eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Henoch-Schonlein Purpura: Treatment & Medication

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): Elena L Jones, MD, Clinical Assistant Professor of Dermatology, College of Physicians and Surgeons of Columbia University; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center
Contributor Information and Disclosures

Updated: Sep 28, 2009

Treatment

Medical Care

Patients with Henoch-Schonlein purpura (HSP) are often admitted to the hospital and monitored for abdominal and renal complications.

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 (steroids, azathioprine, cyclophosphamide) and plasmapheresis have been used to prevent renal disease from progressing. The results have been inconsistent. No data from controlled studies are available.

Consultations

Because Henoch-Schoenlein purpura is a multisystem disease, consultations with the following specialists can be helpful in diagnosis and treatment.

  • Dermatologist
  • Gastroenterologist
  • Nephrologist
  • Rheumatologist

Diet

Dietary restrictions have no clear role in Henoch-Schoenlein purpura.

Activity

Activity can be performed as tolerated.

Medication

To date, no form of therapy appreciably shortens the duration of Henoch-Schoenlein purpura. Therefore, treatment for most patients remains primarily supportive. This is consonant with the understanding that Henoch-Schoenlein purpura is a self-limited disease.

Shenoy et al reported in an uncontrolled study that children with severe Henoch-Schoenlein purpura and IgA nephropathy recover well if treated with plasmapheresis alone without the need for immunosuppressive therapy.32 Plasmapheresis therapy has also been useful in treating rapidly progressive Henoch-Schoenlein purpura nephritis.33

Corticosteroids can ameliorate associated arthralgias and the symptoms associated with GI dysfunction. No definitive evidence shows that corticosteroids affect the outcome of renal disease; nevertheless, corticosteroids may be considered in the following serious 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

No controlled clinical trials have been performed with immunosuppressive drugs, although some claim beneficial results with azathioprine or cyclophosphamide. Guidelines for prescribing azathioprine in dermatology have been established.34

The long-term prognosis of Henoch-Schoenlein purpura directly depends on the severity of renal involvement. The renal dysfunction that Henoch-Schoenlein purpura causes can benefit from therapy. However, the prophylactic treatment of renal complications in Henoch-Schoenlein purpura, although interesting, is not mandated because study has yielded conflicting results and is ultimately unproven. The treatment of overt Henoch-Schoenlein purpura includes methylprednisolone pulse therapy and prednisone and other immunosuppressive medications.

Only cyclophosphamide has been demonstrated as effective in a recent randomized controlled trial. Although some studies have reported success, cyclosporin does not have clinical data to back its use.35 ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be tested before their use is consistently advocated. Plasmapheresis has shown effectiveness in delaying the progression of kidney disease. However, no convincing studies have yet been conducted regarding the use of intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat Henoch-Schoenlein purpura.36

Massive GI hemorrhage in isolated intestinal Henoch-Schoenlein purpura that is responsive to intravenous immunoglobulin infusion has been reported.37

Faedda reported favorable results in patients with severe Henoch-Schoenlein purpura with the following protocols:38

  1. Induction with 250-750 mg of intravenous (IV) methylprednisolone daily for 3-7 days plus cyclophosphamide 100-200 mg/d administered orally (PO)
  2. Maintenance with prednisone 100-200 mg PO every other day plus cyclophosphamide 100-200 mg/d PO 30-75 days
  3. Tapering of prednisone by approximately 25 mg/mo (The cyclophosphamide dose remains constant.)
  4. Discontinuation of treatment after at least 6 mo by abruptly discontinuing cyclophosphamide and complete prednisone taper

Some have noted that parvovirus B19–associated Henoch-Schoenlein purpura must be recognized in adults because the treatment of choice is IV gamma globulin combined with anti–TNF-alfa therapy. In contrast, immunosuppressive therapy may lead to a persistent and/or worsening disease course in these patients.

Successful treatment of progressive Henoch-Schoenlein purpura nephritis with tonsillectomy and corticosteroid pulse therapy has been reported.

Iqbal and Evans reported the use of dapsone therapy for Henoch-Schoenlein purpura and found it effective.39

Plasmapheresis therapy has been useful for treating rapidly progressive Henoch-Schoenlein purpura nephritis.

Immunosuppressive agents

These agents decrease inflammation by suppressing migration of polymorphonuclear (PMN) leukocytes and reversing increased capillary permeability.


