IgA vasculitis with terminal ileitis and giant duodenum ulceration and review of the literatures

Author(s):  
Wang Fengge ◽  
Wang Xiaoli ◽  
Wu Daiqin

Abstract BackgroundImmunoglobulin A vasculitis (IgA V), formerly Henoch–Schönlein purpura, is one of the most common self-limiting vasculitis in children. In 15-20% IgA V cases, gastrointestinal symptoms precede the rashes,they no specific gastrointestinal symptoms and often present as acute abdomen, making the diagnosis difficult.Case presentationWe present two female patients, ages 8 years old and 5 years old, who presented to vomiting and abdominal pain, rashes appeared on the lower limbs a few days later, they were subsequently diagnosed with IgA V. Both patients were given IV methylprednisolone, achieved benign clinical results.ConclusionsWhen gastrointestinal symptoms precede the rashes, it is difficult to diagnose IgA vasculitis, resulting in unnecessary surgery. Early recognition and treatment will promote the remission of the children and avoid unnecessary surgery.

2021 ◽  
Author(s):  
Ana Lia Costa Mano ◽  
Mafalda Casinhas Santos ◽  
Sara Limão ◽  
Florbela Cunha

Henoch–Schönlein purpura (HSP) is characterized by nonthrombocytopenic palpable purpura, arthritis or arthral­gia, and gastrointestinal and/or renal involvement. Gastrointestinal symptoms are reported in 50%-75% and they are related to a previous group A streptococcal infection in 40%. A healthy 5-year-old girl presented with a three-week history of a recurrent purpuric rash on the lower limbs, ar­thralgia and angioedema, without renal involvement. During the third relapse, she had severe, diffuse and persistent abdominal pain and bloody stools. An abdominal ultrasound revealed transmural edema of the last ileal segment, compatible with ileitis. She received prednisolone for five days, with full clinical recovery. Antistreptolysin O titer was elevated. The remaining laboratory tests were normal (antinuclear, anti-neutrophil and anti-Saccharomyces cerevisiae antibodies; rheumatoid factor; stool cultures, parasitological examination and viral antigen tests). One month later, an abdominal ultrasound revealed no abnormalities. Terminal ileitis is a very rare complication of HSP in children but has a good prognosis.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
George Naifa ◽  
George Totikidis ◽  
Sonia Alexiadou ◽  
Christina Kolona ◽  
Elpis Mantadakis

IgA vasculitis (formerly known as Henoch–Schönlein purpura or anaphylactoid purpura) is a usually benign vasculitis that affects children of school age. The disease is characterized by the tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and hematuria. Treatment of IgA vasculitis is mainly supportive, with administration of simple analgesics. Corticosteroids have been shown to reduce and/or ameliorate the occurrence of abdominal pain which may be severe. We present two children with IgA vasculitis and severe abdominal pain despite corticosteroid administration, who responded promptly to intravenous γ globulin (IVIg) with complete resolution of their symptoms and review of the relevant medical literature. Given the toxicity and/or need for long-term administration of other second-line immunosuppressive therapies in corticosteroid-resistant IgA vasculitis, such as rituximab, cyclosporine, cyclophosphamide, azathioprine, or colchicine, we propose that IVIg may be a useful and safe treatment option, although randomized controlled clinical trials are needed in order to clarify its role in the treatment of abdominal pain in IgA vasculitis.


2020 ◽  
Vol 0 ◽  
pp. 1-3
Author(s):  
Pedro Nogarotto Cembraneli ◽  
Julia Brasileiro de Faria Cavalcante ◽  
Renata Brasileiro de Faria Cavalcante ◽  
José Edison da Silva Cavalcante

Henoch-Schönlein purpura (HSP) is a small vessel systemic vasculitis. Typical symptoms include palpable purpura, joint pain, and abdominal pain. Most cases improve after a few weeks, not requiring any treatments other than symptom control. Acute abdomen resulting from vasculitis is very rare and should be treated as a surgical emergency. We report the case of a 9-year-old boy with acute gangrenous appendicitis as the first manifestation of HSP.


2021 ◽  
Vol 14 (11) ◽  
pp. e247188
Author(s):  
James A Maye ◽  
Hsu Pheen Chong ◽  
Vivek Rajagopal ◽  
William Petchey

A 23-year-old man presented to the acute assessment unit with acute-onset haematuria within 24 hours of receiving his second dose of the Pfizer-BioNTech COVID-19 vaccine. He had been diagnosed with IgA vasculitis 8 months previously. IgA vasculitis is an autoimmune condition characterised by palpable purpura affecting the lower limbs, abdominal pain, arthralgia and renal disease. He was diagnosed with an acute exacerbation of IgA vasculitis and was discharged with oral prednisolone. Reactivation or first presentation of IgA vasculitis is a rare but increasingly recognised complication of COVID-19 vaccination. This is an important new differential in the assessment of patients with haematuria following COVID-19 vaccination.


2020 ◽  
Vol 36 (5) ◽  
pp. 417-420
Author(s):  
Florian Kühn ◽  
Matthias Klein ◽  
Henning Laven ◽  
Nikolaus Börner ◽  
Tobias Weinberger ◽  
...  

