Sections

IgA Vasculitis (Henoch-Schonlein Purpura)

Sections IgA Vasculitis (Henoch-Schonlein Purpura)
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
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References

Medication

Medication Summary

Very limited data are available on pharmacologic treatment of IgA vasculitis (IgAV; Henoch-Schönlein purpura). Nonsteroidal anti-inflammatory drugs (NSAIDs) may help joint pain 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, but few prospective placebo-controlled studies have demonstrated their effectiveness. Other drugs are currently under investigation.

Class Summary

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

Cyclosporine (Gengraf, Neoral, Sandimmune)

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Cyclosporine is a cyclic polypeptide immunosuppressant agent consisting of 11 amino acids. It is produced as a metabolite by the fungus species Beauveria nivea. Chemically, cyclosporine is designated as [R-[R*,R*-(E)]]-cyclic(L-alanyl-D-alanyl-N-methyl-L-leucyl-N-methyl-L-leucyl-N-methyl-L-valyl-3-hydroxy-N,4-dimethyl-L-2-amino-6-octenoyl-L-α-amino-butyryl-Nmethylglycyl-N-methyl-L-leucyl-L-valyl-N-methyl-L-leucyl).

Methylprednisolone (Solu-Medrol, Medrol, A-Methapred, Depo-Medrol)

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Methylprednisolone decreases inflammation by suppressing PMN migration and reversing increased capillary permeability.

Prednisone (Rayos)

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Prednisone is an immunosuppressant used to treat autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. It stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Prednisolone (Millipred, Orapred, Orapred ODT, Prelone)

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Corticosteroids act as potent inhibitors of inflammation. They may cause profound and varied metabolic effects, particularly in relation to salt, water, and glucose tolerance, in addition to their modification of the immune response of the body.

Cyclophosphamide

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Cyclophosphamide

Cyclophosphamide is an alkylating agent that is chemically related to nitrogen mustards. It is a cyclic polypeptide that suppresses some humoral activity. Cyclophosphamide is transformed in the liver to active alkylating metabolites, which interfere with the growth of rapidly proliferating cells. When cyclophosphamide is used in autoimmune diseases, its mechanism of action is thought to involve immunosuppression through destruction of immune cells by DNA cross-linking.

Class Summary

Analgesic agents are most commonly used for the relief of mild-to-moderate pain. Although the effects of NSAIDs in the treatment of pain tend to be patient-specific, ibuprofen usually is the drug of choice for initial therapy; other options include flurbiprofen, ketoprofen, and naproxen. Some analgesics (eg, acetaminophen and ibuprofen) are also effective for treating fever.

Ibuprofen (Advil, Motrin, I-Prin, Ibu-200, Neo-Profen)

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Ibuprofen is the drug of choice for treatment of mild-to-moderate pain, if not contraindicated; it is also effective for treating fever. Ibuprofen inhibits inflammatory reactions and pain, probably by decreasing the activity of cyclooxygenase, thereby inhibiting prostaglandin synthesis.

Acetaminophen (FeverAll, Q-Pap, Tylenol, APAP 500, Acephen)

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Acetaminophen is effective for treating fever and relieving mild-to-moderate pain. It inhibits the action of endogenous pyrogens on heat-regulating centers; it also reduces fever by a direct action on the hypothalamic heat-regulating centers, thereby, in turn, increasing the dissipation of body heat via sweating and vasodilation.

Flurbiprofen

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Flurbiprofen has analgesic, antipyretic, and anti-inflammatory effects. It may inhibit cyclooxygenase, causing inhibition of prostaglandin biosynthesis.

Ketoprofen

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Ketoprofen is used for relief of mild-to-moderate pain and inflammation.

Naproxen (Anaprox, Naprelan, Naprosyn, Aleve)

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Naproxen is used for relief of mild-to-moderate pain. It inhibits inflammatory reactions and pain by decreasing the activity of cyclooxygenase, thereby reducing prostaglandin synthesis.

Questions

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Media Gallery

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

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Author

Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal) Associate Professor of Pediatrics, Principal Specialist, Department of Pediatrics and Child Health, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, South Africa

Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal) is a member of the following medical societies: African Association of Nephrology, American Association for the Advancement of Science, International Pediatric Nephrology Association, International Society of Nephrology, National Kidney Foundation, South African Medical Association, South African Paediatric Association, South African Renal Society

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD Tenured Professor of Pediatrics, The First Isaac A Abt, MD, Professor of Kidney Diseases (Emeritus), Northwestern University, The Feinberg School of Medicine; Staff Nephrologist, 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 Federation for Clinical Research, American Society for Bone and Mineral Research, American Society of Nephrology, American Society of Pediatric Nephrology, Association of Clinical Scientists, Cochrane Collaboration, International Bone and Mineral Society, International Conferences on Calcium Regulating Hormones, International Pediatric Nephrology Association, International Society of Nephrology, International Society of Renal Nutrition and Metabolism, Midwest Society for Pediatric Research, Pediatric Academic Societies, ROCK (Research on Calculus Kinetics) Society, Society for Pediatric Research

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Horizon Pharmaceuticals); ; Dicerna Pharmaceuticals<br/>Incyte Pharmaceuticals; Eli Lilly for: Federation bio; Dicerna pharmaceuticals.

Additional Contributors

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: Nothing to disclose.

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.

Louansha Nandlal, PhD, MS Research Assistant, Department of Paediatrics and Child Health, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, South Africa

Louansha Nandlal, PhD, MS is a member of the following medical societies: Golden Key International Honour Society, International AIDS Society

Disclosure: Nothing to disclose.

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