Intravenous immunoglobulin for corticosteroid-resistant intestinal Henoch-Schönlein purpura: worth a controlled trial against corticosteroids?

Rheumatology ◽  
2020 ◽  
Author(s):  
Francesco Morotti ◽  
Giulia Bracciolini ◽  
Roberta Caorsi ◽  
Lorella Cattaneo ◽  
Marco Gattorno ◽  
...  
Keyword(s):  
Controlled Trial ◽  
Patient Characteristics ◽  
Complete Recovery ◽  
Ivig Treatment ◽  
High Dose ◽  
Henoch Schonlein Purpura ◽  
Standardized Protocol ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Ivig Administration

Abstract Objectives Henoch–Schönlein purpura (HScP) may present in children with severe, occasionally refractory, gastrointestinal (GI) involvement. The use of corticosteroids (CSs) is commonplace in the management of the disease, but to date no standardized protocol is available and, although rare, resistance to CS therapy may be challenging to clinicians. IVIG has been proposed as an effective alternative to CSs, but to date no controlled trial has been conducted to ascertain their real efficacy. We share our personal experience of successful IVIG treatment in two cases of GI HScP, comparing it with similar experiences reported in literature. Methods Retrospective clinical data collection, comparison with available literature. Results We describe two children with severe HScP GI vasculitis refractory to high-dose intravenous CSs that responded rapidly to IVIG administration, with complete recovery within a few days. Patient characteristics and response to IVIG administration were comparable to those of other previously reported cases. Conclusion Our observation confirms that IVIG may be useful in the treatment of CS-resistant HScP-related GI vasculitis in children, and highlights the need for more structured research, including a randomized trial against CSs, in order to ascertain their real effectiveness.

scholarly journals Renal Outcomes of Childhood IgA Nephropathy and Henoch Schönlein Purpura Nephritis

2021 ◽  
Vol 73 (10) ◽  
pp. 687-694
Author(s):  
Thanaporn Chaiyapak ◽  
Anirut Pattaragarn ◽  
Suroj Supavekin ◽  
Nuntawan Piyaphanee ◽  
Kraisoon Lomjansook ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Complete Recovery ◽  
Clinicopathological Characteristics ◽  
Henoch Schonlein Purpura ◽  
Renal Outcomes ◽  
Nephritic Syndrome ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

Background: Henoch-Schönlein purpura nephritis (HSPN) is considered the systemic form of IgA nephropathy (IgAN). However, differing clinicopathological features and renal outcomes of children with IgAN and HSPN have been reported in some studies. Methods: This study retrospectively reviewed children with IgAN and HSPN younger than 18 years, between January 2004 and December 2015. The clinicopathological characteristics at diagnosis and the renal outcomes after at least 1 year of follow-up were compared between the two groups. Results: A total of 54 children, comprising 21 with IgAN and 33 with HSPN, were recruited. The children with HSPN were younger than the children with IgAN. Gross hematuria and nephritic syndrome at the initial presentation were more common in children with IgAN. Regarding the pathological findings, IgAN had greater chronicity than HSPN. After a median follow-up period from first presentation to renal outcomes measurement of 4.0 years (1.3-12.2) in children with IgAN and 4.2 years (1.1-11.4) in children with HSPN, the renal outcomes were better in the latter group. The incidence of chronic kidney disease (CKD) was 28.6% in children with IgAN and 6.1% in children with HSPN (p = 0.02). Complete recovery was observed more frequently in children with HSPN than in children with IgAN (57.1% in IgAN vs. 87.9% in HSPN, p = 0.01). Conclusions: Childhood IgAN has greater chronicity and worse renal outcomes than childhood HSPN, with a lower rate of complete recovery and a higher frequency of CKD. We recommend long-term follow-up for CKD in children with IgAN.

scholarly journals Recurrent Henoch-Schönlein Purpura with Bullous Rash and Pulmonary Nodules

2020 ◽  
Author(s):  
Christopher Zixiang Zheng ◽  
Julie Childers ◽  
Egla Rabinovich ◽  
Kristina Nazareth-Pidgeon
Keyword(s):  
Oral Corticosteroid ◽  
Pulmonary Nodules ◽  
High Dose ◽  
Henoch Schonlein Purpura ◽  
Case Presentation ◽  
Abdominal Symptoms ◽  
Intravenous Corticosteroid ◽  
Dose Oral ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract 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.

scholarly journals Acute renal insufficiency and pancreatitis in a child with atypical Henoch–Schönlein purpura: efficacy of a single dose of cyclophosphamide

2018 ◽  
Vol 46 (12) ◽  
pp. 5285-5290 ◽  
Author(s):  
Maria Cristina Maggio ◽  
Silvio Maringhini ◽  
Saveria Sabrina Ragusa ◽  
Giovanni Corsello
Keyword(s):  
Abdominal Pain ◽  
Skin Lesions ◽  
Severe Anaemia ◽  
High Dose ◽  
Henoch Schonlein Purpura ◽  
Acute Renal Insufficiency ◽  
High Doses ◽  
Schonlein Purpura ◽  
Persistent Response ◽  
Henoch Schönlein Purpura

