Materiały źródłowe

• #1 Henoch-Schonlein purpura – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/henoch-schonlein-purpura/diagnosis-treatment/drc-20354045

  Henoch-Schonlein purpura usually goes away on its own within a month with no lasting ill effects. Rest, plenty of fluids and over-the-counter pain relievers may help with symptoms. […] Corticosteroids, such as prednisone, may help shorten the time and intensity of joint and abdominal pain. Because these drugs can have serious side effects, discuss the risks and benefits of using them with your doctor. […] If a section of the bowel has folded in on itself or ruptured, surgery may be needed.

• #2 Henoch-Schonlein Purpura | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/henoch-schonlein-purpura

  Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body. […] In most cases, the condition does not require treatment; it will resolve on its own and the child will recover completely. […] However, some children with Henoch-Schönlein purpura will develop arthritis and abdominal pain. These children can be treated with pain control medications. […] Treatments for Henoch-Schönlein purpura include supportive care, such as: Adequate hydration, or fluid intake, Careful attention to nutrition, Pain control with medications such as acetaminophen, Glucocorticoids (to control inflammation), Blood pressure medication if elevated blood pressure occurs. […] Specific treatment for Henoch-Schönlein purpura will be determined by your child’s doctor based on: Your child’s overall health and medical history, Extent of the condition, Your child’s tolerance for specific medications, procedures and therapies, Expectation for the course of the disease, Specific organs that are affected, Your opinion or preference.

• #3 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #4 Henoch-Schonlein Purpura | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/henoch-schonlein-purpura

  Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body. […] In most cases, the condition does not require treatment; it will resolve on its own and the child will recover completely. […] However, some children with Henoch-Schönlein purpura will develop arthritis and abdominal pain. These children can be treated with pain control medications. […] Treatments for Henoch-Schönlein purpura include supportive care, such as: Adequate hydration, or fluid intake, Careful attention to nutrition, Pain control with medications such as acetaminophen, Glucocorticoids (to control inflammation), Blood pressure medication if elevated blood pressure occurs. […] Specific treatment for Henoch-Schönlein purpura will be determined by your child’s doctor based on: Your child’s overall health and medical history, Extent of the condition, Your child’s tolerance for specific medications, procedures and therapies, Expectation for the course of the disease, Specific organs that are affected, Your opinion or preference.

• #5 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Treatment of IgAV is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and kidney complications. For minor complaints of arthritis, edema, fever, or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration. […] Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. Such agents are often effective and do not seem to worsen the purpura; however, they should be used cautiously in patients with kidney insufficiency. […] Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in IgAV, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, high-quality, large, prospective studies regarding the treatment of IgAV are lacking, and the evidence does not yet support the use of steroids to prevent or treat kidney disease.

• #6 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #7 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Treatment of IgAV is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and kidney complications. For minor complaints of arthritis, edema, fever, or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration. […] Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. Such agents are often effective and do not seem to worsen the purpura; however, they should be used cautiously in patients with kidney insufficiency. […] Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in IgAV, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, high-quality, large, prospective studies regarding the treatment of IgAV are lacking, and the evidence does not yet support the use of steroids to prevent or treat kidney disease.

• #8 Henoch-Schönlein Purpura (HSP) | Doctor

  https://patient.info/doctor/henoch-schonlein-purpura-pro

  HSP is usually self-limiting and no form of therapy has been shown appreciably to shorten the duration of disease or prevent complications. Therefore, treatment for most patients remains primarily supportive and is entirely symptomatic unless there is renal involvement. This includes rehydration, pain relief, wound care for ulcerative lesions and treatment for intussusception where present. […] Non-steroidal anti-inflammatory drugs (NSAIDs) may help joint pain but should be used with caution in patients with renal insufficiency or gastrointestinal symptoms. […] There is no consensus on prevention or treatment of renal involvement. A Cochrane review found no significant benefit for treatment with steroids, antiplatelet agents or cyclophosphamide. […] However, randomised trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications.

• #9 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube

  https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/

  HSP is usually self-limited. Therefore, treatment is not indicated in all cases, and full recovery is the rule. […] NSAIDs may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Dapsone (100 mg/day) may be effective in cases of HSP, perhaps through disrupting the abnormal immune response. Although steroids have not been evaluated rigorously in HSP, they appear to ease joint and gastrointestinal symptoms, in many (but not all) patients. Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own. […] Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. Keeping the legs elevated may help prevent purpura during flares of active disease. Additionally, many patients’ purpura will recur after they start to feel better and become more active, inherently increasing their exposures to very minor trauma (e.g. jogging, leg shaving, increasing gravity exposures). Often, the recurring purpura is less prevalent, and additional HSP symptoms are often absent. In many fewer cases, primarily in adults, HSP can progress from hematuria (blood in the urine) to renal insufficiency (decreased kidney function). HSP patients who experience this symptom should be followed more closely, with regular testing of their urine for blood and protein. Recurrences, found in 33% of patients, usually develop within the first few months after resolution of the first bout.