Methylprednisolone (Solu-Medrol)

Decreases inflammation by suppressing PMN leukocyte migration and reversing increased capillary permeability.

Adult

0.25 mg/kg/d IV (typically about 1 g/d) for 3-7 d

Pediatric

Severe HSP: 250-750 mg IV qd for 3-7 d (administer with cyclophosphamide)

Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels; phenobarbital, phenytoin, and rifampin may decrease levels (adjust dose); monitor for hypokalemia with coadministration of diuretics; grapefruit juice increases concentrations; inhibits cyclosporine and vice versa, increasing plasma levels of both

Documented hypersensitivity; viral, fungal, or tubercular skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections possible complications of glucocorticoid use


Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Adult

5-60 mg/d PO qd or divided bid/qid; taper over 2 wk, as symptoms resolve

Pediatric

Short-term treatment: 4-5 mg/m2/d PO or 0.05-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve adjust dose accordingly if potent forms of cortisone used
Severe HSP: After induction with IV methylprednisolone, start maintenance therapy with prednisone 100-200 mg PO qod for 30-75 d (in addition to cyclophosphamide); then taper by 25 mg/mo for >6 mo; then begin final slow taper

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral, fungal, or tubercular skin lesions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in hyperthyroidism, cirrhosis, nonspecific ulcerative colitis, osteoporosis, peptic ulcer, diabetes mellitus, and myasthenia gravis; abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use


Cyclophosphamide (Cytoxan)

Chemically related to nitrogen mustards. As alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Cyclic polypeptide that suppresses some humoral activity. Transformed in liver to active alkylating metabolites, which interfere with growth of rapidly proliferating cells. When used in autoimmune diseases, mechanism of action thought to involve immunosuppression because of destruction of immune cells by DNA cross-linking.

Adult

10 mg/kg/d IV q2wk

Pediatric

Administer as in adults or 100-200 PO mg/d

Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; toxicity may increase with chloramphenicol; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia; coadministration with succinylcholine may increase neuromuscular blockade by inhibiting cholinesterase activity

Documented hypersensitivity; severely depressed bone marrow function

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; instruct patient to consume a large quantity of water each day while taking cyclophosphamide

More on Henoch-Schonlein Purpura

Overview: Henoch-Schonlein Purpura
Differential Diagnoses & Workup: Henoch-Schonlein Purpura
Treatment & Medication: Henoch-Schonlein Purpura
Follow-up: Henoch-Schonlein Purpura
Multimedia: Henoch-Schonlein Purpura
References
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Further Reading

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Keywords

Henoch Schönlein purpura, Henoch-Schönlein purpura, Henoch Schonlein purpura, Henoch-Schonlein purpura, Henoch Schoenlein purpura, Henoch-Schoenlein purpura, HSP, allergic purpura, Henoch's purpura, Schönlein purpura, Schönlein's purpura, Schönlein disease, Schönlein's disease, Henoch-Schönlein purpura, Henoch-Schönlein syndrome, Schönlein-Henoch syndrome, vascular purpura, acute vascular purpura, anaphylactoid purpura, hemorrhagic exudative erythema, purpura nervosa, purpura rheumatica, rheumatocelis, purpura fulminans, purpura hemorrhagica, nonthrombocytopenic purpura, rheumatoid purpura, allergic purpura

hemorrhagic capillary toxicosis, nonthrombocytopenic idiopathic purpura, peliosis rheumatica, rheumatic purpura, acute hemorrhagic edema of infancy, AHEI, postinfectious cockade purpura, Finkelstein disease, Finkelstein's disease, Seidelmayer syndrome, Seidelmayer's syndrome, infantile postinfectious irislike purpura and edema, vasculitis, arthritis, cutaneous purpura, orchitis, nephritis, species, adenoviruses, Epstein-Barr virus, EBV, parvoviruses, varicella, hypertension, proteinuria, mononucleosis, group A streptococcal infection, hepatitis, varicella-zoster infection

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Elena L Jones, MD, Clinical Assistant Professor of Dermatology, College of Physicians and Surgeons of Columbia University; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center
Disclosure: Nothing to disclose.

Medical Editor

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: The Osler Institute Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Adrian Spitzer, MD, Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director of NIH Training Program, Children's Hospital at Montefiore Medical Center
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.

CME Editor

Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD, The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology
Disclosure: Amgen Grant/research funds None; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
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