scholarly journals Clinical and Demographic Features of 195 Pediatric Patients with Henoch-Schönlein Purpura, 12-Year Single Center Experience in Shiraz

2020 ◽  
Vol 8 (4) ◽  
Author(s):  
Hossein Esmaeilzadeh ◽  
Seyed Alireza Masoudi ◽  
Narjes Ebrahimi ◽  
Soheyla Alyasin ◽  
Hesamodin Nabavizadeh

Background: Henoch-Schönlein purpura (HSP) is one of the most common systemic types of vasculitis in children. Although it is a self-limited disease, life-threatening complications such as nephritis may occur. Early diagnosis and follow up might improve the long term outcome in renal involvement. There are few studies that have evaluated HSP in Iran. Objectives: The purpose of this study was to investigate demographic, laboratory data and clinical presentations of admitted HSP patients in a tertiary referral center, over a twelve-year period. Methods: This retrospective descriptive study evaluated 195 patients, diagnosed with HSP, who were admitted to Namazi Hospital in southwest of Iran (2006 - 2018). Demographic, clinical and laboratory findings, as well as treatment outcome of HSP patients were collected. Results: There were 118 males and 77 females with the mean age of 6.7 ± 3.21 years. About 70 (36%) patients showed common cold symptoms two weeks before HSP presentations. Admission course was 1 - 17 days (mean 4.55 ± 2.83) and autumn was recorded with the highest number of admitted patients (44.1%). In the course of hospitalization, 100% of the patients presented with palpable purpura, 61.02% with joint pain and 19.49% with abdominal pain. Moreover, 17.95% of the patients were noted with renal involvement. Laboratory data shows that more than half of patients (54%) had leukocytosis, only 9% of patients had positive CRP but all the patients had high erythrocyte sedimentation rate (ESR). Total of 43.1% of the patients received corticosteroids. Conclusions: The observed number of male patients with HSP was higher than females and the highest frequency of the HSP cases was observed in autumn. Joint pain and abdominal pain were the predominant clinical presentations, following skin purpura. The presented data can help with further HSP diagnosis and treatment plan.

2020 ◽  
Author(s):  
Maria Francesca Gicchino ◽  
Dario Iafusco ◽  
Maria Maddalena Marrapodi ◽  
Rosa Melone ◽  
Giovanna Cuomo ◽  
...  

Abstract Background : Henoch Schonlein purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. Although the cause is unknown, IgA seems to play a central role in the pathogenesis of Henoch Schonlein purpura. The major clinical features include a palpable purpuric rash on the lower extremities, abdominal pain or renal involvement, and arthritis. Cutaneous manifestations are the essential elements in the diagnosis of Henoch Schonlein purpura. The palpable purpura is characteristically 2 to 10mm in diameter and is usually present on the lower extremities. There are no specific diagnostic tests available for diagnosing this condition. Laboratory studies are useful to exclude other conditions that may mimic Henoch Schonlein purpura. In majority of the cases, the disease is self-limited. Relapsing can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. Corticosteroids are effective in rapid resolution of renal and abdominal manifestations. Immunosuppressive drugs, such as Mycophenolate Mofetil may be a better treatment choice in case of renal involvement.Case report : We report a case of a 14 years old girl affected from recurrent Henoch Schonlein Purpura. From the age of nine years patient presented three episodes of purpura with gastrointestinal involvement, in particular hematemesis, abdominal pain and diarrhoea. Each episode was treated with high doses of corticosteroids (methylprednisolone in vein or prednisone per os). Patient came to our Department during the third episode of Purpura. In consideration of the recurrence of the Henoch Schonlein Purpura and the gastrointestinal involvement we decided to start Mycophenolate Mofetil treatment. Patient’s conditions improved thanks to Mycophenolate Mofetil treatment. Conclusion: In our case of recurrent HSP Mycophenolate Mofetil treatment has been very effective, avoiding the adverse events of a prolonged steroid treatment. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistence, recurrence or complicated forms of Henoch Schonlein purpura in children.


2020 ◽  
Vol 8 ◽  
pp. 232470962092556 ◽  
Author(s):  
Amanda S. Weissman ◽  
Viral Sanjay Patel ◽  
Omar Mushfiq

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Guo Zhen Fan ◽  
Rui Xue Li ◽  
Qi Jiang ◽  
Man Man Niu ◽  
Zhen Qiu ◽  
...  

Abstract Background The present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. Methods Two thousand seventy-four Chinese children with HSP were recruited from January 2015 to December 2019. Patients’ histories associated with HSP onset were obtained by interviews and questionnaires. Laboratory data of urine tests, blood sample and infectious agents were collected. Renal biopsy was performed by the percutaneous technique. Results (1) Streptococcal infection was identified in 393 (18.9%) HSP patients, and served as the most frequent infectious trigger. (2) Among the 393 cases with streptococcal infection, 43.0% of them had arthritis/arthralgia, 32.1% had abdominal pain and 29.3% had renal involvement. (3) 26.1% of HSP patients relapsed or recurred more than 1 time within a 5-year observational period, and the relapse/recurrence rate in streptococcal infectious group was subjected to a 0.4-fold decrease as compared with the non-infectious group. (4) No significant differences in renal pathological damage were identified among the streptococcal infectious group, the other infectious group and the non-infectious group. Conclusions Streptococcal infection is the most frequent trigger for childhood HSP and does not aggravate renal pathological damage; the possible elimination of streptococcal infection helps relieve the relapse/recurrence of HSP.


