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Adult-onset Henoch-Schonlein purpura (HSP) tends to become chronic-relapsing, yet rarely leads to organ impairment, e.g. due to chronic glomerulonephritis. Bed rest, compression and nonsteroidal anti-inflammatory drugs are usually sufficient to control the active phases. We report 2 cases of adult HSP with an unusually severe evolution. One patient...
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... be refractory and at the third day of inpatient treatment (15 days after the first consultation) the patient de- veloped acute and profuse hematemesis and melena, which led to hypovolemic shock within a few hours. The patient was stabilized and transferred to the intensive- care unit. Gastroscopic images showed a serious hemorrhagic duodenitis ( fig. 3 ), while colonoscopy showed hemorrhagic colitis and terminal ileitis. Histologies provided evidence of exclusively necrotic colon mucosa, but no vasculitic lesions. Abdominal contrast computed tomogra- phy showed a pan-enterocolitis, but no ev- idence of mesenterial artery obstructions. She was put on intravenous methyl- prednisolone ...
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Background: Henoch-Schönlein purpura (HSP) is a rare systemic small vessel vasculitis, which commonly occur in children between 2 and 10 years of age. The course of the disease is often self-limiting, although may manifest long-term renal morbidity. The severity of renal involvement decides about the prognosis of this disease. Many factors can trig...
Abstract
Cardiac involvement in Henöch-Schonlein purpura (HSP) is rare with only a few case reports available in the literature . We report
a 21-year-old male who presented with rash, arthralgia, and chest pain. Raised cardiac enzymes indicated myocardial injury. There
was evidence of pericarditis on echocardiography. The patient was diagnosed as h...
Citations
... It is difficult to make evidence-based recommendations for management of very severe forms of IgAV since they are rare. There are no clinical trials, only isolated case reports on administration of immunoglobulins [70] or use of plasmapheresis [71][72][73][74] in addition to intravenous steroids and immunosuppressants in patients with severe gastrointestinal, pulmonary or renal involvement, suggesting their benefit in those indications. ...
IgA vasculitis (IgAV) is an inflammation of small vessels caused by perivascular deposition of IgA and activation of neutrophils. It may present as systemic vasculitis (IgAV - Henoch-Schönlein purpura) or as a variant restricted to the skin (skin-limited IgAV), while IgA nephropathy presents a variant restricted to the kidneys. Systemic IgAV affects children more frequently than adults (150 to 200 for 1; incidence 1 in 1 million/year). In the latter, disease more often leads to chronic renal disease. The dominant clinical features include round or oval and retiform palpable purpura predominantly on the lower legs, arthralgia or arthritis, gastrointestinal bleeding or pain and glomerulonephritis with mesangial IgA deposits (IgAVN). Pulmonary, cardiac, genital and neurological involvement occurs, but is rare. Immune complexes containing galactose-deficient IgA1 play a pivotal role in the pathophysiology of IgAV; via the Fc alpha receptor (CD89), they induce neutrophilic inflammation around cutaneous vessels and mesangial proliferation and inflammation in the glomerulus. In case of self-limited disease, only symptomatic treatment is recommended. Treatment of severe IgAV, nephritis or gastrointestinal manifestations, is not established, but some studies reported a benefit of corticosteroids, combined with immunosuppressive drugs. Short-term outcome depends on the severity of gastrointestinal manifestations, while long-term prognosis depends on the severity of nephritis.
... Il est difficile de recommander une prise en charge des formes très sévères de VIgA puisqu'elles sont exceptionnelles. Il n'y a aucun essai clinique, seulement des cas isolés, rapportés qui suggèrent l'intérêt d'ajouter des immunoglobulines [41] ou des plasmaphérèses [8,[42][43][44] aux stéroïdes intraveineux et aux immunosuppresseurs dans les atteintes digestives, pulmonaires, rénales, voire cutanées sévères. ...
IgA vasculitis (IgAV) is a systemic IgA vasculitis affecting small vessels. IgAV usually affects children; it is rare in adults (150-200 for 1). The disease is often more serious in adults with more frequent and severe nephritis. Prevalence of adult IgAV is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis, and gastrointestinal symptoms. Sometimes nephritis occurs, typically as glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital, and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of IgAV. Only symptomatic treatment is advised in case of selflimited disease. Treatment of severe IgAV, nephritis, or gastrointestinal manifestations, is not established but some studies, which need to be confirmed, reported the benefit of corticosteroids combined with immunosuppressive drugs. Short-term outcome depends on the severity of the gastrointestinal manifestations. The long-term prognosis is heavily dependent on the presence and severity of nephritis. Studies with prolonged follow-up show up to one third of adult patients reaching end stage renal failure.
