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Henoch-Schonlein Purpura Treatment & Management

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Craig B Langman, MD  more...
 
Updated: Sep 28, 2015
 

Approach Considerations

To date, no form of therapy has been found to shorten the duration of Henoch-Schönlein purpura (HSP) to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consonant with the understanding that HSP is a self-limited disease. The majority of patients recover quickly (ie, within several weeks) without treatment.[55]

Management of HSP includes adequate hydration; immediate discontinuance of any exposure to antigenic stimulants (eg, drugs); and follow-up each week for the first month, every other week for the second month, and monthly thereafter until abnormal urinary findings subside.

Patients with HSP are often admitted to the hospital and monitored for abdominal and renal complications, which may be severe and may occur precipitously (eg, acute abdomen, acute scrotum, and renal failure). Hospitalization should be strongly considered for HSP patients with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked renal insufficiency.

HSP may mimic an abdominal emergency and, in its severest form, result in small-bowel infarction, perforation, or both.

In adults with HSP, permanent renal involvement is not uncommon. Hematuria at disease onset and persistence of renal manifestations during the occurrence of HSP can be significant predictors of possible development of renal sequelae. These manifestations, along with other features (eg, onset in summer, anemia at disease onset, or relapses of the disease), may predict the development of renal sequelae in most patients. All pregnant women with even mild renal symptoms at the onset of HSP should be carefully observed during and after pregnancy.

Nephropathy is treated supportively. Patients’ fluid and electrolyte balance should be monitored, their salt intake should be restricted, and antihypertensives should be prescribed when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and plasmapheresis have been used to prevent renal disease from progressing. The results have been inconsistent. No data from controlled studies are available.

Dietary restrictions have no clear role in the management of HSP. Activities can be performed as tolerated.

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Supportive Management

Treatment of HSP is primarily supportive and includes ensuring adequate hydration and monitoring for abdominal and renal 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. All unnecessary drugs should be discontinued if a drug-related etiology is suspected.

Most patients with self-limited cases can be safely discharged home with close follow-up by the primary physician. The decision for or against hospital admission depends on the physician’s customary practice and individual preference. Admission to the hospital is recommended for control of abdominal pain or vomiting, monitoring of renal function, confirming a doubted diagnosis, and observation and monitoring. Patients with renal involvement require close attention to their fluid balance, electrolyte status, and use of antihypertensives (if indicated).

A study examining prevention and treatment of renal disease in patients with HSP revealed no significant difference in the risk of persistent kidney disease at 6 months and 12 months in children given prednisone for 14-28 days upon presentation in comparison with placebo or supportive treatment.[56] Also, no significant difference was noted in the risk of persistent renal disease in children given cyclophosphamide compared with supportive treatment and with cyclosporin compared with methylprednisolone.

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Pharmacologic Therapy

Analgesics

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 renal insufficiency.

Corticosteroids

Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in HSP, as well as to ameliorate associated arthralgias and symptoms associated with GI dysfunction. However, good, large, prospective studies regarding the treatment of HSP are lacking,[56, 57, 58] and the evidence does not yet support the use of steroids to prevent or treat renal disease.[59, 60] Some authors recommend steroids; others do not. Nevertheless, 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

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.[61, 62]

A review of randomized clinical trials for any intervention used to improve renal disease in children with HSP noted that data were very limited except for short-term prednisone; moreover, prednisone had no benefit in preventing serious long-term renal disease.[60]

The long-term prognosis of HSP directly depends on the severity of renal involvement. Patients with HSP-related renal dysfunction may benefit from therapy. However, prophylaxis of renal complications in HSP, though interesting, is not currently recommended. Treatment of overt HSP includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications. If prednisone is used, a regimen consisting of 1-2 mg/kg/day PO for 7 days is recommended. Antihypertensives may be indicated with renal involvement.