• #10 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Treatment of IgAV is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and kidney complications. For minor complaints of arthritis, edema, fever, or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration. […] Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. Such agents are often effective and do not seem to worsen the purpura; however, they should be used cautiously in patients with kidney insufficiency. […] Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in IgAV, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, high-quality, large, prospective studies regarding the treatment of IgAV are lacking, and the evidence does not yet support the use of steroids to prevent or treat kidney disease.

• #11 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Treatment of IgAV is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and kidney complications. For minor complaints of arthritis, edema, fever, or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration. […] Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. Such agents are often effective and do not seem to worsen the purpura; however, they should be used cautiously in patients with kidney insufficiency. […] Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in IgAV, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, high-quality, large, prospective studies regarding the treatment of IgAV are lacking, and the evidence does not yet support the use of steroids to prevent or treat kidney disease.

• #12 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Prednisone in a dosage of 1 mg/kg/day for 2 weeks and then tapered over 2 more weeks may shorten the duration of abdominal pain and joint symptoms, but this benefit must be weighed against the potential adverse effects of steroids. […] The long-term prognosis of IgAV directly depends on the severity of kidney involvement. Patients with IgAV-related kidney dysfunction may benefit from therapy. However, prophylaxis of kidney complications in IgAV, though interesting, is not currently recommended. Treatment of overt IgAV includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications. […] Plasmapheresis may be effective in delaying the progression of kidney disease. A case series demonstrated good outcomes in adults with severe IgAV who were treated with plasma exchange in addition to steroids. […] Surgery may be undertaken to treat severe bowel ischemia. Kidney transplantation may be indicated in patients with severe kidney disease that is resistant to medical therapy. Successful treatment of progressive IgAV nephritis with tonsillectomy and corticosteroid pulse therapy has been reported.

• #13 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Henoch-Schönlein purpura is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis. It is characterized by a clinical triad of palpable purpura (without thrombocytopenia), abdominal pain, and arthritis. Glomerulonephritis and gastrointestinal bleeding are common complications. […] Early oral prednisone treatment at 1 to 2 mg per kg daily for two weeks reduces the intensity of joint and abdominal pain from Henoch-Schönlein purpura in children. […] Early prednisone treatment for Henoch-Schönlein purpura does not prevent renal disease, although it is effective in treating renal involvement and reduces the odds of developing persistent renal disease in children. […] Early prednisone treatment reduces the mean resolution time of abdominal pain from Henoch-Schönlein purpura in children.

• #14 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Hospitalization may be required when adequate outpatient monitoring is unavailable or if dehydration, hemorrhage, or pain control require inpatient management. Nephrology referral is recommended with significant renal involvement. […] Early steroid treatment is most appropriate for children with renal involvement or severe extrarenal symptoms. […] Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat moderate to severe abdominal and joint symptoms, and to hasten the resolution of Henoch-Schönlein purpura in children. […] A double-blind randomized trial found that early treatment with prednisone reduced abdominal and joint pain severity in children. […] Although prednisone did not prevent renal disease, it was useful in treating renal disease after it started.

• #15 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  A meta-analysis found that corticosteroid use in children with Henoch-Schönlein purpura reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. […] Early aggressive therapy is recommended for children and adults with severe renal involvement. […] Treatment options include high-dose steroids with immunosuppressants, high-dose intravenous immunoglobulin, plasmapheresis, and renal transplant. […] A recent trial found that cyclophosphamide (Cytoxan) was effective in patients with overt nephritis, although cyclosporine (Sandimmune) was not helpful.

• #16 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  The treatment of IgAV remains controversial. Treatment of mild skin, joint and GI manifestations is usually symptomatic, including general measures, adequate hydration and analgesics. However, the need to use immunosuppressive (IS) therapy is often determined by the presence of renal involvement. Nonsteroidal anti-inflammatory drugs (NSAIDs) can be beneficial in cases of arthralgia or arthritis in patients without renal or intestinal affection for short periods of time, as long as the patient does not have renal or GI involvement, in which case they should be avoided, due to the risk of deterioration of renal function and GI bleeding. […] Glucocorticoids (GCs) are particularly useful in the acute and early stages of the disease, due to their anti-inflammatory efficacy and rapid onset of action.