During the current COVID-19 pandemic, the triage, assessment, and management of patients presenting to the emergency department with critical conditions has become ­challenging. The clinical features of COVID-19 are heterogeneous and subtle in many cases. They may easily be overlooked in the case of other acute diseases. Gastrointestinal symptoms are common in patients with COVID-19 as SARS-CoV-2 is able to enter gastrointestinal epithelial cells. However, these complaints can also be caused by a COVID-19-independent concomitant abdominal pathology. Therefore, patients with acute abdominal pain and fever need to be assessed very thoroughly. Based on a clinical case, we present our approach of managing emergency patients with acute abdomen and concomitant suspicion of ­COVID-19.


2021 ◽  
Vol 17 (2) ◽  
pp. 164-167
Author(s):  
Anna Fedorczak ◽  
◽  
Dorota Szałowska-Woźniak ◽  
Krzysztof Zeman ◽  
◽  
...  

IgA vasculitis, also known as the Henoch–Schönlein purpura, is the most common systemic vasculitis in children. Inflammation most often involves small blood vessels of the skin, joints, kidneys and the gastrointestinal tract, but other organs may also be involved. Acute pancreatitis is a rare clinical manifestation of IgA vasculitis. We present a case of a 12-year-old girl with cutaneous and abdominal symptoms, who was diagnosed with acute pancreatitis during IgA vasculitis. In patients with IgA vasculitis who report abdominal pain acute pancreatitis should be taken into account and pancreatic enzymes measurement should be considered.


2021 ◽  
Vol 12 ◽  
Author(s):  
Yan Song ◽  
Xiaohan Huang ◽  
Guizhen Yu ◽  
Jianjun Qiao ◽  
Jun Cheng ◽  
...  

Immunoglobin A (IgA) vasculitis (IgAV), formerly called the Henoch-Schönlein purpura (HSP), is a small vessel vasculitis, characterized by IgA1-dominant immune deposition at diseased vessel walls. IgAV is the most common form of vasculitis in children; typical symptoms include palpable purpura, arthritis or arthralgia, abdominal pain, and hematuria or proteinuria. Galactose-deficient IgA1 is detected in the tissues of the kidney and skin in patients with IgAV; it forms immune complexes leading to subsequent immune reactions and injuries. This report provides the recent advances in the understanding of environmental factors, genetics, abnormal innate and acquired immunity, and the role of galactose-deficient IgA1 immunocomplexes in the pathogenesis of IgAV.


Reumatismo ◽  
2017 ◽  
Vol 69 (3) ◽  
pp. 131 ◽  
Author(s):  
F. Almassinokiani ◽  
A. Mehdizadeh Kashi ◽  
A. Musavi ◽  
S. Khodaverdi ◽  
K. Tahermanesh ◽  
...  

Henoch-Schönlein purpura (HSP) is caused by deposition of IgA-containing immune complexes within the blood vessels. HSP mostly occurs in children and is less common in adults. In addition to palpable purpura, arthritis or arthralgia, renal disease and abdominal pain, gastrointestinal symptoms occur in 50% of children. These can be caused by gastrointestinal hemorrhage, bowel ischemia and necrosis, intussusception, and bowel perforation. In adults, intussusception is less frequent than in children. We report a 42-year-old woman referred to our hospital with acute abdominal pain. After laboratory and radiologic examinations, laparatomy was performed which revealed rectal perforation due to HSP. HSP is a multisystem disorder. Abdominal pain in patients with HSP may herald a serious gastrointestinal problem also in adult patients.


2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Caroline Maccallum ◽  
Sarah Eaton ◽  
Daniel Chubb ◽  
Stephen Franzi

Torsion of the fatty appendage of the falciform ligament is an extremely rare condition that leads to severe abdominal pain and raised inflammatory markers. It can be recognised on ultrasound or CT scan. The pathophysiology is the same as that involved in the more common torsion and/or infarction of the greater omentum or epiploic appendages. The condition is best managed conservatively with anti-inflammatory analgesia, and the early recognition of this type of torsion may prevent unnecessary operative intervention to look for a source of abdominal pain. There have been five reported adult cases of a torted fatty appendage of the falciform ligament identified on ultrasound and CT scan, but no paediatric cases. We report a case of torsion of the fatty appendage of the falciform ligament in a ten-year-old boy and describe its imaging characteristics on CT scan.


2020 ◽  
Vol 16 (4) ◽  
pp. 414-417
Author(s):  
Katarzyna Gmachowska ◽  

IgA vasculitis is the most common acute systemic vasculitis in children. Typical symptoms include cutaneous purpura not associated with thrombocytopenia, acute-onset abdominal pain, arthritis, renal symptoms and, less commonly, neurological symptoms. The disease is usually mild and self-limiting. Although the aetiology of IgA vasculitis is unknown, autoimmune involvement is suspected. Symptomatic treatment is mainly used; therapeutic method that would reduce disease duration and prevent recurrence is unknown. Meningococcal sepsis should always be ruled out in a child with cutaneous purpura. We present a case of a 4-year-old boy with IgA vasculitis who developed symptoms of meningeal irritation.


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