... However, it is used in severe cases with crescent formation, combined with steroids. There are several case reports suggesting that plasmapheresis may have a beneficial effect on treatment of patients with rapidly progressive HSPN because plasmapheresis can remove circulating complexes, inflammatory, and procoagulatory substance [68][69][70]. However the benefit of plasmapheresis is still not as definite as it is in Anti-neutrophil cytoplasmic antibodies-associated vasculitis. Recently, a group of pediatric rheumatologists have developed recommendations for the management of patients with IgAV/HSP and its nephritis although the majority lack high grade of evidence and are 'expert opinion' (unpublished data): ...
Introduction: IgA vasculitis/Henoch Schönlein Purpura (IgAV/HSP) is the most common childhood vasculitis in most parts of the world. This is basically a self-limited disease, however the association of gastrointestinal and renal morbidity is well known.
Areas covered: This review focuses on the classification of IgAV/HSP and management of specific organ involvements based on recent recommendations.
Expert opinion: The new pediatric classification criteria allow pediatricians to pursue multicenter controlled studies for the unmet needs in the disease. Clinicians should be aware of the early morbidity associated with GI involvement and the late morbidity associated with renal involvement. Recurrences are possible. Treatment depends on the extent of clinical features and organ involvement.
... In addition, plasmapheresis should be considered as an optional treatment, if no obvious therapeutic effects are obtained after multiple drug therapy. 11 ...
Hemothorax is an extremely rare complication of Henoch–Schönlein purpura. In this report, we encountered a 9-year-old girl who suffered from nonthrombocytopenic purpura on her eyelids, buttocks, and lower limbs with hypertension, heme-positive stools, high serum immunoglobulin A, and low serum complement component 3. Her skin biopsy revealed leukocytoclastic vasculitis with immunoglobulin A deposition. In addition, physical examination showed dullness on percussion and quiet breath sounds on the left chest. Chest X-ray showed a large pleural effusion on the left hemithorax. Computed tomography of her thorax revealed massive left-sided hemothorax with mediastinal shift to the right side. Most critically, pleural fluid evacuated by the chest tube was bloody. The patient was diagnosed as having Henoch–Schönlein purpura in combination with hemothorax, and treated with pulse methylprednisolone followed by oral corticosteroids plus cyclophosphamide. Fortunately, we achieved excellent outcomes and the patient recovered gradually within a week.
... Role of plasmapheresis in HSP nephritis is unclear. Some small studies have shown an improvement in patients with HSP and cerebritis, but others have no clear benefit in HSP associated RPGN [57,58]. ...
Glomerulonephritis [GN] is one of the common acquired pediatric renal disorders encountered in clinical practice. The clinical manifestations include gross or microscopic hematuria, proteinuria, and nephrotic syndrome. Renal dysfunction and hypertension may also be present in many patients. Etiopathogenesis of GN can be idiopathic in a large majority, while some may result from infections or known immune disorders. Several of these disorders are now believed to arise from dysfunctions of podocytes and are grouped under the heading of “podocytopathies”. This review focuses on the clinical manifestations and management of the common forms of acute GN encountered in children.
... Various treatment modalities have been used, including steroids and immunosuppression, but there is currently no consensus on the most effective treatment [6]. Therapeutic plasma exchange (TPE) has also been used in adults and children for a number of years but has been limited to short-term therapy only [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. Here we present a young adult with HSP and rapidly progressive kidney disease in whom we established long-term regular TPE for over three years to successfully hold off progression to end-stage renal disease (ESRD). ...
... The use of TPE alone or in combination with immunomodulation therapies in the acute phase of HSP is well reported in the literature [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. However to the best of our knowledge there has been no previous report of the long-term use of TPE to prevent the onset of ESRD. ...
A 27-year-old man presented with a palpable purpuric skin rash and joint and abdominal pain in April 2010. He had acute kidney injury and his creatinine quickly deteriorated to 687
μ
mol/L, with associated nephrotic range proteinuria. Kidney biopsy showed crescentic Henoch-Schonlein nephritis. He was treated with intravenous cyclophosphamide and prednisolone despite which his renal function deteriorated; he required haemodialysis for a short duration and seven sessions of therapeutic plasma exchange (TPE). Renal function improved, but after discharge from hospital he suffered 2 further relapses, each with AKI, in 4 months. Cyclophosphamide was not effective and therefore Rituximab was introduced. He initially had a partial response but his renal function deteriorated despite continued therapy. TPE was the only treatment that prevented rapid renal functional deterioration. A novel long-term treatment strategy involving regular TPE every one to two weeks was initiated. This helped to slow his progression to end-stage kidney disease over a 3-year period and to prolong the need for renal replacement therapy over this time.
