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EULAR/PRINTO/PRES diagnostic criteria of Henoch- Schönlein Purpura Palpable non-thrombocytopenic purpura (mandatory criterion) with lower limb predominance in the presence of at least one of the follow- ing four features:
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Background:
Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the most frequent clinical presentation. Little is known about the real incidence of lung involvement during HSP in the pediatric age...
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Context 1
... diagnosis of HSP is based on clinical findings. The diagnostic criteria for HSP were recently revised by various study groups (EULAR European League Against Rheumatism, PRINTO Pediatric Rheumatology International Trials Organisation and PRES Pediatric Rheumatology European Society), and are listed in Table 1 [11,12]. ...Similar publications
A 47-year-old female presented with complaints of abdominal pain and a history of new-onset maculopapular rash. A workup including laboratory and imaging studies, colonoscopy, and biopsy was performed that led to the diagnosis of adult-onset IgA vasculitis. The patient responded well to intravenous methylprednisolone and was followed up as an outpa...
Background
The long-term prognosis of Henoch-Schönlein purpura (HSP) depends on the severity of renal involvement, and crescent formation is considered an important risk factor for poor prognosis of Henoch-Schönlein purpura nephritis (HSPN). The objective of this study was to evaluate factors affecting crescent formation in children with HSPN.
Met...
Henoch–Schönlein purpura (HSP) is a typical cutaneous immune skin disease, usually diagnosed by invasive biopsy. In this study, we develop a noninvasive optical method by combining in vivo optical clearing, confocal microscopy and immune-staining together to present the real-time in vivo dynamics of blood vessels, IgA molecules, and T cells in a HS...
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 wit...
Objective
To investigate the characteristics of renal pathological grades in Henoch–Schönlein purpura nephritis (HSPN) children with mild to moderate proteinuria and the correlation between pathological grade and severity of proteinuria among this population.
Methods
HSPN children who were presented with mild (150 mg <24 h urinary protein <25 mg/k...
Citations
... For example, in an early study reported in 1992, Chaussain and colleagues evaluated a cohort of patients hospitalized for IgA vasculitis and identified interstitial lung changes (69%) and impaired lung diffusion capacity (97%) despite the absence of significant respiratory symptoms [55]. However, severe lung involvement and pulmonary hemorrhage are rare in children and are primarily reported in adults and adolescents [56]. ...
The clinical association of purpura, arthralgia, and arthritis was first described in 1837 in a publication by Johann Lukas Schönlein, a German physician. In 1874, Eduard Henoch, a student of Schönlein, reported cases of children with purpura, abdominal pain, bloody diarrhea, and joint pain. IgA vasculitis, or Henoch-Schönlein purpura, is a systemic hypersensitivity vasculitis caused by the deposition of immune complexes in small blood vessels, including the renal glomeruli and mesangium. In the skin, the presentation is with non-thrombocytopenic purpura or urticaria. Worldwide, IgA nephropathy is the most common cause of primary glomerulonephritis. Detection of IgA deposition in small blood vessels and the renal glomeruli is diagnostic in most cases. This article aims to review the history, current classification, epidemiology, presentation, and diagnosis of IgA vasculitis and nephropathy, disease associations or trigger factors, including infections, vaccines, and therapeutic agents, and highlights some future approaches to improve diagnosis and clinical management.
... In severe cases, especially in the presence of renal dysfunction, immunosuppression may be required, including corticosteroids, mycophenolate mofetil, cyclosporine, rituximab, and dapsone. (8,9) In the acute phase, the outcome depends mainly on the extent of gastrointestinal involvement, while the long-term prognosis depends mainly on the extent of renal involvement. Recurrence occurs in approximately one-third of patients, but long-term follow-up is warranted in high-risk patients. ...
... IgAV is mostly a disease of childhood, with 90% of affected individuals under the age of 10 and a mean age of diagnosis of six years. (2,9) The incidence is probably underestimated at 10-56 children per 100,000 per year due to underreporting. (11,12) There is a predominance of cases in winter. ...
