de início tardia foi a única variável relacionada com a presença de recorrência (p < 0,05). CONCLUSÕES: Como observado na literatura médica, o curso monocíclico é mais freqüente nas crianças com início da doença em idade mais precoce. Pacientes com púrpura de Henoch-Schõnlein devem ser seguidos a longo prazo, uma vez que recorrências e/ou cronicidade são observadas em mais de 20 por cento dos casos.

OBJECTIVES: To describe a group of patients treated at our service for Henoch-Schõnlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. METHODS: Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return for follow-up consultations after less than 3 months, leaving a total of 55 children in the study sample. Recurrence was defined as the presence of a fresh episode after a period of at least 3 months without symptoms, and cases were defined as chronic when cutaneous, abdominal and renal manifestations persisted for a period of 12 months or more. RESULTS: Recurrence was observed in 8/55 patients (14.4 percent) and four cases were chronic (7.2 percent). In 29/55 patients (52.7 percent), infection was identified as the trigger factor. A monocyclic clinical course was observed in 43 patients (26 of whom were girls, with a mean age of 5.4 years). Gastrointestinal and renal involvement was observed in 55.8 and 20.9 percent of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schõnlein purpura, three had arthritis, four exhibited signs and symptoms of abdominal involvement and seven of kidney disease: microscopic hematuria in five, macroscopic hematuria in one and hematuria with proteinuria in one other. Late onset was the only variable related to recurrence (p < 0.05). CONCLUSIONS: As is observed in medical literature, monocyclic cases are more common among children with early onset disease. Patients with Henoch-Schõnlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20 percent of the total.

Henoch-Schönlein purpura remitting in pregnancy and during sex steroid therapy.

This paper reports on a 22-yr old female patient with recurrent bouts of Henoch-Schönlein purpura since puberty occurring monthly at the time of menstruation. Remissions occurred during pregnancy, and on several occasions and in a dose responsive manner when the patient was prescribed birth control pills containing progesterone and oestradiol. Flares recurred when this therapy was discontinued. We hypothesize that this patient has an inflammatory disease involving neutrophil activation by excessive IgA, which is modulated by exogenous and endogenous sex steroids. It is unlikely that these hormonal influences are a common occurrence, but rather represent, in this case, a singular example of potential endocrine modulation of inflammatory disease.