Faedda reported favorable results from the following protocols in patients with severe HSP[63] :

  • Induction with 250-750 mg of intravenous (IV) methylprednisolone daily for 3-7 days plus cyclophosphamide 100-200 mg/d administered orally (PO)
  • Maintenance with prednisone 100-200 mg PO every other day plus cyclophosphamide 100-200 mg/d PO 30-75 days
  • Tapering of prednisone by approximately 25 mg/month (with the cyclophosphamide dose remaining constant)
  • Discontinuance of treatment after at least 6 months by abruptly discontinuing cyclophosphamide and tapering prednisone completely

Other agents

Other treatment regimens have included IV or oral steroids with or without any of the following:

  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine
  • Dipyridamole
  • High-dose IV immunoglobulin G (IVIg)
  • Danazol
  • Fish oil

Of these, only cyclophosphamide has been shown to be effective in a randomized controlled trial. Although some studies have reported success, cyclosporine does not have sufficient clinical data to establish its utility in this setting.[64]

Azathioprine, mycophenolate mofetil, and urokinase must be tested before their use is consistently advocated. Guidelines for prescribing azathioprine in dermatology have been established.[65] No convincing studies have yet been conducted regarding the use of IVIg, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP.[66]

A randomized clinical trial of cyclosporine with methylprednisolone pulses in HSP with nephritis found that cyclosporine was superior and had many fewer complications.[67] A study of 12 patients with severe HSP nephritis indicated that patients did well with methylprednisolone at 30 mg/kg/day for 3 days followed by oral corticosteroids at 2 mg/kg/day for 2 months, cyclophosphamide at 2 mg/kg/day for 2 months, and dipyridamole at 5 mg/kg/day for 6 months.[68]

Some have noted that parvovirus B19–associated HSP must be recognized in adults because the treatment of choice is IV gamma globulin combined with anti–tumor necrosis factor (TNF)-α therapy. In contrast, immunosuppressive therapy may lead to a persistent or worsening disease course in these patients.

Massive GI hemorrhage in isolated intestinal HSP that is responsive to IVIg infusion has been reported.[69] IVIg was used in a complex case of HSP with brain hemorrhage, but more work must be done to validate the use of this treatment.[70]

Dapsone has been used to treat associated purpuras and arthralgias. Iqbal and Evans found it to be effective for HSP.[71]

Rituximab has been noted to be a successful treatment for severe refractory chronic HSP.[72]

Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated. Recombinant factor VIIa has been used with very pronounced factor XIII deficiency and compartment syndrome.

Treatment of complicated HSP with mycophenolate mofetil has been reported in a series of patients.[73]

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Plasmapheresis

Plasmapheresis may be effective in delaying the progression of kidney disease. A case series demonstrated good outcomes in adults with severe HSP who were treated with plasma exchange in addition to steroids.[74] In an uncontrolled study, Shenoy et al reported that children with severe HSP and IgA nephropathy recover well if treated with plasmapheresis alone, without the need for immunosuppressive therapy.[75] Plasmapheresis has been useful in treating rapidly progressive HSP nephritis.[76]

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Surgical Intervention

Surgery may be undertaken to treat severe bowel ischemia. Kidney transplantation may be indicated in patients with severe renal disease that is resistant to medical therapy. Successful treatment of progressive HSP nephritis with tonsillectomy and corticosteroid pulse therapy has been reported.

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Consultations

Because HSP is a multisystem disease, consultations with the following specialists can be helpful in diagnosis and treatment:

  • Dermatologist
  • Gastroenterologist
  • Nephrologist (particularly for assistance in determining if dialysis is indicated)
  • Rheumatologist
  • Dermatopathologist [77] (the utility of the consultation is limited to untypical or incomplete manifestations of HSP to buttress positive vascular IgA immunohistochemical evidence of the deposits)

Consultation is recommended for patients with renal involvement before discharge from the emergency department and for all patients who appear acutely ill.

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Long-Term Monitoring

In all patients, urinalysis and blood pressure monitoring to evaluate for renal involvement should be continued for up to 6 months after presentation, even if initial urinalysis results were normal. Once the initial course of prednisone is administered, additional prednisone appears to have no role.