• #17 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Prednisone in a dosage of 1 mg/kg/day for 2 weeks and then tapered over 2 more weeks may shorten the duration of abdominal pain and joint symptoms, but this benefit must be weighed against the potential adverse effects of steroids. […] The long-term prognosis of IgAV directly depends on the severity of kidney involvement. Patients with IgAV-related kidney dysfunction may benefit from therapy. However, prophylaxis of kidney complications in IgAV, though interesting, is not currently recommended. Treatment of overt IgAV includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications. […] Plasmapheresis may be effective in delaying the progression of kidney disease. A case series demonstrated good outcomes in adults with severe IgAV who were treated with plasma exchange in addition to steroids. […] Surgery may be undertaken to treat severe bowel ischemia. Kidney transplantation may be indicated in patients with severe kidney disease that is resistant to medical therapy. Successful treatment of progressive IgAV nephritis with tonsillectomy and corticosteroid pulse therapy has been reported.

• #18 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  A meta-analysis found that corticosteroid use in children with Henoch-Schönlein purpura reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. […] Early aggressive therapy is recommended for children and adults with severe renal involvement. […] Treatment options include high-dose steroids with immunosuppressants, high-dose intravenous immunoglobulin, plasmapheresis, and renal transplant. […] A recent trial found that cyclophosphamide (Cytoxan) was effective in patients with overt nephritis, although cyclosporine (Sandimmune) was not helpful.

• #19 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  The beneficial effect of methylprednisolone pulses has been shown in patients receiving combinations of multiple immunosuppressive drugs. […] In IgAV nephritis, a disease considered to be benign, long-term follow-up studies showed delayed development of chronic kidney disease in this population in the absence of rapidly progressive glomerulonephritis when steroids and other immunosuppressants were used. […] Other treatment regimens have included IV or oral steroids with or without any of the following: Azathioprine, Cyclophosphamide, Cyclosporine, Hydroxychloroquine, Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, Dipyridamole, High-dose IV immunoglobulin G (IVIg), Danazol. […] Plasmapheresis may be effective in delaying the progression of kidney disease. Angiotensin converting enzyme inhibitors or receptor blockers may be given for moderately severe proteinuria.

• #20 IgA Vasculitis (Henoch-Schönlein Purpura) – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/henoch-schonlein-purpura

  If there is a lot of protein leaking out, you may be asked to start a drug called an Angiotensin converting enzyme inhibitor (ACE) inhibitor or an angiotension receptor blocker (ARB). These are blood pressure lowering drugs that are especially good at lowering protein leaks from the kidney and reducing the rate of decline in kidney function.

• #21 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  In a retrospective study including 95 adult patients with IgAV who met the 2010 EULAR/PRINTO/PRES classification criteria and had persistent proteinuria, despite treatment with renin–angiotensin–aldosterone system (RAAS) inhibitors, MMF combined with low-dose glucocorticoids (0.4–0.5 mg/kg/day) was shown to be more effective than RAAS inhibition alone in reducing proteinuria and achieving remission after one year of treatment, with fewer adverse effects than high-dose glucocorticoid (0.8–1.0 mg/kg/day) monotherapy. […] Rituximab (RTX) binds to CD20+ lymphocytes causing a drastic decrease in B cells through complement- and antibody-dependent cellular cytotoxicity mechanisms. Currently, RTX use is mainly restricted to refractory IgAVN, due to the scarce evidence on its efficacy regarding renal outcomes or Gd-IgA1 levels, which is supported mainly by isolated clinical reports and case series in both pediatric and adult patients.

• #22 Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

  https://pubmed.ncbi.nlm.nih.gov/30428889/

  Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is characterized by non-thrombocytopenic purpura, abdominal pain, arthritis, and glomerulonephritis. Typically, HSP is self-limited requiring only supportive care, but more severe cases may require corticosteroid (CS) treatment. Rarely, a subset of these patients has persistent rash, arthritis, abdominal involvement, or renal disease despite treatment with CS, or has disease recurrence on CS tapering. Refractory HSP has been effectively treated with a variety of CS sparing therapies. For life-threatening refractory HSP, the B cell depleting agent, rituximab (RTX), has been reported as beneficial for children with substantial renal or central nervous system involvement. […] Herein, we describe 8 children treated with RTX for chronic refractory HSP and report a reduction in recurrent hospitalizations and eventual CS discontinuation. […] Overall, RTX effectively reduced the number of hospital admissions and oral CS burden. RTX also helped most all children achieve clinical remission. RTX appears to be an effective and safe alternative for chronic CS dependent and immunomodulatory refractory childhood HSP.

• #23 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  In a retrospective study including 95 adult patients with IgAV who met the 2010 EULAR/PRINTO/PRES classification criteria and had persistent proteinuria, despite treatment with renin–angiotensin–aldosterone system (RAAS) inhibitors, MMF combined with low-dose glucocorticoids (0.4–0.5 mg/kg/day) was shown to be more effective than RAAS inhibition alone in reducing proteinuria and achieving remission after one year of treatment, with fewer adverse effects than high-dose glucocorticoid (0.8–1.0 mg/kg/day) monotherapy. […] Rituximab (RTX) binds to CD20+ lymphocytes causing a drastic decrease in B cells through complement- and antibody-dependent cellular cytotoxicity mechanisms. Currently, RTX use is mainly restricted to refractory IgAVN, due to the scarce evidence on its efficacy regarding renal outcomes or Gd-IgA1 levels, which is supported mainly by isolated clinical reports and case series in both pediatric and adult patients.