... (11,12) There is a predominance of cases in winter. (1,9) Several risk factors for renal involvement have been described, and the most important contributors include: older age of onset (especially over 10 years) and wintertime, longer interval between symptom onset and diagnosis, rural residence, persistent purpura, severe gastrointestinal symptoms, recurrence, angioedema, obesity, and decreased C3. (1,10,13) Data on sex predominance are conflicting, but most studies suggest a slight male predominance of 1.2-1.8:1. (11) The role of sex in renal manifestations is uncertain. ...
Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.
... The incidence of IgAV is reported to range from 10 to 20 cases per 100,000 children (2). In general, children with IgAV have a good prognosis, but serious complications such as myocarditis and disorders of the nervous system (such as intracranial hemorrhage), respiratory system (such as pulmonary hemorrhage), and reproductive system (such as scrotal edema, orchitis, testicular torsion) may occur owing to individual differences (3)(4)(5)(6). Genital involvement in IgAV is infrequent, and testicular necrosis is extremely rare. To the best of our knowledge, only four cases of testicular necrosis have been reported worldwide (7)(8)(9)(10). ...
Testicular necrosis is a rare and severe complication of immunoglobulin A (IgA) vasculitis (IgAV). Herein, We report a case of a 10-year-old boy who was admitted to the hospital due to skin purpura and intermittent abdominal pain for 10 days and bilateral testicular pain for 2 days. Scrotal ultrasonography indicated right testicle ischemia, right epididymo-orchitis, and bilateral hydrocele of the testis. Scrotal surgical exploration revealed significant swelling and darkening of the right testicle. Conservative treatment led to improvement in his condition, and he was discharged. During 3 months of follow-up, there was no recurrence of skin purpura or pain, and the urine tests were normal. Color ultrasound indicated only partial blood flow signal to the right testicle tissue, which was slightly smaller than the left testicle. This case highlights the need for continuous attention from clinicians to the signs and symptoms of the reproductive system during the diagnosis and treatment of IgAV. Continuous monitoring with ultrasound can aid in early detection, diagnosis, and treatment of reproductive system lesions of IgA vasculitis.
... As the most common form of systemic vasculitis in children, annual incidence rate is up to ~27/100,000 (127). HSP presents as a skin impairment; multi-systemic involvement, which can be life threatening, is a research focus. ...
... HSP presents as a skin impairment; multi-systemic involvement, which can be life threatening, is a research focus. Despite conventional medications to control HSP, such as anti-inflammatory drugs, corticosteroids, cytotoxic drugs and immunosuppressants (8, 127,128). These measures are unsuitable for long-term use due to the high cost, transient efficacy and severe adverse effects, like long-term and large-scale use of glucocorticoids could cause infections and Cushing's syndrome; long-term use of cytotoxic drugs that could result in gastrointestinal reactions, hepatotoxicity, bone marrow suppression, reproductive toxicity, cardiotoxicity (12). ...
Henoch-Schonlein purpura (HSP), a recurrent and immunoglobulin (Ig)A-mediated vasculitis, presents not only as skin lesions but also as systemic involvement that can be life-threatening. Although the etiology of HSP remains unknown, immune imbalance and oxidative stress (OS) are primary contributors to its pathogenesis, alongside the abnormal activation of Toll-like receptor (TLR)/myeloid differentiation primary response gene 88 (MyD88)/nuclear factor-κB (NF-κB) pathway. TLRs, especially TLR4, stimulate downstream signaling molecules such as NF-κB and proinflammatory cytokines, which are released when TLRs combine with the key adapter molecule MyD88. This leads to the activation of T helper (Th) cell 2/Th17 and overproduction of reactive oxygen species (ROS). The function of regulatory T (Treg) cells is suppressed in the process. Th17/Treg imbalance then produces various inflammatory cytokines to promote proliferation and differentiation of B cells and the secretion of antibodies. IgA is secreted, and it binds to vascular endothelial surface receptors where the complex induces injury of the vascular endothelial cells. Additionally, excessive ROS creates OS that leads to an inflammatory response and vascular cell apoptosis or necrosis, thereby contributing to vascular endothelial damage and HSP occurrence. Proanthocyanidins are active compounds naturally enriched in fruits, vegetables and plants. Proanthocyanidins have diverse properties, including anti-inflammatory, antioxidant, antibacterial, immunoregulatory, anticarcinogenic and vascular protective effects. Proanthocyanidins are used in the management of various diseases. Proanthocyanidins regulate T cells, equilibrate immunity and arrest OS by inhibiting the TLR4/MyD88/NF-κB signaling pathway. Considering the pathogenesis of HSP and the properties of proanthocyanidins, the present study hypothesized that these compounds may potentially lead to HSP recovery through modulating the immune equilibrium and preventing OS by inhibiting the TLR4/MyD88/NF-κB pathway. To the best of our knowledge, however, little is known about the positive effects of proanthocyanidins against HSP. The present review summarizes the potential of proanthocyanidins to treat HSP.