A 9-year-old boy with petechiae on the legs and abdominal pain was unsuccessfully treated with steroids. He was admitted to our hospital for the onset of fever, ecchymosis, and arthralgia. Skin lesions suggested vasculitis, but they were not typical of Henoch–Schönlein purpura. He showed ecchymosis of the scrotal bursa, diffusion of petechiae to the trunk and arms, vomiting, severe abdominal pain, oliguria with hyponatremia, hypoalbuminemia, low C3 levels, high levels of creatinine, blood urea nitrogen, and tubular enzymes, proteinuria, and glycosuria. The urinary sediment showed macrohaematuria, and hyaline and cellular casts. Ultrasound showed polyserositis. He was treated with intravenous furosemide, albumin, and methylprednisolone. He underwent colonoscopy and gastroscopy because of development of acute pancreatitis and severe anaemia. Typical lesions of Henoch–Schönlein purpura were observed in the small intestine and colon mucosa. He received three high doses of methylprednisolone, followed by intravenous cyclophosphamide. A dramatic and persistent response was observed after these doses. A single high dose of cyclophosphamide is appropriate in Henoch–Schönlein purpura with acute renal failure and severe pancreatitis that are non-responsive to high-dose steroids.

scholarly journals Henoch Schonlein Purpura as a Cause of Renal Failure in an Adult

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Shweta Kukrety ◽  
Pradeepa Vimalachandran ◽  
Rajesh Kunadharaju ◽  
Vishisht Mehta ◽  
Agnes Colanta ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Renal Involvement ◽  
High Dose ◽  
Henoch Schonlein Purpura ◽  
Increased Risk ◽  
Stage Renal Disease ◽  
End Stage ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch Schonlein purpura (HSP) is an immune mediated disease associated Immunoglobulin A (IgA) deposition within the affected organs. While the disease is commonly seen in the pediatric age group, it is rarely seen in adults. We report the case of a 93-year-old Caucasian lady who presented with nonthrombocytopenic purpuric rash and acute kidney injury after an episode of bronchitis. Rapid and progressive deterioration of renal function prompted a kidney biopsy, which showed findings consistent with IgA nephropathy confirming the diagnosis of HSP. The patient was treated with high dose intravenous methylprednisolone followed by oral prednisone; however, her kidney disease progressed to end stage renal disease requiring hemodialysis. HSP is usually a self-limiting disease in children. However, adults are at an increased risk of severe renal involvement including end stage renal disease. Purpuric skin rash with renal involvement should raise suspicion for HSP. This is the oldest known patient with HSP.

Treatment of Henoch-Schönlein Purpura Glomerulonephritis in Children with High-Dose Corticosteroids plus Oral Cyclophosphamide

2001 ◽  
Vol 21 (2) ◽  
pp. 128-133 ◽  
Author(s):  
Joseph T. Flynn ◽  
William E. Smoyer ◽  
Timothy E. Bunchman ◽  
David B. Kershaw ◽  
Aileen B. Sedman
Keyword(s):  
High Dose ◽  
Oral Cyclophosphamide ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

A randomized controlled trial on the effect of dietary guidance on the treatment of Henoch-Schonlein purpura in children

2021 ◽  
pp. jim-2021-001984
Author(s):  
Li Wang ◽  
Chunyan Yin ◽  
Meizhen Zhang ◽  
Hua Mao ◽  
Huixiang Hao ◽  
...  
Keyword(s):  
Nutrient Intake ◽  
Dietary Intervention ◽  
Controlled Trial ◽  
Diet Group ◽  
Control Group ◽  
Restricted Diet ◽  
Henoch Schonlein Purpura ◽  
Intake Level ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

The amino acid-based formulae were extensively added to diet of children for the treatment of Henoch-Schonlein purpura (HSP), and the nutrition and growth situation of children were evaluated after giving new dietary intervention. Patients were randomly divided into restricted diet group (n=30) and dietary guidance group (n=30). Besides, 30 cases with bronchiolitis who had normal diet were selected as the control group. The dietary questionnaire was designed to record the types and intakes of various foods taken by children every day, and the intake levels of nutrients were analyzed. Physical examination, biochemical analysis of blood and urine routine were carried out to evaluate the effect of dietary guidance on their growth and development. The results showed that restricted diet group had lower levels of nutrient intake and the actual/recommended percentage. However, overall nutrient intake level of the dietary guidance group was higher, basically equal to the recommended intake level. Besides, the actual intake and actual/recommended percentage of nutrients of dietary guidance group were significantly higher than those of restricted diet group (p<0.05). Dietary guidance can improve nutrients and protein intake of children with HSP, and reduce the relapse of rash and incidence of complications.

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