• #24 Azathioprine therapy for steroid-resistant Henoch-Schönlein purpura: a report of 6 cases | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0100-x

  A small percentage of children with Henoch-Schnlein purpura (HSP) develop a chronic form of the disease that often requires prolonged corticosteroid therapy. […] Azathioprine is a DMARD that has been reported to be effective in HSP nephritis and in adult cutaneous leukocytoclastic vasculitis, a condition with cutaneous histology similar to HSP. […] All 6 cases met the classification criteria for the diagnosis of HSP, had relapsing symptoms despite corticosteroid use, were successfully treated with azathioprine and were tapered off of corticosteroids. […] Azathioprine is effective in controlling prolonged relapsing symptoms of HSP, allowing earlier discontinuation of corticosteroids. […] This report shows that azathioprine can be included in the therapeutic options for relapsing HSP and is the first case series in the literature of azathioprine use in HSP without significant renal involvement.

• #25 Azathioprine therapy for steroid-resistant Henoch-Schönlein purpura: a report of 6 cases | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-016-0100-x

  In this case series, we describe 6 patients meeting the classification criteria for HSP, who developed prolonged and relapsing symptoms and were successfully treated with azathioprine. […] The duration of treatment with azathioprine ranged from 7 to 21 months without a relapse of disease after discontinuing treatment. […] Azathioprine appeared to be an effective steroid-sparing medication, allowing all steroid-dependent patients to be successfully tapered off of steroids. […] This case series demonstrates improved symptom control, steroid-sparing effect, and induction of remission with azathioprine in patients with recalcitrant HSP without renal disease.

• #26 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube

  https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/

  HSP is usually self-limited. Therefore, treatment is not indicated in all cases, and full recovery is the rule. […] NSAIDs may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Dapsone (100 mg/day) may be effective in cases of HSP, perhaps through disrupting the abnormal immune response. Although steroids have not been evaluated rigorously in HSP, they appear to ease joint and gastrointestinal symptoms, in many (but not all) patients. Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own. […] Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. Keeping the legs elevated may help prevent purpura during flares of active disease. Additionally, many patients’ purpura will recur after they start to feel better and become more active, inherently increasing their exposures to very minor trauma (e.g. jogging, leg shaving, increasing gravity exposures). Often, the recurring purpura is less prevalent, and additional HSP symptoms are often absent. In many fewer cases, primarily in adults, HSP can progress from hematuria (blood in the urine) to renal insufficiency (decreased kidney function). HSP patients who experience this symptom should be followed more closely, with regular testing of their urine for blood and protein. Recurrences, found in 33% of patients, usually develop within the first few months after resolution of the first bout.

• #27 Henoch-Schonlein purpura (anaphylactoid purpura)

  https://dermnetnz.org/topics/henoch-schoenlein-purpura

  In the majority of cases, no specific treatment is required for HSP and the rash fades over one week. Recurrent crops of lesions occur over the next 6-16 weeks in up to one-third of cases. Five to ten per cent may have persistent disease. […] Non-steroidal-anti-inflammatory-drugs (eg, ibuprofen) are effective at relieving joint and abdominal pain. […] In more severe cases, oral steroids are often used. […] Other options include dapsone or colchicine. […] If significant kidney involvement is present, steroids are usually combined with more potent immunosuppressive medications or intravenous immunoglobulin.

• #28 High-dose methylprednisolone pulse therapy for treatment of refractory intestinal involvement caused by Henoch–Schönlein purpura: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-015-0545-4

  High-dose methylprednisolone pulse therapy can be used as the ultimate treatment for delayed-onset Henoch–Schönlein purpura with severe abdominal pain when symptoms do not improve after low-dose steroid and intravenous immunoglobulin treatments. […] High-dose intravenous methylprednisolone pulse therapy (30mg/kg/day, with a maximum of 1g/day), which dramatically alleviated her abdominal symptoms. […] When HSP with GI involvement is diagnosed, a low-dose steroid (1 to 2mg/kg/day) can be administered initially, followed by IVIg if the symptoms persist. […] Treatment for HSP is controversial. Although in some cases the symptoms may resolve without any therapy, corticosteroids and IVIg have been used in patients with HSP with severe GI involvement as standard and alternative therapy, respectively.

• #29 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  Surgical interventions that may be considered in specific circumstances include the following: Surgery for severe bowel ischemia, Kidney transplantation for severe kidney disease that is resistant to medical therapy, Tonsillectomy together with corticosteroid pulse therapy for progressive IgAV nephritis.