... There are also several reports [45,46] suggesting that plasmapheresis is an effective way to improve the prognosis of patients with rapidly progressive HSPN because plasmapheresis can remove circulating complexes and inflammatory and procoagulatory substances. Donghi et al [47] reported 2 cases of adult HSP with an unusually severe evolution. In both patients, the disease was refractory to common immunosuppression with systemic corticosteroids (oral and pulse) and additive steroid-sparing immunosuppressive drugs. ...
Henoch-Schönlein purpura (HSP) is one of the most common vasculitides in children. It is manifested by skin purpura, arthritis, abdominal pain, renal involvement, etc. Typically, HSP is considered to be self-limiting, although renal involvement (HSP purpura nephritis, HSPN) is the principal cause of morbidity from this disease. For this reason, it is important to clarify the mechanism of onset and clinical manifestations of HSPN and to ascertain the most appropriate treatment for HSPN. In this article, we review the updated pathophysiology and treatment strategies for HSPN.
We searched databases including PubMed, Elsevier and Wanfang for the following key words: Henoch-Schönlein purpura, nephritis, mechanism and treatment, and we selected those publications written in English that we judged to be relevant to the topic of this review.
Based on the data present in the literature, we reviewed the following topics: 1) the possible pathogenesis of HSPN: several studies suggest that immunoglobulin A immune complexes deposit in the mesangium and induce renal injury; 2) multiple-drug treatment for HSPN: although there have been few evidence-based treatment strategies for HSPN, several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity.
HSPN is a severe disease of childhood. To better understand this disease, detailed investigations into the pathogenesis of HSPN and prospective randomized controlled treatment studies on children with severe HSPN are needed.
... 0.12% of plasma exchange procedures were associated with severe complications such as anaphylactic reactions and bleeding. And the incidence of death with plasma exchange has been estimated to be 0.05% (2,17,18) There have been some cases with HSP GIS involvement, which were refractory to immunosuppressive therapy and have been successfully treated with plasma exchange (4,5,11,19). Moreover, plasma exchange has been used as an effective therapy choice for HSP patients having severe cerebral involvement (20,21). ...
... We did not previously use plasma exchange as a first-line therapy and in addition started immunosuppressive agents. Similar to the literature, we thought that using immune modulator therapy before or concurrently with plasma exchange may be necessary to suppress the inflammation more effectively and to inhibit the production of new cytokines (5,11). Among our small case series with severe GIS involvement, three had the diagnosis of FMF. ...
The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schönlein purpura (HSP) when refractory to conventional therapies.
We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted.
We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment.
Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.
... Available data are scarce in adults and limited to case reports in which PE was used as the sole therapy, in association with steroids or as a rescue treatment. [19][20][21][22][23] These reports are summarized in Table 4. Even if the pathophysiology of childhood and adulthood-onset HSP may not overlap, interesting data come from pediatric studies. ...
Adult Henoch-Schönlein purpura (HSP) has been associated with poor outcome and end-stage renal disease in >20% of cases. Although the benefit of adding another immunosuppressant to steroids in severe adult HSP has not been shown, the benefit of plasma exchange (PE) therapy has been poorly evaluated.
Case series.
11 consecutive patients with severe and newly diagnosed HSP since 1988 who were treated with steroids and PE.
Patients' characteristics and outcome were analyzed. Birmingham Vasculitis Activity Score (BVAS), estimated glomerular filtration rate (eGFR), and proteinuria were measured at baseline, at the end of PE treatment, at months 6 and 12, and at the last visit. Side effects of corticoid treatment and PE were recorded.
11 patients were identified in 1988-2010. Patients received intravenous corticoid pulses in 64% of cases, followed by oral prednisone for a median of 6.6 months. They received a median of 12 PE sessions. BVAS, eGFR, and proteinuria improved significantly between baseline and the last PE at a median of 2 months. PE sessions were well tolerated, except in one patient who developed central catheter-associated septicemia. One patient required dialysis therapy 15 days after HSP diagnosis and did not recover kidney function. At the last medical evaluation at a mean follow-up of 6 years, median eGFR and proteinuria were 83 ± 22 mL/min/1.73 m(2) and protein excretion of 140 ± 10 mg/d, respectively. 3 women had pregnancy without complications.
This case series did not have a control group.
The combination of PE and corticoid therapy in severe forms of HSP was associated with fast improvement and good long-term outcome.
The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. In the Ninth Edition, the JCA Special Issue Writing Committee has incorporated systematic review and evidence-based approaches in the grading of evidence and categorization of apheresis indications to make recommendations on the use of apheresis in a wide variety of diseases and conditions. This edition has largely maintained the general layout and concept of a fact sheet introduced in the Fourth Edition (2007). Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease or medical condition. The Ninth Edition of the JCA Special Issue comprises 91 fact sheets and 166 graded and categorized indications. This includes seven new fact sheets, nine new indications on existing fact sheets, and eight changes in the category for existing indications. The Ninth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.