When terminal renal failure develops, long-term hemodialysis should be instituted until a kidney is available for transplantation. Mesangial deposits of IgA are common in the graft, but they rarely lead to clinical manifestations of recurrent glomerulonephritis.

Children who have demonstrated renal manifestations in the acute phase and continue to have hematuria or proteinuria should be examined every 3-6 months because renal failure or hypertension can develop up to 10 years after disease onset.

NSAIDs may be administered to address joint problems. Because of the risk of Reye syndrome, the use of NSAIDs should be discussed with the patient’s physician.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Coauthor(s)

Elena L Jones, MD Clinical Assistant Professor of Dermatology, Columbia University College of Physicians and Surgeons; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Raptor Pharmaceuticals; Eli Lilly and Company; Dicerna<br/>Received grant/research funds from NIH for none; Received grant/research funds from Raptor Pharmaceuticals, Inc for none; Received grant/research funds from Alexion Pharmaceuticals, Inc. for none; Received consulting fee from DiCerna Pharmaceutical Inc. for none.

Acknowledgements

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians

Disclosure: Nothing to disclose.

Philip Bossart, MD Professor, Department of Surgery, Division of Emergency Medicine, University of Utah Hospital, University of Utah School of Medicine

Philip Bossart, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte Medical Center

Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Pamela L Dyne, MD Professor of Clinical Medicine/Emergency Medicine, University of California, Los Angeles, David Geffen School of Medicine; Attending Physician, Department of Emergency Medicine, Olive View-UCLA Medical Center

Pamela L Dyne, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Gina M Forte Medical Assistant, The Skin Cancer Surgery Center

Disclosure: Nothing to disclose.

Edmond A Hooker II, MD, DrPH, FAAEM Associate Professor, Department of Health Services Administration, Xavier University, Cincinnati, Ohio; Assistant Professor, Department of Emergency Medicine, University of Cincinnati College of Medicine

Edmond A Hooker II, MD, DrPH, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American Public Health Association, Society for Academic Emergency Medicine, and Southern Medical Association

Disclosure: Nothing to disclose.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Heather Kesler DeVore, MD Assistant Professor, Clinical Attending Physician, Department of Emergency Medicine, Georgetown University Hospital and Washington Hospital Center

Heather Kesler DeVore, MD is a member of the following medical societies: Emergency Medicine Residents Association and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Andrew D Montemarano, DO Consulting Staff, The Skin Cancer Surgery Center

Andrew D Montemarano, DO is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Society for Dermatologic Surgery, and MedChi

Disclosure: Nothing to disclose.

Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical & Dental Society, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Stacy Sawtelle, MD Clinical Instructor, Department of Emergency Medicine, University of California, San Francisco, School of Medicine

Disclosure: Nothing to disclose.

Debra Slapper, MD Consulting Staff, Department of Emergency Medicine, St Anthony's Hospital

Debra Slapper, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Robert J Willard, MD Dermatologist and Mohs Surgeon, Private Practice, Dermatology and Mohs Surgery Center, PC

Robert J Willard, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center; Chairman, Pediatric Institutional Review Board, Mercy St Vincent Medical Center, Toledo, Ohio

Wayne Wolfram, MD, MPH, is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

David Timothy Woodley, MD Professor and Chair, Department of Dermatology, Keck School of Medicine of the University of Southern California

David Timothy Woodley, MD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association

Disclosure: Lotus Tissue Repair Ownership interest owner and advisor

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Purpuric papules and plaques of the lower extremity characteristic of Henoch-Schönlein purpura.
Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura.
Typical rash distribution of Henoch-Schönlein purpura.
Characteristic rash of Henoch-Schönlein purpura.
Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation with ecchymoses on dorsal foot and ankle.
A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Image courtesy of Pamela L Dyne, MD.
A 7-year-old girl with Henoch-Schönlein purpura. Image courtesy of Pamela L Dyne, MD.
 
 
 
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