• #30 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  Treatment remains primarily supportive in most cases, though pharmacotherapy, plasmapheresis, and surgical interventions may also be considered in select cases. […] Supportive measures may include the following: Ensuring adequate hydration, Monitoring for GI and kidney complications, Treating minor symptoms of arthritis, edema, fever, or malaise, Eating a bland diet, Discontinuing any drugs suspected of playing a causative role. […] Joint and soft tissue discomfort may be reduced by giving analgesics, such as the following: Acetaminophen, Ibuprofen, Flurbiprofen, Ketoprofen, Naproxen. […] Corticosteroids may be considered in the following situations: Persistent nephrotic syndrome, Crescents in more than 50% of glomeruli, Severe abdominal pain, Substantial GI hemorrhage, Severe soft tissue edema, Severe scrotal edema, Neurologic system involvement, Intrapulmonary hemorrhage.

• #31 Gastrointestinal and renal involvement in vasculitis: Management strategies in Henoch-Schönlein purpura | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/66/5/312

  The joint pain, inflammation, and painful cutaneous edema of Henoch-Schonlein purpura (HSP) are effectively treated with analgesics, nonsteroidal anti-inflammatory agents, and corticosteroids, but the optimal management of HSP-associated gastrointestinal and renal involvement has not yet been determined. […] The results of mostly anecdotal and uncontrolled studies favor a short course of oral corticosteroids for severe abdominal pain and aggressive immunosuppressive therapy for patients with progressive HSP nephritis.

• #32 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Hospitalization may be required when adequate outpatient monitoring is unavailable or if dehydration, hemorrhage, or pain control require inpatient management. Nephrology referral is recommended with significant renal involvement. […] Early steroid treatment is most appropriate for children with renal involvement or severe extrarenal symptoms. […] Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat moderate to severe abdominal and joint symptoms, and to hasten the resolution of Henoch-Schönlein purpura in children. […] A double-blind randomized trial found that early treatment with prednisone reduced abdominal and joint pain severity in children. […] Although prednisone did not prevent renal disease, it was useful in treating renal disease after it started.

• #33 High-dose methylprednisolone pulse therapy for treatment of refractory intestinal involvement caused by Henoch–Schönlein purpura: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-015-0545-4

  High-dose methylprednisolone pulse therapy can be used as the ultimate treatment for delayed-onset Henoch–Schönlein purpura with severe abdominal pain when symptoms do not improve after low-dose steroid and intravenous immunoglobulin treatments. […] High-dose intravenous methylprednisolone pulse therapy (30mg/kg/day, with a maximum of 1g/day), which dramatically alleviated her abdominal symptoms. […] When HSP with GI involvement is diagnosed, a low-dose steroid (1 to 2mg/kg/day) can be administered initially, followed by IVIg if the symptoms persist. […] Treatment for HSP is controversial. Although in some cases the symptoms may resolve without any therapy, corticosteroids and IVIg have been used in patients with HSP with severe GI involvement as standard and alternative therapy, respectively.

• #34 Henoch Schönlein Purpura (HSP)

  https://rheumatology.org/patients/henoch-schonlein-purpura-hsp

  Most children with HSP do not require any specific treatment and recover with time alone. […] Joint pain can often be controlled with rest and over-the-counter medications. […] Steroids given by mouth or through an IV infusion are typically given for severe abdominal pain. […] Many children with HSP can be treated by their primary care provider. […] If symptoms are severe or additional expertise is needed, a rheumatologist can help evaluate and treat a child for HSP. […] One of the most important parts of HSP management is monitoring for kidney disease. […] Children with worsening urinary findings like high blood pressure or rising creatinine (a blood marker of kidney function) should be seen by a nephrologist (kidney doctor). […] A biopsy may be needed to assess for kidney disease. […] Some children with kidney involvement will need treatment with long-term immune suppressive medications to prevent kidney failure.

• #35 Henoch-Schonlein purpura (anaphylactoid purpura)

  https://dermnetnz.org/topics/henoch-schoenlein-purpura

  In the majority of cases, no specific treatment is required for HSP and the rash fades over one week. Recurrent crops of lesions occur over the next 6-16 weeks in up to one-third of cases. Five to ten per cent may have persistent disease. […] Non-steroidal-anti-inflammatory-drugs (eg, ibuprofen) are effective at relieving joint and abdominal pain. […] In more severe cases, oral steroids are often used. […] Other options include dapsone or colchicine. […] If significant kidney involvement is present, steroids are usually combined with more potent immunosuppressive medications or intravenous immunoglobulin.

