Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database

Lei, Wei‐Te; Tsai, Po-Li; Chu, Szu-Hung; Kao, Yu-Hsuan; Lin, Chien‐Yu; Fang, Li-Ching; Shyur, Shyh-Dar; Lin, Yung‐Chieh; Wu, Shu‐I

doi:10.1186/s12969-018-0247-8

Cited by 61 publications

(57 citation statements)

References 44 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although HSP tends to be a self-limited disease, it has been suggested the recurrence rate is about 16% [ 6 ]. Treatment for CS dependent and DMARD refractory HSP, however, can be challenging.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

et al. 2018

View full text Add to dashboard Cite

BackgroundHenoch-Schonlein purpura (HSP) is a small vessel vasculitis that is characterized by non-thrombocytopenic purpura, abdominal pain, arthritis, and glomerulonephritis. Typically, HSP is self-limited requiring only supportive care, but more severe cases may require corticosteroid (CS) treatment. Rarely, a subset of these patients has persistent rash, arthritis, abdominal involvement, or renal disease despite treatment with CS, or has disease recurrence on CS tapering. Refractory HSP has been effectively treated with a variety of CS sparing therapies. For life-threatening refractory HSP, the B cell depleting agent, rituximab (RTX), has been reported as beneficial for children with substantial renal or central nervous system involvement. However, RTX use for children with less severe HSP, but chronic CS dependent disease refractory to CS sparing immunomodulatory agents, has been less well explored. Herein, we describe 8 children treated with RTX for chronic refractory HSP and report a reduction in recurrent hospitalizations and eventual CS discontinuation.MethodsThis is a retrospective analysis of eight children who were treated with RTX for chronic CS dependent HSP during the years 2006–2014 at a single institution. A chart review of the electronic medical record was performed to determine the presenting symptoms, the type and duration of treatment received, and the number of hospitalizations prior to and after RTX. The number of hospitalizations and oral corticosteroid burden were analyzed using the Wilcoxon signed rank test.ResultsPrior to receiving RTX, seven patients had at least one hospitalization for HSP (median 1.5, range 0–3). Following RTX, only two patients were hospitalized, each a single time for recurrent abdominal pain. The median oral CS burden was 0.345 mg/kg/day before RTX and 0 mg/kg/day at 6 months (p = 0.078), 1 year (p = 0.0625), and 2 years (p = 0.03) following RTX infusion. Seven out of eight children met remission criteria, defined as no active rash, arthritis, nephritis (hematuria and proteinuria), or gastrointestinal distress following RTX. No serious adverse events were noted.ConclusionOverall, RTX effectively reduced the number of hospital admissions and oral CS burden. RTX also helped most all children achieve clinical remission. RTX appears to be an effective and safe alternative for chronic CS dependent and immunomodulatory refractory childhood HSP.

show abstract

Section: Discussionmentioning

confidence: 99%

“…CS remains the standard treatment for symptomatic HSP. A small subset of patients are unable to wean off CS and subsequently require long term CS or additional steroid-sparing agents for disease control [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

et al. 2018

View full text Add to dashboard Cite

show abstract

“…Henoch-Schönlein purpura (HSP) is a small vessel vasculitis that features immunoglobulin A (IgA) deposition, mainly in the joints, skin, and kidneys. The incidences of HSP are approximately 20 and 10 per 100,000 personyears in children and adults, respectively [1], wherein the risk is higher in Asians than in other ethnic groups [2]. When the disease affects the kidneys, it is called HSP nephritis (HSPN).…”

Section: Introductionmentioning

confidence: 99%

MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Schönlein purpura nephritis

Yun

Joo

et al. 2020

BMC Nephrol

View full text Add to dashboard Cite

Background: Henoch-Schönlein purpura nephritis (HSPN), a small-vessel vasculitis, shares renal pathological features with immunoglobulin A nephropathy. Oxford classification of immunoglobulin A nephropathy pathology has been updated to the MEST-C score, but its application in HSPN remains unresolved. Methods: Two hundred and thirteen patients with biopsy-proven HSPN were retrieved from the Seoul National University Hospital between 2000 and 2017. Renal outcome risks (i.e., end-stage renal disease or doubling of serum creatinine) were evaluated according to MEST-C scores after stratification by age: 113 children aged < 18 years (9.2 ± 3.6 years) and 100 adults aged ≥18 years (38.6 ± 18.3 years). We pooled our data with four previous cohort studies in which MEST or MEST-C scores were described in detail.Results: Twenty-one child (19%) and 16 adult (16%) patients reached the renal outcome during the median followup periods of 12 years and 13 years, respectively (maximum 19 years). In children, M1 and T1/T2 scores revealed worse renal outcomes than did M0 and T0 scores, respectively, whereas the T score was the only factor related to worse outcomes in adult patients after adjusting for multiple clinical and laboratory variables. The pooled data showed that M1, S1, and T1/T2 in children and E1 and T1/T2 in adults were correlated with poorer renal outcomes than those of their counterpart scores. Conclusions: The Oxford classification MEST-C scores can predict long-term renal outcomes in patients with HSPN.

show abstract

“…Henoch-Schönlein Purpura is an IgA-mediated leukocytoclastic vasculitis involving small vessels. It affects approximately 10-20 children per 100,000 each year(1), with recurrence in approximately one-fifth to one-fourth of cases (2,3). The characteristic initial presentation of palpable purpura with a lower extremity predominance is typically followed by gastrointestinal manifestations, renal involvement, and/or joint pain.…”

Section: Introductionmentioning

confidence: 99%

Recurrent Henoch-Schönlein Purpura with Bullous Rash and Pulmonary Nodules

Zheng

Childers

Rabinovich

et al. 2020

Preprint

View full text Add to dashboard Cite

Background Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. Case presentation We present a novel case of recurrent pediatric HSP with bullous rash and pulmonary nodules, successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper. Conclusion The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient’s abdominal symptoms, rash and pulmonary nodules.

show abstract

Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database

Cited by 61 publications

References 44 publications

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Schönlein purpura nephritis

Recurrent Henoch-Schönlein Purpura with Bullous Rash and Pulmonary Nodules

Contact Info

Product

Resources

About