scholarly journals Seizures, renal failure and acute respiratory failure: not your typical case of Henoch-Schonlein purpura

2019 ◽  
Vol 12 (7) ◽  
pp. e229939
Author(s):  
Subhasish Bose ◽  
Samata Pathireddy ◽  
Krishna M Baradhi ◽  
Narothama Reddy Aeddula

A 30-year-old Caucasian woman with no prior medical history presented with pedal oedema, arthralgias and abdominal pain with diarrhoea, following a respiratory infection. She had mild abdominal tenderness along with a purpuric rash on the extremities and was anaemic. Following initial workup for anaemia and rash, her condition deteriorated with renal impairment, respiratory failure and seizures necessitating ventilatory support, dialysis and steroids. Serologies were negative, and skin biopsy showed leucocytoclastic vasculitis without vascular IgA deposition, and renal biopsy showed subendothelial, mesangial deposits of IgA with C3 indicative of Henoch-Schonlein purpura (HSP). She was treated with steroids, haemodialysis and on 6-month follow-up recovered renal function. We present the case to illustrate that HSP, though rare in adults, can present with multiorgan failure, with renal, pulmonary and central nervous system involvement, and the need for early diagnosis and prompt treatment for rapid clinical recovery.

2021 ◽  
Vol 73 (10) ◽  
pp. 687-694
Author(s):  
Thanaporn Chaiyapak ◽  
Anirut Pattaragarn ◽  
Suroj Supavekin ◽  
Nuntawan Piyaphanee ◽  
Kraisoon Lomjansook ◽  
...  

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.


2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
A. A. Nikibakhsh ◽  
H. Mahmoodzadeh ◽  
M. Karamyyar ◽  
S. Hejazi ◽  
M. Noroozi ◽  
...  

Background. Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis with an incidence of approximately 10 per 100 000 children. There is some evidence to support steroid therapy in the treatment of severe abdominal pain, severe nephritis, and central nervous system involvement. However, the routine use of corticosteroids is controversial. Frequent relapses, lack of response to steroid, steroid dependency, and steroid side effects may occur in some patients. Mycophenolate mofetil (MMF) gains increasing popularity in the treatment of autoimmune disorders, but hitherto, the available evidence to support the use of MMF in HSP is limited to some case study reports.Case Presentation. We report six children with HSP who failed to respond to systemic steroid therapy, whereas MMF successfully treated the manifestations of the disease.Conclusion. The manifestations of HSP disappeared mainly during the first week of treatment with MMF and all the patients were in a complete remission at the end and after discontinuation of the therapy. In our experience, MMF appeared to be safe and effective for the maintenance of remission in the HSP patients.


2020 ◽  
Author(s):  
Yan Chen ◽  
Haitao Bai ◽  
Yang Yang ◽  
Zhulan Lian

Abstract Background Henoch-Schönlein purpura (HSP) is the commonest vasculitis of childhood, affecting multiple organ systems. The extrarenal manifestations are prominent in some HSP cases, for which no standard treatment is available. It is interesting to examine the value of tacrolimus in such cases. Findings Seventeen children received tacrolimus to manage their refractory extrarenal symptoms of HSP in an open-label prospective study. Tacrolimus was administered at daily dose of 0.05–0.15 mg/kg. Tacrolimus was effective in all cases after treatment for 7–21 days. Tacrolimus treatment resulted in complete resolution of extrarenal symptoms in 15 cases, and partial resolution in the remaining 2 cases. During follow-up, skin purpura reoccurred in 2 cases, abdominal pain and loss of follow-up in 1 case each. Drug-related adverse reaction was not observed in any case. Abnormal renal function test was identified in 7 patients by urinary screening tests before tacrolimus therapy, 4 of them received renal biopsy. Only one patient showed evidence of renal impairment during follow-up. Conclusions Tacrolimus works quickly without drug-related adverse reaction in managing the HSP patients featuring recurrent extrarenal symptoms. Our preliminary experience suggests good clinical efficacy and safety of tacrolimus in such off-label use.


Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii95-iii100
Author(s):  
Seza Ozen ◽  
Erdal Sag

Abstract Vasculitis is a challenging disease for paediatricians. Certain vasculitides are quite common in children whereas others are much rarer compared with adults. The most common vasculitides in childhood are IgA-associated vasculitis (Henoch–Schönlein purpura) and Kawasaki disease, which are usually self-limiting vasculitides although children do develop complications as a result. We now have much better knowledge of how to manage these patients and prevent the deleterious complications. This review provides an up-to-date discussion on childhood vasculitides, including diagnosis, treatment and follow-up strategies, together with a comparison with vasculitides in adults. It also discusses the newly defined monogenic vasculitides that often present during early childhood.


2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Iliyana H. Pacheva ◽  
Ivan S. Ivanov ◽  
Krastina Stefanova ◽  
Elena Chepisheva ◽  
Lyubov Chochkova ◽  
...  

Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009–2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.


2020 ◽  
Author(s):  
Supatjaree Chanvitan ◽  
Kanchana Tangnararatchakit ◽  
Pawaree Saisawat ◽  
Songkiat Chantarogh ◽  
Suchin Worawichawong ◽  
...  

Abstract Introduction: There are limited data on the outcomes in children with Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) in limited-resource countries. This study was aimed to evaluate the outcome of HSPN and IgAN and to evaluate the pathological findings associated with unfavorable outcomes.Materials and methods: This was a retrospective study conducted in children (≤18 years) diagnosed with HSPN or IgAN, had estimated glomerular filtration rate (eGFR) ≥ 60 mL/min/1.73 m2 and underwent renal biopsy between year 2002 - 2018 at Ramathibodi Hospital, Bangkok, Thailand. Clinical outcomes were graded according to the outcome classification by Counahan, as follows: favorable [A, normal; B, minor urinary abnormalities (proteinuria < 1 g/1.73 m2/day)]; unfavorable [C, active renal disease (proteinuria > 1 g/1.73 m2/day and/or hypertension); D, renal insufficiency (eGFR < 60 mL/min/1.73 m2 or died)]. Pathologies were graded according to Oxford classification.Results: A total of 47 patients (28 HSPN and 19 IgAN) were included with means of age at 9.4 ± 2.8 vs 11.3 ± 4.3 years, respectively. After a median follow-up time of 50 months, proportions of favorable outcomes in the patients with HSPN and IgAN were 82.1% (23/28) and 89.5% (17/19), respectively (p-value = 0.685). In multivariate analysis, only endocapillary proliferation (E) was associated with unfavorable outcomes in both diseases with the odds ratio (95% CI) of 12.46 (1.36 - 114.51, p-value = 0.026).Conclusion: The clinical outcomes of most patients with HSPN and IgAN were favorable and comparable. Endocapillary proliferation (E) was the only factor associated with poor outcome in both diseases.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Shuai Qin ◽  
Dan Li ◽  
Bo Zhang

By applying the PDCA model to the care of children with Henoch-Schonlein purpura, the nursing process can be divided into four stages: planning, execution, inspection, and treatment. According to the age characteristics and disease progression of pediatric patients, a complete nursing plan is formulated to efficiently implement the nursing content and improve the nursing effect. This paper studies the application of the PDCA nursing model in the nursing of children with Henoch-Schonlein purpura and statistically analyzes the disappearance of skin rash, joint pain relief, disappearance of urine protein, disappearance of gastrointestinal symptoms, etc. Finally, this paper combines the experiment to evaluate the intervention effect of this nursing model and provides reference for the follow-up care of children with Henoch-Schonlein purpura. From the results of experimental research, it can be known that PDCA nursing can improve the comfort of children with Henoch-Schonlein purpura, shorten the time for symptom disappearance, and speed up the recovery process.


2016 ◽  
Vol 35 (11) ◽  
pp. 2847-2850 ◽  
Author(s):  
Izabela Grabska-Kobylecka ◽  
Dariusz Nowak ◽  
Anna Wlodarczyk ◽  
Piotr Bialasiewicz

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