• #36 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  Treatment of IgAV is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and kidney complications. For minor complaints of arthritis, edema, fever, or malaise, symptomatic treatment is advised, including use of acetaminophen, elevation of swollen extremities, eating a bland diet, and adequate hydration. […] Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. Such agents are often effective and do not seem to worsen the purpura; however, they should be used cautiously in patients with kidney insufficiency. […] Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in IgAV, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, high-quality, large, prospective studies regarding the treatment of IgAV are lacking, and the evidence does not yet support the use of steroids to prevent or treat kidney disease.

• #37 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube

  https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/

  HSP is usually self-limited. Therefore, treatment is not indicated in all cases, and full recovery is the rule. […] NSAIDs may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Dapsone (100 mg/day) may be effective in cases of HSP, perhaps through disrupting the abnormal immune response. Although steroids have not been evaluated rigorously in HSP, they appear to ease joint and gastrointestinal symptoms, in many (but not all) patients. Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own. […] Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. Keeping the legs elevated may help prevent purpura during flares of active disease. Additionally, many patients’ purpura will recur after they start to feel better and become more active, inherently increasing their exposures to very minor trauma (e.g. jogging, leg shaving, increasing gravity exposures). Often, the recurring purpura is less prevalent, and additional HSP symptoms are often absent. In many fewer cases, primarily in adults, HSP can progress from hematuria (blood in the urine) to renal insufficiency (decreased kidney function). HSP patients who experience this symptom should be followed more closely, with regular testing of their urine for blood and protein. Recurrences, found in 33% of patients, usually develop within the first few months after resolution of the first bout.

• #38 Henoch-Schonlein purpura (anaphylactoid purpura)

  https://dermnetnz.org/topics/henoch-schoenlein-purpura

  In the majority of cases, no specific treatment is required for HSP and the rash fades over one week. Recurrent crops of lesions occur over the next 6-16 weeks in up to one-third of cases. Five to ten per cent may have persistent disease. […] Non-steroidal-anti-inflammatory-drugs (eg, ibuprofen) are effective at relieving joint and abdominal pain. […] In more severe cases, oral steroids are often used. […] Other options include dapsone or colchicine. […] If significant kidney involvement is present, steroids are usually combined with more potent immunosuppressive medications or intravenous immunoglobulin.

• #39 IgA Vasculitis (Henoch-Schönlein Purpura) – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/henoch-schonlein-purpura

  IgA vasculitis, previously known as Henoch-Schönlein Purpura (HSP), is a systemic vasculitis which can affect the skin, joints, bowel and kidneys. […] No specific treatment is needed for most cases of IgAV in children or adults with the symptoms resolving spontaneously over time. Anti-inflammatory drugs (such as ibuprofen) are often prescribed for the joint pains, unless there is kidney involvement. There is some evidence that steroid treatment may lead to more rapid improvement of bowel symptoms in children, as well as clearing up the rash. Skin disease may also be treated with drugs such as dapsone. If the kidney is involved and there is inflammation in the kidney or if there are severe bowel symptoms then treatment with steroids or other immunosuppressants may be needed to reduce inflammation. Long term protection of kidney function may require certain blood pressure lowering drugs.

• #40 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #41 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  A meta-analysis found that corticosteroid use in children with Henoch-Schönlein purpura reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. […] Early aggressive therapy is recommended for children and adults with severe renal involvement. […] Treatment options include high-dose steroids with immunosuppressants, high-dose intravenous immunoglobulin, plasmapheresis, and renal transplant. […] A recent trial found that cyclophosphamide (Cytoxan) was effective in patients with overt nephritis, although cyclosporine (Sandimmune) was not helpful.

• #42 IgA Vasculitis (Henoch-Schönlein Purpura) – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/henoch-schonlein-purpura

  If there is a lot of protein leaking out, you may be asked to start a drug called an Angiotensin converting enzyme inhibitor (ACE) inhibitor or an angiotension receptor blocker (ARB). These are blood pressure lowering drugs that are especially good at lowering protein leaks from the kidney and reducing the rate of decline in kidney function.

• #43 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10–30% of those affected eventually progressing to end-stage renal disease. […] Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium–glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors.

• #44 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10–30% of those affected eventually progressing to end-stage renal disease. […] Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium–glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors.

• #45 IgA Vasculitis – Henoch-Schönlein Purpura – UF Health

  https://ufhealth.org/conditions-and-treatments/iga-vasculitis-henoch-sch%C3%B6nlein-purpura

  There is no specific treatment. Most cases go away on their own. Joint pain may improve with NSAIDs such as naproxen. If symptoms do not go away, you may be prescribed a corticosteroid medicine such as prednisone. […] The disease most often gets better on its own. Two thirds of children with IgA vasculitis have only one episode. One third of children have more episodes. People should have close medical follow-up for 6 months after episodes to look for signs of kidney disease. Adults have a greater risk of developing chronic kidney disease.

