eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases
Polyarteritis Nodosa: Treatment & Medication
Updated: Sep 28, 2006
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Currently, corticosteroids plus cyclophosphamide is the standard of care for idiopathic PAN and for patients with more severe disease. This combination can provide prolonged survival for these patients. In contrast, for hepatitis B–related PAN, treatment consists of schemes that include plasmapheresis and antiviral agents.
Treatment may include a regimen of prednisone (1 mg/kg/d) and cyclophosphamide (2 mg/kg/d). In general, patients respond better to combined therapy with immunosuppressants and corticosteroids than to steroids alone (see Mortality/Morbidity).
Surgical Care
Microcoil embolization of cerebral aneurysm may be indicated.
Consultations
- Cardiologist
- Gastroenterologist
- Dermatologist
- Rheumatologist
- Neurologist
- Physiatrist: Physical and occupational therapies are initiated promptly. Once the patient's condition is stable, ambulation and maximizing activities of daily living are the chief goals.
Medication
Immunosuppression has been the standard therapy for PAN. Recent data suggest that a combination of 2 or more different immunosuppressants can improve the outcome.
Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Orasone, Sterapred)
May inhibit cyclooxygenase, which in turn inhibits prostaglandin biosynthesis. These effects may result in analgesic, antipyretic, and anti-inflammatory activities.
Adult
1 mg/kg/d PO
Pediatric
Administer as in adults
Estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism (consider increasing maintenance dose); monitor for hypokalemia when diuretics are coadministered
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Abrupt discontinuation may cause adrenal crisis; serious adverse reactions with long-term use include hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections
Alkylating/antineoplastic agents
These agents inhibit cell growth and proliferation.
Cyclophosphamide (Cytoxan, Neosar, Procytox [Canada])
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.
Adult
2 mg/kg/d IV
Pediatric
Not established
Allopurinol may increase risk of bleeding or infection and exacerbate myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Documented hypersensitivity; severely depressed bone marrow function
Pregnancy
D - Unsafe in pregnancy
Precautions
Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis
Methotrexate (Rheumatrex, Folex, Abitrexate, Methotrate, Mexate)
Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust dose gradually to attain satisfactory response.
Adult
5-7.5 mg/wk PO/IM
Pediatric
Not established
Coadministration with NSAIDs may be fatal
Oral aminoglycosides may decrease absorption and blood levels; charcoal lowers levels; etretinate may increase hepatotoxicity; folic acid or its derivatives contained in some vitamins may decrease response; indomethacin and phenylbutazone can increase plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia)
Pregnancy
D - Unsafe in pregnancy
Precautions
Monitor CBC counts monthly, and liver and renal function q1-3mo, during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated levels, eg, dehydration)—discontinue if significant drop in blood counts; has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested)
More on Polyarteritis Nodosa |
| Overview: Polyarteritis Nodosa |
| Differential Diagnoses & Workup: Polyarteritis Nodosa |
 Treatment & Medication: Polyarteritis Nodosa |
| Follow-up: Polyarteritis Nodosa |
| Multimedia: Polyarteritis Nodosa |
| References |
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References
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Further Reading
Keywords
PAN, microscopic polyangiitis, systemic necrotizing vasculitis, arteritis nodosa, Kussmaul disease, Kussmaul's disease, periarteritis nodosa, microscopic polyarteritis, polyarteritis nodosa