... Another study showed a case of Henoch-Schonlein purpura (HSP) with DAH [15]. HSP is a type of small vessel vasculitis that most commonly affects young children. ...
... The gold stander diagnosis of DAH is bronchoalveolar lavage, but it can also be diagnosed with a chest X-ray. DAH is managed with pulse methylprednisolone, unless it progresses to respiratory failure, it managed with immunosuppressive including azathioprine, cyclophamide, and corticosteroid [15]. We could not find any reported cases of patients with HUVS presented DAH. ...
This case report represents a rare case of 14-year-old female who diagnosed with hypocompementemic urticarial vasculitis syndrome that presents with glomerulonephritis, diffuse alveolar hemorrhage, and acute disseminated encephalomyelitis. The progression of the symptoms explained in the text below in which the final diagnosis was reached after a challenging approach. Patient was managed properly and followed up after treating with rituximab, although she represents no sign of the disease after a total of two cycles.
... IgA vasculitis (formerly known as Henoch-Schonlein purpura) typically causes IgA nephropathy and commonly affects the skin, gastrointestinal tract, and joints [2]. It is rare to have pulmonary symptoms, although diffuse alveolar hemorrhage (DAH) has been associated with the disease [3]. The most common trigger is a recent upper respiratory infection [4]. ...
... The main differences are that IgA vasculitis typically also presents with skin manifestations (purpura), GI manifestations (abdominal pain), and arthralgias [2], none of which were present in this patient. In IgA vasculitis, it is rare to have pulmonary involvement, although DAH is the most common form [3]. In many cases, IgA vasculitis occurs after an upper respiratory tract infection but certain medications, insect bites, immunizations, and foods have been associated with the disease as well [4]. ...
Immunoglobulin A (IgA) nephropathy is the most common cause of primary glomerulonephritis worldwide. IgA vasculitis (formerly known as Henoch-Schonlein purpura) typically presents with IgA nephropathy on renal biopsy in addition to extrarenal symptoms like purpura, abdominal pain, and arthritis. Diffuse alveolar hemorrhage (DAH) is the most common pulmonary complication, but this is rarely seen. In this case report, we describe a 35-year-old male with chronic untreated hepatitis B infection who presented with pulmonary-renal syndrome. He was found to have clinical findings of DAH and concomitant IgA nephropathy on renal biopsy, without having any other typical manifestations of IgA vasculitis. This shows that IgA nephropathy should be considered in the differential diagnosis of DAH and emphasizes the importance of a renal biopsy in patients presenting with pulmonary-renal syndrome.
... Laboratorialmente, os achados são aumento de IgA e plaquetas normais ou aumentadas. O tratamento clínico é de suporte e autolimitado, e pode haver associação com a corticoterapia em casos complicados que envolvem o sistema renal, nervoso e o trato gastrointestinal (Di Pietro et al., 2019;Guliaev et al., 2018;Pillebout & Sunderkötter, 2021). ...
Púrpura de Henoch-Schönlein (PHS) é uma doença caracterizada pela inflamação sistêmica de vasos de pequeno calibre, mediada por IgA. A tétrade clássica é composta por púrpura palpável não trombocitopênita, artrite, dor abdominal e glomerulite com hematúria. A doença é desencadeada por inúmeras infecções virais e bacterianas e é, em geral, autolimitada. Porém, há casos em que a PHS acomete múltiplos órgãos e, através de fatores predisponentes, torna-se recorrente e grave. Portanto, o objetivo do estudo é analisar a incidência e fatores predisponentes para recorrência da Púrpura de Henoch-Schönlein em pacientes pediátricos. Trata-se de uma revisão bibliográfica sistemática, que utilizou as plataformas PubMed (Medline), Scientific Eletronic Library On-line (SciELO) e Cochrane Library como bases de dados para a seleção dos artigos científicos, todos na língua inglesa, com recorte temporal de 2017 a 2022. De acordo com as literaturas analisadas, conclui-se que a incidência média anual da Púrpura de Henoch-Schönlein em pacientes pediátricos é de 9 por 100.000 habitantes, que varia de acordo com a localidade. Ademais, Os principais fatores de risco para a recorrência da patologia são as PHS com envolvimento renal, pacientes em corticoterapia, além das alergopatias, como rinite alérgica e dermatite atópica.