• #46 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Henoch-Schonlein-Purpura-Treatment-and-Follow-Up.aspx

  People who have elevated blood pressure as a result of HSP require its careful control. […] Patients need to have a follow-up urinalysis performed at weekly intervals at first, and then progressively further apart. These tests should be continued for at least 6 months after the initial episode of HSP to detect any persistent or progressive renal damage.

• #47 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #48 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Hospitalization may be required when adequate outpatient monitoring is unavailable or if dehydration, hemorrhage, or pain control require inpatient management. Nephrology referral is recommended with significant renal involvement. […] Early steroid treatment is most appropriate for children with renal involvement or severe extrarenal symptoms. […] Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat moderate to severe abdominal and joint symptoms, and to hasten the resolution of Henoch-Schönlein purpura in children. […] A double-blind randomized trial found that early treatment with prednisone reduced abdominal and joint pain severity in children. […] Although prednisone did not prevent renal disease, it was useful in treating renal disease after it started.

• #49 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #50 Henoch- Schoenlein Purpura

  https://www.printo.it/pediatric-rheumatology/AU/info/8/Henoch–Schoenlein-Purpura

  In most cases of HSP, drug treatment is not necessary or is administered only for a short time; hence, no severe side effects are expected. In rare cases, when severe renal disease requires the use of prednisone/prednisolone and immunosuppressive drugs for a long time, drug side effects may be a problem. […] The entire course of the disease is about 4-6 weeks. Half of children with HSP have at least one recurrence within a 6-week period, which is usually briefer and milder than the first episode. Relapses rarely last longer. A recurrence is not indicative of the severity of the disease. The majority of patients recover completely.

• #51 IgA Vasculitis – Henoch-Schönlein Purpura – UF Health

  https://ufhealth.org/conditions-and-treatments/iga-vasculitis-henoch-sch%C3%B6nlein-purpura

  There is no specific treatment. Most cases go away on their own. Joint pain may improve with NSAIDs such as naproxen. If symptoms do not go away, you may be prescribed a corticosteroid medicine such as prednisone. […] The disease most often gets better on its own. Two thirds of children with IgA vasculitis have only one episode. One third of children have more episodes. People should have close medical follow-up for 6 months after episodes to look for signs of kidney disease. Adults have a greater risk of developing chronic kidney disease.

• #52 Henoch-Schönlein Purpura (HSP): Symptoms and Treatment

  https://patient.info/allergies-blood-immune/henoch-schonlein-purpura-leaflet

  Other treatment will depend on whether complications develop. For example, if the kidneys become involved, referral to a kidney specialist for assessment and their advice about treatment may be advised. A kidney sample (biopsy) may be suggested to help give the specialist more information and guide them as to the best treatment. Treatment may include steroids and other medicines to help suppress the body’s defence (immune) system. […] Occasionally a process called plasma exchange is needed if the complications caused by HSP are very serious. […] In many people with Henoch-Schönlein purpura, no complications develop. But, complications sometimes develop. They can include the following: […] The long-term outlook (prognosis) mainly depends on whether and how badly the kidneys have been affected: […] HSP may come back within six months of first having HSP. It is more likely to come back if the kidneys have been affected.

• #53 Treatment of Henoch-Schonlein purpura (HSP) Nephritis | PEDSnet

  https://pedsnet.org/study/treatment-of-henoch-schonlein-purpura-hsp-nephritis/

  Treatment of Henoch-Schonlein purpura (HSP) Nephritis […] Henoch-Schonlein purpura (HSP) is one of the most common forms of vasculitis in children, with an incidence of around 6-24 cases per 100,000 per year and with 90% of cases presenting before age ten. While many cases are self-limited, 30-50% of patients with HSP will develop renal involvement with about 20% having a nephritic or nephrotic syndrome, and 1-3% of patients progressing to end stage renal disease. […] Currently there is a lack of evidence to guide treatment decisions for patients with HSP nephritis (HSPN), and therefore there is wide variation in treatment practices. Many small, uncontrolled, retrospective studies have attempted to describe various treatment regimens and their outcomes, but a large, multi-center, prospective trial has not been attempted. Current treatment recommendations are based on case series, retrospective analyses, and extrapolation of studies in patients with IgA nephropathy.

• #54 Treatment of Henoch-Schonlein purpura (HSP) Nephritis | PEDSnet

  https://pedsnet.org/study/treatment-of-henoch-schonlein-purpura-hsp-nephritis/

  Thus, a prospective, multicenter clinical trial is needed to examine the outcomes in children with HSPN treated with different agents and protocols (e.g. ACE inhibition, corticosteroids, and other immunosuppressive agents). With the results of such a study, specific evidence-based guidelines could be created to guide the management of children with HSPN. […] We hypothesize that wide practice variation exists in the treatment of children with HSPN. Aim 1: To evaluate clinical and laboratory factors at initial presentation which can predict the course of patients with HSP nephritis, including duration of proteinuria and development of chronic kidney disease/end stage renal disease. Aim 2: To evaluate the association of various treatment regimens (e.g. ACE inhibitors, oral/IV steroids, other immunosuppressive agents, and conservative management without pharmaceutical agents) with outcomes such as time to remission of proteinuria and change in GFR in children with HSP nephritis.