2018 ◽  
Vol 64 (2) ◽  
pp. 74-77 ◽  
Author(s):  
Rabia Miray Kisla Ekinci ◽  
Sibel Balci ◽  
Mahir Serbes ◽  
Gulsah Duyuler Ayçin ◽  
Dilek Dogruel ◽  
...  

Introduction Henoch Schönlein purpura is characterised by palpable purpura, abdominal pain, arthritis/arthralgia, often with a self-limiting course. Herein, we report a patient with recurrent Henoch Schönlein purpura and severe gastrointestinal involvement, successfully treated with methotrexate. Case presentation A 12-year-old boy was admitted to our department with palpable purpura, abdominal pain and arthralgia. Since gastrointestinal complaints were severe, systemic steroids were administered, with tapering of dosage. Henoch Schönlein purpura recurred several times with severe abdominal pain, maelena and purpura during next two months. Colchicine and hydroxychloroquine were initiated. After four months, we also introduced methotrexate, which enabled discontinuation of previous medications including corticosteroids. Methotrexate was ceased four months later, and remission was sustained without any medications for 24 months. Conclusion Besides the conflicting data regarding the use of methotrexate in recurrent Henoch Schönlein purpura, our case introduces successful methotrexate experience in a child with Henoch Schönlein purpura and recurrent severe gastrointestinal involvement.


2016 ◽  
Vol 136 (5) ◽  
pp. S22
Author(s):  
J. St. John ◽  
A. Garza-Mayers ◽  
P. Vedak ◽  
M. Hoang ◽  
S. Nigwekar ◽  
...  

PEDIATRICS ◽  
1986 ◽  
Vol 78 (1) ◽  
pp. 183-184
Author(s):  
J. J. LEFRÈRE ◽  
A. M. COUROUCÉ ◽  
J. P. SOULIER ◽  
M. P. CORDIER ◽  
M. C. GUESNE GIRAULT ◽  
...  

To the Editor.— Human parvovirus is already known to be responsible for aplastic crisis in chronic hemolytic anemia,1 for erythema infectiosum or fifth disease,2 and for arthropathies,3,4 and it has recently been isolated from the serum of patients with vascular purpura.5 We report the case of Henoch-Schonlein purpura associated with human parvovirus infection. H. T., a 6-year-old girl, without any significant past history, was admitted on March 8, 1985, for joint pain and swelling (wrists, knees, ankles) associated with intense abdominal pain.


Author(s):  
Eda Didem Kurt-Şükür ◽  
Thivya Sekar ◽  
Kjell Tullus

Abstract Background Knowledge on normal progress and treatment of Henoch-Schönlein purpura nephritis (HSPN) is limited. This study reviews outcome, clinical, pathological, and therapeutic factors affecting the prognosis of HSPN patients. Methods Forty-nine children with biopsy-confirmed HSPN diagnosed between September 2008 and 2018 were included. Demographics, clinical and laboratory data, treatment, and outcome were recorded at the time of biopsy, 3, 6, 12, and 24 months and at last visit. Clinical outcome was graded according to Meadow’s criteria. Results The median age at time of biopsy was 10.1 years (IQR:5.7) and female/male ratio 24/25. At presentation, 40.8% of patients had nonnephrotic proteinuria, 18.4% nephrotic syndrome (NS), 4.1% nephritic syndrome (NephrS), and 36.7% NephrS+NS. There were 11 patients with an estimated glomerular filtration rate below 90 ml/min/1.73 m2. Biopsy specimens were classified according to International Study of Kidney Diseases in Children (ISKDC) and Oxford Classification MEST-C scoring systems. Forty-one patients received angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, 37 patients steroids, and 35 patients other immunosuppressive medications. At last visit, 24 patients had stage 1 chronic kidney disease (CKD), three stage 2 CKD, and two had stage 5 CKD. Neither clinical parameters nor ISKDC biopsy grade or treatment modalities effected the final outcome. The Oxford classification showed significantly increased segmental glomerulosclerosis in patients with unfavorable outcome. Favorable outcome was associated with shorter time from kidney involvement to biopsy and start of treatment. Conclusion A large proportion of patients continued to show signs of CKD at last follow-up while only a small proportion developed stage 5 CKD.


2016 ◽  
Vol 85 (2) ◽  
Author(s):  
Daša Kumprej ◽  
Tomaž Krenčnik ◽  
Aleksandra Aleksandrova Oberstar ◽  
Nataša Toplak

Background: Henoch-Schönlein purpura is the most common vasculitis of small blood vessels in children. The diagnosis of the disease is confirmed in a patient with a specific rash, joint inflamation, abdominal pain or renal disease. The specific rash is necesary for the confirmation of the diagnosis. Henoch-Schönlein purpura can rarely present with a complication withot a prior presentation of the rash. In these cases diagnosis is difficult until the presentation of the specific skin manifestation. In the majority of patients the disease course is not complicated and has a good prognosis.Conclusion: In this article we present three patients with an atypical presentation of the disease and a review of current literature on the topic.


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