... The classic symptoms of Henoch-Schonlein Purpura include (1) erythema purpura (without thrombocytopenia); (2) joints pain (polyarthralgia of the knee, ankle, hand, and wrist joints); (3) gastrointestinal complaints (nausea, vomiting, abdominal pain, acute enteritis, hematemesis, melena, complications of intestinal ischemia, perforation, and intussusception); (4) renal involvement (in 30 -50% of patients it is characterized by asymptomatic hematuria, proteinuria, acute renal failure, progressive glomerulonephritis, and chronic renal failure) (2,3,4). ...
Introduction: Henoch-Schonlein Purpura or Immunoglobulin-A vasculitis is a systemic vasculitis caused by immune complexes that attack small blood vessels. The classic symptoms of Henoch-Schonlein Purpura include erythema purpura, arthralgia, gastrointestinal complaints, and renal involvement. Some cases show that pregnancy itself could be the trigger for its recurrence and lead to early delivery. Case report: A 33-year-old patient, G2P1A0 and 35 weeks and 4 days pregnant complained of diarrhea 8 days before hospital admission (8-15 times per day). The patient was diagnosed with Henoch-Schonlein Purpura 3 years ago. Upon monitoring in the ward, the fetus was found to be in a compromised condition and an emergency cesarean section was needed. The patient was assessed as having an ASA II physical status and was anesthetized with regional anesthesia epidural in the sitting position, with a median approach, puncture at L3-L4 level, and with 12 ml of Bupivacaine 0.5% isobaric. Postoperative care was continued in the ward. Discussion: As long as there are no contraindications, a neuraxial block could be performed on parturient patients with Henoch-Schonlein Purpura who would undergo a cesarean section. Neuraxial block, namely epidural block, has the added advantage of being a postoperative analgesic and helps to avoid the use of Non-Steroidal Anti Inflammatory Drugs (NSAIDs) in Henoch-Schonlein Purpura patients who often have renal complications. Conclusion: Caesarean Section with Henoch-Schonlein Purpura disease has been reported with Epidural Block Anesthesia without complications.
... It appears that the safety profile is similar for adults. 1,8,11,22 It is advised to prescribe treatment for 6 months to a year and then taper off AZA therapy over 6 months to a year. 1 ...
Most patients with atopic dermatitis (AD) have a good response to topical treatment. However, some need systemic therapy in order to satisfactorily control the disease. Azathioprine is an accessible drug for patients in many countries, including underdeveloped countries, and therefore it's used by many dermatologists in moderate and severe AD. It is important to have a deep knowledge and understanding about this drug since it is an alternative therapy as a steroid‐sparing agent and an affordable one. However, when it comes to systemic therapy for AD, it's not always clear its indications and it is necessary to have a closer follow‐up of the patient. In this paper we describe thoroughly it's indications in AD, the mechanism of action of the drug, as well as the interactions, adverse effects, adequate monitoring, and precautions in special population that must be considered when prescribing azathioprine. This review will help dermatologists prescribe it safely to all patients who require it. This article is protected by copyright. All rights reserved.
... Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood and pulmonary involvement is rare. 2 In a review of 23 cases, the median age was 10.2 years and most patients presented with haemoptysis due to alveolar haemorrhage; however, two cases were asymptomatic. Other symptoms included cough, epistaxis, dyspnoea, chest pain and respiratory failure. ...
... All patients met clinical criteria for HSP; most had renal involvement without positive autoantibodies. 2 Investigations showed normal full blood count, C-reactive protein, coagulation, renal/liver function and vasculitis screen. Transthoracic echocardiography is normal. ...