• #55 IgA Vasculitis (Henoch–Schönlein Purpura): An Update on Treatment

  https://www.mdpi.com/2077-0383/13/21/6621

  IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10–30% of those affected eventually progressing to end-stage renal disease. […] Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium–glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors.

• #56 IgA Vasculitis (Henoch-Schonlein Purpura) Treatment & Management: Approach Considerations, Supportive Management, Pharmacologic Therapy

  https://emedicine.medscape.com/article/984105-treatment

  To date, no form of therapy has been found to shorten the duration of IgA vasculitis (IgAV; Henoch-Schnlein purpura) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that IgAV is a self-limited disease. Most patients recover quickly (within several weeks) without treatment. […] Hospital admission and monitoring for complications should be considered, especially for IgAV patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked kidney insufficiency. Complications such as acute abdomen, acute scrotum, and acute kidney injury may be severe and may occur precipitously. Severe GI bleeding may warrant acute immunosuppressive treatment. […] Nephropathy is treated supportively, with monitoring of fluid and electrolyte balance, restriction of salt intake, and administration of antihypertensives when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent progression of kidney disease, but the results have been inconsistent, and data from controlled studies do not support the use of prophylaxis.

• #57 Henoch-Schonlein Purpura | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/henoch-schonlein-purpura

  Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body. […] In most cases, the condition does not require treatment; it will resolve on its own and the child will recover completely. […] However, some children with Henoch-Schönlein purpura will develop arthritis and abdominal pain. These children can be treated with pain control medications. […] Treatments for Henoch-Schönlein purpura include supportive care, such as: Adequate hydration, or fluid intake, Careful attention to nutrition, Pain control with medications such as acetaminophen, Glucocorticoids (to control inflammation), Blood pressure medication if elevated blood pressure occurs. […] Specific treatment for Henoch-Schönlein purpura will be determined by your child’s doctor based on: Your child’s overall health and medical history, Extent of the condition, Your child’s tolerance for specific medications, procedures and therapies, Expectation for the course of the disease, Specific organs that are affected, Your opinion or preference.

• #58 Henoch-Schönlein Purpura: A Literature Review | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2733

  Henoch-Schnlein purpura is the most common childhood vasculitis, but may also affect adults. […] Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. […] The treatment strategies for HSP remain controversial. The general agreement is to base therapy on the presence or absence of renal involvement. Without renal involvement, the treatment is purely symptomatic. […] There is disagreement between researchers and clinicians whether to use corticosteroids. […] An updated Cochrane review from 2015 aimed to clarify the different treatment options for kidney involvement in patients with HSP, compared with placebo or other treatments.

• #59 Henoch-Schönlein Purpura: A Literature Review | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2733

  Henoch-Schnlein purpura is the most common childhood vasculitis, but may also affect adults. […] Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. […] The treatment strategies for HSP remain controversial. The general agreement is to base therapy on the presence or absence of renal involvement. Without renal involvement, the treatment is purely symptomatic. […] There is disagreement between researchers and clinicians whether to use corticosteroids. […] An updated Cochrane review from 2015 aimed to clarify the different treatment options for kidney involvement in patients with HSP, compared with placebo or other treatments.

• #60 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  A meta-analysis found that corticosteroid use in children with Henoch-Schönlein purpura reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. […] Early aggressive therapy is recommended for children and adults with severe renal involvement. […] Treatment options include high-dose steroids with immunosuppressants, high-dose intravenous immunoglobulin, plasmapheresis, and renal transplant. […] A recent trial found that cyclophosphamide (Cytoxan) was effective in patients with overt nephritis, although cyclosporine (Sandimmune) was not helpful.

• #61 Henoch–Schönlein purpura – Wikipedia

  https://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

  As of 2017, the optimal way to treat Henoch–Schönlein purpura remains controversial. Analgesics may be needed for the abdominal and joint pains. Wound care is warranted if skin death and ulcerations occur. It is uncertain as to whether HSP needs treatment beyond controlling the symptoms. Most people do not receive therapy because of the high spontaneous recovery rate. Experts disagree on whether to routinely use corticosteroids as treatment for HSP. However, if they are given early in the disease episode, the duration of symptoms may be shortened, and abdominal pain can improve significantly. Moreover, the chance of severe kidney problems may be reduced. A systematic review did not find any evidence that steroid treatment (prednisone) is effective at decreasing the likelihood of developing long-term kidney disease.

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