eMedicine Specialties > Emergency Medicine > Hematology & Oncology
Henoch-Schonlein Purpura: Treatment & Medication
Updated: Jun 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Emergency Department Care
Treatment of Henoch-Schönlein purpura is mostly supportive and includes ensuring adequate hydration and monitoring for abdominal and renal complications.
Consultations
Consultation is recommended for patients with renal involvement prior to discharge from the ED and for all patients who appear acutely ill.
Medication
Very limited data are available on the treatment of Henoch-Schönlein purpura. Fortunately, most patients recover quickly in several weeks without treatment.5
Nonsteroidal anti-inflammatory drugs (NSAIDs) may help joint pain and do not seem to worsen the purpura. However, NSAIDs should be used cautiously in patients with renal insufficiency.
Clinicians often use corticosteroids to treat abdominal pain, subcutaneous edema, and nephritis, but few prospective placebo-controlled studies have demonstrated their effectiveness. Some authors recommend steroids and others do not. Good, large, prospective studies regarding the treatment of Henoch-Schönlein purpura (HSP) are lacking.6,7,8
Prednisone in a dose of 1 mg/kg/d for 2 weeks and then tapered over 2 more weeks has been shown to improve gastrointestinal and joint symptoms. Although this regimen did not decrease the incidence of renal disease, it did lessen the severity of nephritis in some patients.9
Other treatment regimens have included IV or oral steroids with or without any of the following: azathioprine, cyclophosphamide, cyclosporine, dipyridamole, plasmapheresis, high-dose IV immunoglobulin G (IVIg), danazol, or fish oil. A recent study of 12 patients with severe Henoch-Schönlein purpura (HSP) nephritis indicated that patients did well with a treatment of methylprednisolone at 30 mg/kg/d for 3 days followed by oral corticosteroids at 2 mg/kg/d for 2 months, cyclophosphamide at 2 mg/kg/d for 2 months, and dipyridamole at 5 mg/kg/d for 6 months.10
Nonsteroidal anti-inflammatory drugs (NSAIDs)
These agents are most commonly used for the relief of mild to moderately severe pain. Although the effects of NSAIDs in the treatment of pain tend to be patient specific, ibuprofen usually is the DOC for initial therapy. Other options include flurbiprofen, ketoprofen, and naproxen.
Ibuprofen (Ibuprin, Advil, Motrin)
DOC for treatment of mild to moderately severe pain, if no contraindications. Inhibits inflammatory reactions and pain, probably by decreasing activity of enzyme cyclooxygenase, which results in inhibition of prostaglandin synthesis.
Adult
600 mg PO qid
Pediatric
10 mg/kg PO q6h
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy
Flurbiprofen (Ansaid)
Has analgesic, antipyretic, and anti-inflammatory effects; may inhibit enzyme cyclooxygenase, causing inhibition of prostaglandin biosynthesis.
Adult
200-300 mg/d PO divided bid/qid
Pediatric
Not established
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug
Ketoprofen (Oruvail, Orudis, Actron)
Used for relief of mild to moderately severe pain and inflammation.
Adult
25-50 mg PO q6-8h prn; not to exceed 300 mg/d
Pediatric
<3 months: Not established
3 months to 12 years: 0.1-1 mg/kg PO q6-8h
>12 years: Administer as in adults
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity—it increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Administer small dosages initially to patients with small body size, to those who are elderly, and to those with renal or liver disease; doses higher than 75 mg do not increase therapeutic effects; administer high doses with caution and closely observe patient for response; avoid in GI disease, cardiovascular disease, and renal or hepatic impairment; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; caution in patients who have coagulation defects or are receiving anticoagulant therapy
Naproxen (Anaprox, Naprelan, Naprosyn)
Used for relief of mild to moderately severe pain. Inhibits inflammatory reactions and pain by decreasing activity of enzyme cyclooxygenase, which results in decrease of prostaglandin synthesis.
Adult
250-500 mg PO bid; may increase to 1.5 g/d for limited periods
Pediatric
2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; low WBC counts occur rarely and usually return to normal during ongoing therapy; discontinuation of therapy may be necessary if leukopenia, granulocytopenia, or thrombocytopenia persists; exercise caution in patients who have coagulation defects or are receiving anticoagulant therapy
Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, they modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Sterapred, Orasone)
Useful in treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.
Adult
60 mg/d PO qd
Pediatric
1-2 mg/kg/d PO divided bid
Estrogens may decrease clearance; concurrent digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; peptic ulcer disease; hepatic dysfunction; viral, fungal, connective tissue, or tubercular skin infections
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation may cause adrenal crisis; adverse effects include hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, infections, and weight gain
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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References
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M, Gonzalez-Gay MA. Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. May 1997;40(5):859-64. [Medline].
Szer IS. Henoch-Schonlein purpura. Curr Opin Rheumatol. Jan 1994;6(1):25-31. [Medline].
Gedalia A. Henoch-Schönlein purpura. Curr Rheumatol Rep. Jun 2004;6(3):195-202. [Medline].
Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. May 2002;13(5):1271-8. [Medline].
O'Brien WM, O'Connor KP, Horan JJ, Eggli DF, Gibbons MD. Acute scrotal swelling in Henoch-Schonlein syndrome: evaluation with testicular scanning. Urology. Apr 1993;41(4):366-8. [Medline].
[Best Evidence] Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review. Arch Dis Child. Feb 2009;94(2):132-7. [Medline].
Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. Mar 24 2007;369(9566):976-8. [Medline].
Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383]. BMC Med. Apr 2 2004;2:7. [Medline].
[Best Evidence] Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. Aug 2006;149(2):241-7. [Medline].
Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schönlein Purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol. Mar-Apr 2001;21(2):128-33. [Medline].
Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].
[Best Evidence] Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics. Nov 2007;120(5):1079-87. [Medline].
Further Reading
Keywords
Henoch-Schonlein purpura, Henoch-Schönlein purpura, HSP, symptoms, treatment, vasculitis, small-vessel vasculitis, purpura, arthritis, skin rash, spots, abdominal pain, hematuria, nonthrombocytopenic purpura, purpura rheumatica, upper respiratory illness, URI, HSP nephritis, immunoglobulin A nephropathy, IgA nephropathy, rash, subcutaneous edema, scrotal edema, palpable purpura, HSP-related intussusception, idiopathic thrombocytopenic purpura, ITP, thrombotic thrombocytopenic purpura, TTP
